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1. Increased incidence of inflammatory bowel disease on etanercept in juvenile idiopathic arthritis regardless of concomitant methotrexate use

Author

van Straalen, J, Krol, R, Giancane, G, Panaviene, V, Ailioaie, L, Doležalová, P, Cattalini, M, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Sawhney, S, Rygg, M, Oliveira, S, Nordal, E, Saad-Magalhães, C, Rubio-Perez, N, Jelusic, M, de Roock, S, Wulffraat, N, Ruperto, N, Swart, J, van Straalen JW, Krol RM, Giancane G, Panaviene V, Ailioaie LM, Doležalová P, Cattalini M, Susic G, Sztajnbok FR, Maritsi D, Constantin T, Sawhney S, Rygg M, Oliveira SK, Nordal EB, Saad-Magalhães C, Rubio-Perez N, Jelusic M, de Roock S, Wulffraat NM, Ruperto N, Swart JF, van Straalen, J, Krol, R, Giancane, G, Panaviene, V, Ailioaie, L, Doležalová, P, Cattalini, M, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Sawhney, S, Rygg, M, Oliveira, S, Nordal, E, Saad-Magalhães, C, Rubio-Perez, N, Jelusic, M, de Roock, S, Wulffraat, N, Ruperto, N, Swart, J, van Straalen JW, Krol RM, Giancane G, Panaviene V, Ailioaie LM, Doležalová P, Cattalini M, Susic G, Sztajnbok FR, Maritsi D, Constantin T, Sawhney S, Rygg M, Oliveira SK, Nordal EB, Saad-Magalhães C, Rubio-Perez N, Jelusic M, de Roock S, Wulffraat NM, Ruperto N, and Swart JF

Abstract

Objective: To describe risk factors for IBD development in a cohort of children with JIA. Methods: JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different DMARDs were calculated, and differences between therapies were expressed as relative risks (RR). Results: Out of 8942 patients, 48 (0.54%) developed IBD. These were more often male (47.9% vs 32.0%) and HLA-B27 positive (38.2% vs 21.0%) and older at JIA onset (median 8.94 vs 5.33 years) than patients without IBD development. They also had more often a family history of autoimmune disease (42.6% vs 24.4%) and enthesitis-related arthritis (39.6% vs 10.8%). The strongest predictors of IBD on multivariable analysis were enthesitis-related arthritis [odds ratio (OR): 3.68, 95% CI: 1.41, 9.40] and a family history of autoimmune disease (OR: 2.27, 95% CI: 1.12, 4.54). Compared with methotrexate monotherapy, the incidence of IBD on etanercept monotherapy (RR: 7.69, 95% CI: 1.99, 29.74), etanercept with methotrexate (RR: 5.70, 95% CI: 1.42, 22.77) and infliximab (RR: 7.61, 95% CI: 1.27, 45.57) therapy was significantly higher. Incidence on adalimumab was not significantly different (RR: 1.45, 95% CI: 0.15, 13.89). Conclusion: IBD in JIA was associated with enthesitis-related arthritis and a family history of autoimmune disease. An increased IBD incidence was observed for etanercept therapy regardless of concomitant methotrexate use. Methods: JIA patients who developed IBD were identified from the international Pharmachild register. Characteristics were compared between IBD and non-IBD patients and predictors of IBD were determined using multivariable logistic regression analysis. Incidence rates of IBD events on different DMARDs were calculated, and differences between therapies were expressed as r

Published
2022

3. Decreasing Incidence of the Multisystem Inflammatory Syndrome in Children over 3 Pandemic Waves

Author

Eleftheriou, I. Maritsi, D. Lampidi, S. Charisi, K. Vantsi, P. Skourti, K. Filippatos, F. Amplianitis, I. Dimou, D. Papadopoulou-Legbelou, K. Papadopoulou-Alataki, E. Kampouridou, P. Koletsi, P. Fotis, L. Vergadi, E. Gkentzi, D. Farmaki, E. Papaevangelou, V. Galanakis, E. Grivea, I.N. Syrogiannopoulos, G.A. Spoulou, V. Spyridis, N. Michos, A. Roilides, E. Tsolia, M.N. and Eleftheriou, I. Maritsi, D. Lampidi, S. Charisi, K. Vantsi, P. Skourti, K. Filippatos, F. Amplianitis, I. Dimou, D. Papadopoulou-Legbelou, K. Papadopoulou-Alataki, E. Kampouridou, P. Koletsi, P. Fotis, L. Vergadi, E. Gkentzi, D. Farmaki, E. Papaevangelou, V. Galanakis, E. Grivea, I.N. Syrogiannopoulos, G.A. Spoulou, V. Spyridis, N. Michos, A. Roilides, E. Tsolia, M.N.

Abstract

In this nationwide retrospective study, a substantial decline in the incidence of multisystem inflammatory syndrome in children over 3 successive pandemic waves characterized by different severe acute respiratory syndrome coronavirus 2 variants was documented - from 3.4 of 1000 to 1.1 of 1000 and finally to 0.25 of 1000 confirmed severe acute respiratory syndrome coronavirus 2 positive cases (P < 0.0001), respectively, whereas clinical findings and severity did not significantly vary. © 2023 Lippincott Williams and Wilkins. All rights reserved.

Published
2023

4. Antiphospholipid syndrome after streptococcal toxic shock syndrome later diagnosed as Behcet's disease

Author

Kourti, A. Mentesidou, L. Michalopoulou, A. Maritsi, D. Bachou, T. and Kourti, A. Mentesidou, L. Michalopoulou, A. Maritsi, D. Bachou, T.

Abstract

Streptococcal toxic shock syndrome is a severe complication of group A streptococci. The production of antiphospholipid antibodies has been associated with streptococcal infections and with autoimmune diseases. Furthermore, streptococcal infections could be a trigger of Behcet's disease. We report a case of a boy who presented antiphospholipid syndrome after streptococcal toxic shock syndrome later he was diagnosed with Behcet's disease. © 2023 Lippincott Williams and Wilkins. All rights reserved.

Published
2023

5. Treatment of Multisystem Inflammatory Syndrome in Children

Author

McArdle A. J., Vito O., Patel H., Seaby E. G., Shah P., Wilson C., Broderick C., Nijman R., Tremoulet A. H., Munblit D., Ulloa-Gutierrez R., Carter M. J., De T., Hoggart C., Whittaker E., Herberg J. A., Kaforou M., Cunnington A. J., Levin M., Vazquez J. A., Carmona R., Perez L., Rubinos M., Veliz N., Yori S., Haerynck F., Hoste L., Leal I. A., Da Silva A. R. A., Silva A. E. A., Barchik A., Barreiro S. T. A., Cochrane N., Teixeira C. H., Arauj J. M., Ossa R. A. P. -D. L., Vieira C. S., Dimitrova A., Ganeva M., Stefanov S., Telcharova-Mihaylovska A., Biggs C. M., Scuccimarri R., Withington D., Raul B. B., Ampuero C., Aravena J., Casanova D., Cruces P., Diaz F., Garcia-Salum T., Godoy L., Medina R. A., Galaz G. V., Avila-Aguero M. L., Brenes-Chacon H., Ivankovich-Escoto G., Yock-Corrales A., Badib A., Badreldin K., Elkhashab Y., Heshmat H., Heinonen S., Angoulvant F., Belot A., Ouldali N., Beske F., Heep A., Masjosthusmann K., Reiter K., Heuvel I. V. D., Both U. V., Agrafiotou A., Antachopoulos C., Eleftheriou I., Farmaki E., Fotis L., Kafetzis D., Lampidi S., Liakopoulou T., Maritsi D., Michailidou E., Milioudi M., Mparmpounaki I., Papadimitriou E., Papaevangelou V., Roilides E., Tsiatsiou O., Tsolas G., Tsolia M., Vantsi P., Pineda L. Y. B., Aguilar K. L. B., Quintero E. M. C., Ip P., Kwan M. Y. W., Kwok J., Lau Y. L., To K., Wong J. S. C., David M., Farkas D., Kalcakosz S., Szekeres K., Zsigmond B., Aslam N., Andreozzi L., Bianco F., Bucciarelli V., Buonsenso D., Cimaz R., D'Argenio P., Dellepiane R. M., Fabi M., Mastrolia M. V., Mauro A., Mazza A., Romani L., Simonini G., Tipo V., Valentini P., Verdoni L., Reel B., Pace D., Torpiano P., Flores M. F., Dominguez M. G., Vargas A. L. G., Hernandez L. L., Figueroa R. P. M., Gaxiola G. P., Valadez J., Klevberg S., Knudsen P. K., Maseide P. H., Carrera J. M., Castano E. G., Timana C. A. D., Leon T. D., Estripeaut D., Levy J., Norero X., Record J., Rojas-Bonilla M., Iramain R., Hernandez R., Huaman G., Munaico M., Peralta C., Seminario D., Yarleque E. H. Z., Gadzinska J., Mandziuk J., Okarska-Napierala M., Alacheva Z. A., Alexeeva E., Ananin P. V., Antsupova M., Bakradze M. D., Bobkova P., Borzakova S., Chashchina I. L., Fisenko A. P., Gautier M. S., Glazyrina A., Kondrikova E., Korobyants E., Korsunskiy A. A., Kovygina K., Krasnaya E., Kurbanova S., Kurdup M. K., Mamutova A. V., Mazankova L., Mitushin I. L., Nargizyan A., Orlova Y. O., Osmanov I. M., Polyakova A. S., Romanova O., Samitova E., Sologub A., Spiridonova E., Tepaev R. F., Tkacheva A. A., Yusupova V., Zholobova E., Grasa C. D., Segura N. L., Martinon-Torres F., Melendo S., Echevarria A. M., Guzman J. M. M., Argueta J. R. P., Rivero-Calle I., Riviere J., Rodriguez-Gonzalez M., Rojo P., Manubens J. S., Soler-Palacin P., Soriano-Arandes A., Tagarro A., Villaverde S., Altman M., Brodin P., Horne A., Palmblad K., Brotschi B., Sauteur P. M., Schmid J. P., Prader S., Relly C., Schlapbach L. J., Seiler M., Truck J., Wutz D., Ketharanathan N., Vermont C., Ozkan E. A., Erdeniz E. H., Borisova G., Boychenko L., Diudenko N., Kasiyan O., Katerynych K., Melnyk K., Miagka N., Teslenko M., Trykosh M., Volokha A., Akomolafe T., Al-Abadi E., Alders N., Avram P., Bamford A., Bank M., Roy R. B., Beattie T., Boleti O., Broad J., Carrol E. D., Chandran A., Cooper H., Davies P., Emonts M., Evans C., Fidler K., Foster C., Gong C., Gongrun B., Gonzalez C., Grandjean L., Grant K., Hacohen Y., Hall J., Hassell J., Hesketh C., Hewlett J., Hnieno A., Holt-Davis H., Hossain A., Hudson L. D., Johnson M., Johnson S., Jyothish D., Kampmann B., Kavirayani A., Kelly D., Kucera F., Langer D., Lillie J., Longbottom K., Lyall H., MacKdermott N., Maltby S., McLelland T., McMahon A. -M., Miller D., Morrison Z., Mosha K., Muller J., Myttaraki E., Nadel S., Osaghae D., Osman F., Ostrzewska A., Panthula M., Papachatzi E., Papadopoulou C., Penner J., Polandi S., Prendergast A. J., Ramnarayan P., Rhys-Evans S., Riordan A., Rodrigues C. M. C., Romaine S., Seddon J., Shingadia D., Srivastava A., Struik S., Taylor A., Tran S., Tudor-Williams G., Van Der Velden F., Ventilacion L., Wellman P. A., Yanney M. P., Yeung S., Badheka A., Badran S., Bailey D. M., Burch A. K., Burns J. C., Cichon C., Cirks B., Dallman M. D., Delany D. R., Fairchok M., Friedman S., Geracht J., Langs-Barlow A., Mann K., Padhye A., Quade A., Ramirez K. A., Rockett J., Sayed I. A., Shahin A. A., Umaru S., Widener R., Angela M. H., Kandawasvika G., McArdle A.J., Vito O., Patel H., Seaby E.G., Shah P., Wilson C., Broderick C., Nijman R., Tremoulet A.H., Munblit D., Ulloa-Gutierrez R., Carter M.J., De T., Hoggart C., Whittaker E., Herberg J.A., Kaforou M., Cunnington A.J., Levin M., Vazquez J.A., Carmona R., Perez L., Rubinos M., Veliz N., Yori S., Haerynck F., Hoste L., Leal I.A., Da Silva A.R.A., Silva A.E.A., Barchik A., Barreiro S.T.A., Cochrane N., Teixeira C.H., Arauj J.M., Ossa R.A.P.-D.L., Vieira C.S., Dimitrova A., Ganeva M., Stefanov S., Telcharova-Mihaylovska A., Biggs C.M., Scuccimarri R., Withington D., Raul B.B., Ampuero C., Aravena J., Casanova D., Cruces P., Diaz F., Garcia-Salum T., Godoy L., Medina R.A., Galaz G.V., Avila-Aguero M.L., Brenes-Chacon H., Ivankovich-Escoto G., Yock-Corrales A., Badib A., Badreldin K., Elkhashab Y., Heshmat H., Heinonen S., Angoulvant F., Belot A., Ouldali N., Beske F., Heep A., Masjosthusmann K., Reiter K., Heuvel I.V.D., Both U.V., Agrafiotou A., Antachopoulos C., Eleftheriou I., Farmaki E., Fotis L., Kafetzis D., Lampidi S., Liakopoulou T., Maritsi D., Michailidou E., Milioudi M., Mparmpounaki I., Papadimitriou E., Papaevangelou V., Roilides E., Tsiatsiou O., Tsolas G., Tsolia M., Vantsi P., Pineda L.Y.B., Aguilar K.L.B., Quintero E.M.C., Ip P., Kwan M.Y.W., Kwok J., Lau Y.L., To K., Wong J.S.C., David M., Farkas D., Kalcakosz S., Szekeres K., Zsigmond B., Aslam N., Andreozzi L., Bianco F., Bucciarelli V., Buonsenso D., Cimaz R., D'Argenio P., Dellepiane R.M., Fabi M., Mastrolia M.V., Mauro A., Mazza A., Romani L., Simonini G., Tipo V., Valentini P., Verdoni L., Reel B., Pace D., Torpiano P., Flores M.F., Dominguez M.G., Vargas A.L.G., Hernandez L.L., Figueroa R.P.M., Gaxiola G.P., Valadez J., Klevberg S., Knudsen P.K., Maseide P.H., Carrera J.M., Castano E.G., Timana C.A.D., Leon T.D., Estripeaut D., Levy J., Norero X., Record J., Rojas-Bonilla M., Iramain R., Hernandez R., Huaman G., Munaico M., Peralta C., Seminario D., Yarleque E.H.Z., Gadzinska J., Mandziuk J., Okarska-Napierala M., Alacheva Z.A., Alexeeva E., Ananin P.V., Antsupova M., Bakradze M.D., Bobkova P., Borzakova S., Chashchina I.L., Fisenko A.P., Gautier M.S., Glazyrina A., Kondrikova E., Korobyants E., Korsunskiy A.A., Kovygina K., Krasnaya E., Kurbanova S., Kurdup M.K., Mamutova A.V., Mazankova L., Mitushin I.L., Nargizyan A., Orlova Y.O., Osmanov I.M., Polyakova A.S., Romanova O., Samitova E., Sologub A., Spiridonova E., Tepaev R.F., Tkacheva A.A., Yusupova V., Zholobova E., Grasa C.D., Segura N.L., Martinon-Torres F., Melendo S., Echevarria A.M., Guzman J.M.M., Argueta J.R.P., Rivero-Calle I., Riviere J., Rodriguez-Gonzalez M., Rojo P., Manubens J.S., Soler-Palacin P., Soriano-Arandes A., Tagarro A., Villaverde S., Altman M., Brodin P., Horne A., Palmblad K., Brotschi B., Sauteur P.M., Schmid J.P., Prader S., Relly C., Schlapbach L.J., Seiler M., Truck J., Wutz D., Ketharanathan N., Vermont C., Ozkan E.A., Erdeniz E.H., Borisova G., Boychenko L., Diudenko N., Kasiyan O., Katerynych K., Melnyk K., Miagka N., Teslenko M., Trykosh M., Volokha A., Akomolafe T., Al-Abadi E., Alders N., Avram P., Bamford A., Bank M., Roy R.B., Beattie T., Boleti O., Broad J., Carrol E.D., Chandran A., Cooper H., Davies P., Emonts M., Evans C., Fidler K., Foster C., Gong C., Gongrun B., Gonzalez C., Grandjean L., Grant K., Hacohen Y., Hall J., Hassell J., Hesketh C., Hewlett J., Hnieno A., Holt-Davis H., Hossain A., Hudson L.D., Johnson M., Johnson S., Jyothish D., Kampmann B., Kavirayani A., Kelly D., Kucera F., Langer D., Lillie J., Longbottom K., Lyall H., MacKdermott N., Maltby S., McLelland T., McMahon A.-M., Miller D., Morrison Z., Mosha K., Muller J., Myttaraki E., Nadel S., Osaghae D., Osman F., Ostrzewska A., Panthula M., Papachatzi E., Papadopoulou C., Penner J., Polandi S., Prendergast A.J., Ramnarayan P., Rhys-Evans S., Riordan A., Rodrigues C.M.C., Romaine S., Seddon J., Shingadia D., Srivastava A., Struik S., Taylor A., Tran S., Tudor-Williams G., Van Der Velden F., Ventilacion L., Wellman P.A., Yanney M.P., Yeung S., Badheka A., Badran S., Bailey D.M., Burch A.K., Burns J.C., Cichon C., Cirks B., Dallman M.D., Delany D.R., Fairchok M., Friedman S., Geracht J., Langs-Barlow A., Mann K., Padhye A., Quade A., Ramirez K.A., Rockett J., Sayed I.A., Shahin A.A., Umaru S., Widener R., Angela M.H., Kandawasvika G., Pediatric Surgery, Pediatrics, University of Zurich, National Institute of Health and Medical Research, Wellcome Trust, Medical Research Foundation, Shah, Priyen [0000-0001-9164-8862], Ulloa-Gutierrez, Rolando [0000-0002-9157-9227], Herberg, Jethro A [0000-0001-6941-6491], Cunnington, Aubrey J [0000-0002-1305-3529], Levin, Michael [0000-0003-2767-6919], and Apollo - University of Cambridge Repository

Subjects
Inotrope, Male, medicine.medical_treatment, 2700 General Medicine, 030204 cardiovascular system & hematology, Antibodies, Viral, Medical and Health Sciences, Cohort Studies, 0302 clinical medicine, Glucocorticoid, hemic and lymphatic diseases, Medicine and Health Sciences, 030212 general & internal medicine, Viral, Child, 11 Medical and Health Sciences, OUTCOMES, Respiration, Immunoglobulins, Intravenous, General Medicine, Systemic Inflammatory Response Syndrome, 3. Good health, Hospitalization, Treatment Outcome, Child, Preschool, Combination, Artificial, Regression Analysis, Drug Therapy, Combination, Female, Original Article, Intravenous, Life Sciences & Biomedicine, Cohort study, Human, medicine.medical_specialty, BATS Consortium, Adolescent, Immunoglobulins, 610 Medicine & health, Regression Analysi, Antibodies, Immunomodulation, 03 medical and health sciences, Medicine, General & Internal, Pharmacotherapy, Drug Therapy, Clinical Research, Internal medicine, General & Internal Medicine, medicine, MANAGEMENT, Confidence Intervals, Humans, Preschool, Propensity Score, Glucocorticoids, Mechanical ventilation, Science & Technology, business.industry, SARS-CoV-2, Inflammatory and immune system, COVID-19, Odds ratio, medicine.disease, Respiration, Artificial, Confidence interval, KAWASAKI-LIKE DISEASE, COVID-19 Drug Treatment, Systemic inflammatory response syndrome, 10036 Medical Clinic, Immunoglobulins, Intravenou, Propensity score matching, Cohort Studie, business, ACUTE RESPIRATORY SYNDROME, Confidence Interval, TOXIC-SHOCK-SYNDROME
Abstract

BackgroundEvidence is urgently needed to support treatment decisions for children with multisystem inflammatory syndrome (MIS-C) associated with severe acute respiratory syndrome coronavirus 2.MethodsWe performed an international observational cohort study of clinical and outcome data regarding suspected MIS-C that had been uploaded by physicians onto a Web-based database. We used inverse-probability weighting and generalized linear models to evaluate intravenous immune globulin (IVIG) as a reference, as compared with IVIG plus glucocorticoids and glucocorticoids alone. There were two primary outcomes: the first was a composite of inotropic support or mechanical ventilation by day 2 or later or death; the second was a reduction in disease severity on an ordinal scale by day 2. Secondary outcomes included treatment escalation and the time until a reduction in organ failure and inflammation.ResultsData were available regarding the course of treatment for 614 children from 32 countries from June 2020 through February 2021; 490 met the World Health Organization criteria for MIS-C. Of the 614 children with suspected MIS-C, 246 received primary treatment with IVIG alone, 208 with IVIG plus glucocorticoids, and 99 with glucocorticoids alone; 22 children received other treatment combinations, including biologic agents, and 39 received no immunomodulatory therapy. Receipt of inotropic or ventilatory support or death occurred in 56 patients who received IVIG plus glucocorticoids (adjusted odds ratio for the comparison with IVIG alone, 0.77; 95% confidence interval [CI], 0.33 to 1.82) and in 17 patients who received glucocorticoids alone (adjusted odds ratio, 0.54; 95% CI, 0.22 to 1.33). The adjusted odds ratios for a reduction in disease severity were similar in the two groups, as compared with IVIG alone (0.90 for IVIG plus glucocorticoids and 0.93 for glucocorticoids alone). The time until a reduction in disease severity was similar in the three groups.ConclusionsWe found no evidence that recovery from MIS-C differed after primary treatment with IVIG alone, IVIG plus glucocorticoids, or glucocorticoids alone, although significant differences may emerge as more data accrue. (Funded by the European Union's Horizon 2020 Program and others; BATS ISRCTN number, ISRCTN69546370.).

Published
2021

6. Efficacy, Immunogenicity and Safety of Vaccination in Pediatric Patients With Autoimmune Inflammatory Rheumatic Diseases (pedAIIRD): A Systematic Literature Review for the 2021 Update of the EULAR/PRES Recommendations

Author

Jansen, M.H. Rondaan, C. Legger, G. Minden, K. Uziel, Y. Toplak, N. Maritsi, D. van den Berg, M. Berbers, G. Bruijning, P. Egert, Y. Normand, C. Bijl, M. Foster, H. Kone-Paut, I. Wouters, C. Ravelli, A. Elkayam, O. Wulffraat, N.M. Heijstek, M.W. and Jansen, M.H. Rondaan, C. Legger, G. Minden, K. Uziel, Y. Toplak, N. Maritsi, D. van den Berg, M. Berbers, G. Bruijning, P. Egert, Y. Normand, C. Bijl, M. Foster, H. Kone-Paut, I. Wouters, C. Ravelli, A. Elkayam, O. Wulffraat, N.M. Heijstek, M.W.

Abstract

Background: In 2011, the first European League Against Rheumatism (EULAR) vaccination recommendations for pediatric patients with autoimmune inflammatory rheumatic diseases (pedAIIRD) were published. The past decade numerous new studies were performed to assess the safety, efficacy and immunogenicity of vaccinations in pedAIIRD. A systematic literature review (SLR) was therefore performed to serve as the basis for the updated 2021 EULAR/PRES recommendations. Methods: An SLR was performed according to the standard operating procedures for EULAR-endorsed recommendations. Primary outcomes were efficacy, immunogenicity and safety of vaccination in pedAIIRD. The search was performed in Medline, Embase and the Cochrane Library and included studies published from November 2010 until July 2020. Results: The SLR yielded 57 studies which were included for critical appraisal and data extraction. Only 8 studies described the occurrence of vaccine-preventable infections after vaccination (efficacy), none of these studies were powered to assess efficacy. The majority of studies assessed (humoral) immune responses as surrogate endpoint for vaccine efficacy. Studies on non-live vaccines showed that these were safe and in general immunogenic. Biologic disease-modifying antirheumatic drugs (bDMARDs) in general did not significantly reduce seroprotection rates, except for B-cell depleting therapies which severely hampered humoral responses. Four new studies on human papilloma virus vaccination showed that this vaccine was safe and immunogenic in pedAIIRD. Regarding live-attenuated vaccinations, level 1 evidence of the measles mumps rubella (MMR) booster vaccination became available which showed the safety of this booster for patients treated with methotrexate. In addition, level 3 evidence became available that suggested that the MMR and varicella zoster virus (VZV) vaccination for patients on low dose glucocorticosteroids and bDMARDs might be safe as well. Conclusions: The past decad

Published
2022

7. EULAR/PRES recommendations for vaccination of paediatric patients with autoimmune inflammatory rheumatic diseases: update 2021

Author

Jansen, M.H.A. Rondaan, C. Legger, G.E. Minden, K. Uziel, Y. Toplak, N. Maritsi, D. Van Den Berg, L. Berbers, G.A.M. Bruijning, P. Egert, Y. Normand, C. Bijl, M. Foster, H.E. Koné-Paut, I. Wouters, C. Ravelli, A. Elkayam, O. Wulffraat, N.M. Heijstek, M.W. and Jansen, M.H.A. Rondaan, C. Legger, G.E. Minden, K. Uziel, Y. Toplak, N. Maritsi, D. Van Den Berg, L. Berbers, G.A.M. Bruijning, P. Egert, Y. Normand, C. Bijl, M. Foster, H.E. Koné-Paut, I. Wouters, C. Ravelli, A. Elkayam, O. Wulffraat, N.M. Heijstek, M.W.

Abstract

Objectives Recent insights supporting the safety of live-attenuated vaccines and novel studies on the immunogenicity of vaccinations in the era of biological disease-modifying antirheumatic drugs in paediatric patients with autoimmune/inflammatory rheumatic diseases (pedAIIRD) necessitated updating the EULAR recommendations. Methods Recommendations were developed using the EULAR standard operating procedures. Two international expert committees were formed to update the vaccination recommendations for both paediatric and adult patients with AIIRD. After a systematic literature review, separate recommendations were formulated for paediatric and adult patients. For pedAIIRD, six overarching principles and seven recommendations were formulated and provided with the level of evidence, strength of recommendation and Task Force level of agreement. Results In general, the National Immunisation Programmes (NIP) should be followed and assessed yearly by the treating specialist. If possible, vaccinations should be administered prior to immunosuppressive drugs, but necessary treatment should never be postponed. Non-live vaccines can be safely given to immunosuppressed pedAIIRD patients. Mainly, seroprotection is preserved in patients receiving vaccinations on immunosuppression, except for high-dose glucocorticoids and B-cell depleting therapies. Live-attenuated vaccines should be avoided in immunosuppressed patients. However, it is safe to administer the measles-mumps-rubella booster and varicella zoster virus vaccine to immunosuppressed patients under specific conditions. In addition to the NIP, the non-live seasonal influenza vaccination should be strongly considered for immunosuppressed pedAIIRD patients. Conclusions These recommendations are intended for paediatricians, paediatric rheumatologists, national immunisation agencies, general practitioners, patients and national rheumatology societies to attain safe and effective vaccination and optimal infection prevention in i

Published
2022

8. Eyes wide open—an atypical presentation of Miller Fisher syndrome (MFS): case report

Author

Korona, A. Maritsi, D. Markante, A. Stamati, A. Mouskou, S. Vartzelis, G. and Korona, A. Maritsi, D. Markante, A. Stamati, A. Mouskou, S. Vartzelis, G.

Abstract

Background: Parinaud’s syndrome, also known as dorsal midbrain syndrome, is characterized by a combination of signs, including upgaze palsy, convergence–retraction nystagmus, eyelid retraction and pupillary dissociation. It is caused by pretectal or tegmental lesions of upper brainstem. Miller Fisher syndrome, characterized by the triad of ataxia, areflexia and ophthalmoplegia, has rarely been reported to present as Parinaud’s syndrome in adults. To our knowledge, this clinical manifestation has not been previously described in children. Case presentation: A previously healthy 13-year-old girl presented with blurred vision and diplopia, 10 days after a viral infection. Initial examination revealed incomplete Parinaud’s syndrome, while rest of neurological examination was normal. Brain imaging (MRI, MRA) did not reveal any abnormal findings and CSF findings were also normal. During the first days after admission she gradually deteriorated, showing complete external ophthalmoplegia, unsteady gait, and absent deep tendon reflexes of lower limbs with normal muscle power. With the clinical suspicion of Miller Fisher syndrome IVIG was administered, leading to subsequent resolution of her symptoms. AntiGQ1b and antiGD1b antibodies came back positive confirming diagnosis. On 1 month follow-up, neurological examination revealed diplopia in left gaze, and a second dose of IVIG was administered with good response. She remains asymptomatic 1 year from disease onset. Conclusions: Miller Fisher syndrome can rarely present as Parinaud’s syndrome in adults. Herein we described the first pediatric patient with similar clinical presentation. As the typical semiology of ataxia and areflexia may not be present initially, high index of suspicion is required to recognise and treat those patients promptly. Serological detection of anti-ganglioside antibodies, such as anti-GQ1b and anti-GD1b, may help confirm diagnosis. © 2022, The Author(s).

Published
2022

9. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: analysis by the Pharmachild Safety Adjudication Committee

Author

Giancane, G, Swart, J, Castagnola, E, Groll, A, Horneff, G, Huppertz, H, Lovell, D, Wolfs, T, Herlin, T, Dolezalova, P, Sanner, H, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Vargova, V, Sawhney, S, Rygg, M, K Oliveira, S, Cattalini, M, Bovis, F, Bagnasco, F, Pistorio, A, Martini, A, Wulffraat, N, Ruperto, N, Giancane G, Swart JF, Castagnola E, Groll AH, Horneff G, Huppertz HI, Lovell DJ, Wolfs T, Herlin T, Dolezalova P, Sanner H, Susic G, Sztajnbok F, Maritsi D, Constantin T, Vargova V, Sawhney S, Rygg M, K Oliveira S, Cattalini M, Bovis F, Bagnasco F, Pistorio A, Martini A, Wulffraat N, Ruperto N, Giancane, G, Swart, J, Castagnola, E, Groll, A, Horneff, G, Huppertz, H, Lovell, D, Wolfs, T, Herlin, T, Dolezalova, P, Sanner, H, Susic, G, Sztajnbok, F, Maritsi, D, Constantin, T, Vargova, V, Sawhney, S, Rygg, M, K Oliveira, S, Cattalini, M, Bovis, F, Bagnasco, F, Pistorio, A, Martini, A, Wulffraat, N, Ruperto, N, Giancane G, Swart JF, Castagnola E, Groll AH, Horneff G, Huppertz HI, Lovell DJ, Wolfs T, Herlin T, Dolezalova P, Sanner H, Susic G, Sztajnbok F, Maritsi D, Constantin T, Vargova V, Sawhney S, Rygg M, K Oliveira S, Cattalini M, Bovis F, Bagnasco F, Pistorio A, Martini A, Wulffraat N, and Ruperto N

Abstract

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. Trial registration: Clinicaltrials.gov NCT 01399281; ENCePP seal: awarded on 25 November 2011.

Published
2020

10. Treatment of Multisystem Inflammatory Syndrome in Children

Author

McArdle, A.J. Vito, O. Patel, H. Seaby, E.G. Shah, P. Wilson, C. Broderick, C. Nijman, R. Tremoulet, A.H. Munblit, D. Ulloa-Gutierrez, R. Carter, M.J. De, T. Hoggart, C. Whittaker, E. Herberg, J.A. Kaforou, M. Cunnington, A.J. Levin, M. Vazquez, J.A. Carmona, R. Perez, L. Rubinos, M. Veliz, N. Yori, S. Haerynck, F. Hoste, L. Leal, I.A. Da Silva, A.R.A. Silva, A.E.A. Barchik, A. Barreiro, S.T.A. Cochrane, N. Teixeira, C.H. Arauj, J.M. Ossa, R.A.P.-D.L. Vieira, C.S. Dimitrova, A. Ganeva, M. Stefanov, S. Telcharova-Mihaylovska, A. Biggs, C.M. Scuccimarri, R. Withington, D. Raul, B.B. Ampuero, C. Aravena, J. Casanova, D. Cruces, P. Diaz, F. Garcia-Salum, T. Godoy, L. Medina, R.A. Galaz, G.V. Avila-Aguero, M.L. Brenes-Chacon, H. Ivankovich-Escoto, G. Yock-Corrales, A. Badib, A. Badreldin, K. Elkhashab, Y. Heshmat, H. Heinonen, S. Angoulvant, F. Belot, A. Ouldali, N. Beske, F. Heep, A. Masjosthusmann, K. Reiter, K. Heuvel, I.V.D. Both, U.V. Agrafiotou, A. Antachopoulos, C. Eleftheriou, I. Farmaki, E. Fotis, L. Kafetzis, D. Lampidi, S. Liakopoulou, T. Maritsi, D. Michailidou, E. Milioudi, M. Mparmpounaki, I. Papadimitriou, E. Papaevangelou, V. Roilides, E. Tsiatsiou, O. Tsolas, G. Tsolia, M. Vantsi, P. Pineda, L.Y.B. Aguilar, K.L.B. Quintero, E.M.C. Ip, P. Kwan, M.Y.W. Kwok, J. Lau, Y.L. To, K. Wong, J.S.C. David, M. Farkas, D. Kalcakosz, S. Szekeres, K. Zsigmond, B. Aslam, N. Andreozzi, L. Bianco, F. Bucciarelli, V. Buonsenso, D. Cimaz, R. D'Argenio, P. Dellepiane, R.M. Fabi, M. Mastrolia, M.V. Mauro, A. Mazza, A. Romani, L. Simonini, G. Tipo, V. Valentini, P. Verdoni, L. Reel, B. Pace, D. Torpiano, P. Flores, M.F. Domínguez, M.G. Vargas, A.L.G. Hernandez, L.L. Figueroa, R.P.M. Gaxiola, G.P. Valadez, J. Klevberg, S. Knudsen, P.K. Maseide, P.H. Carrera, J.M. Castano, E.G. Timana, C.A.D. Leon, T.D. Estripeaut, D. Levy, J. Norero, X. Record, J. Rojas-Bonilla, M. Iramain, R. Hernandez, R. Huaman, G. Munaico, M. Peralta, C. Seminario, D. Yarleque, E.H.Z. Gadzinska, J. Mandziuk, J. Okarska-Napierała, M. Alacheva, Z.A. Alexeeva, E. Ananin, P.V. Antsupova, M. Bakradze, M.D. Bobkova, P. Borzakova, S. Chashchina, I.L. Fisenko, A.P. Gautier, M.S. Glazyrina, A. Kondrikova, E. Korobyants, E. Korsunskiy, A.A. Kovygina, K. Krasnaya, E. Kurbanova, S. Kurdup, M.K. Mamutova, A.V. Mazankova, L. Mitushin, I.L. Nargizyan, A. Orlova, Y.O. Osmanov, I.M. Polyakova, A.S. Romanova, O. Samitova, E. Sologub, A. Spiridonova, E. Tepaev, R.F. Tkacheva, A.A. Yusupova, V. Zholobova, E. Grasa, C.D. Segura, N.L. Martinon-Torres, F. Melendo, S. Echevarria, A.M. Guzman, J.M.M. Argueta, J.R.P. Rivero-Calle, I. Riviere, J. Rodriguez-Gonzalez, M. Rojo, P. Manubens, J.S. Soler-Palacin, P. Soriano-Arandes, A. Tagarro, A. Villaverde, S. Altman, M. Brodin, P. Horne, A. Palmblad, K. Brotschi, B. Sauteur, P.M. Schmid, J.P. Prader, S. Relly, C. Schlapbach, L.J. Seiler, M. Truck, J. Wutz, D. Ketharanathan, N. Vermont, C. Ozkan, E.A. Erdeniz, E.H. Borisova, G. Boychenko, L. Diudenko, N. Kasiyan, O. Katerynych, K. Melnyk, K. Miagka, N. Teslenko, M. Trykosh, M. Volokha, A. Akomolafe, T. Al-Abadi, E. Alders, N. Avram, P. Bamford, A. Bank, M. Roy, R.B. Beattie, T. Boleti, O. Broad, J. Carrol, E.D. Chandran, A. Cooper, H. Davies, P. Emonts, M. Evans, C. Fidler, K. Foster, C. Gong, C. Gongrun, B. Gonzalez, C. Grandjean, L. Grant, K. Hacohen, Y. Hall, J. Hassell, J. Hesketh, C. Hewlett, J. Hnieno, A. Holt-Davis, H. Hossain, A. Hudson, L.D. Johnson, M. Johnson, S. Jyothish, D. Kampmann, B. Kavirayani, A. Kelly, D. Kucera, F. Langer, D. Lillie, J. Longbottom, K. Lyall, H. MacKdermott, N. Maltby, S. McLelland, T. McMahon, A.-M. Miller, D. Morrison, Z. Mosha, K. Muller, J. Myttaraki, E. Nadel, S. Osaghae, D. Osman, F. Ostrzewska, A. Panthula, M. Papachatzi, E. Papadopoulou, C. Penner, J. Polandi, S. Prendergast, A.J. Ramnarayan, P. Rhys-Evans, S. Riordan, A. Rodrigues, C.M.C. Romaine, S. Seddon, J. Shingadia, D. Srivastava, A. Struik, S. Taylor, A. Taylor, A. Taylor, A. Tran, S. Tudor-Williams, G. Van Der Velden, F. Ventilacion, L. Wellman, P.A. Yanney, M.P. Yeung, S. Badheka, A. Badran, S. Bailey, D.M. Burch, A.K. Burns, J.C. Cichon, C. Cirks, B. Dallman, M.D. Delany, D.R. Fairchok, M. Friedman, S. Geracht, J. Langs-Barlow, A. Mann, K. Padhye, A. Quade, A. Ramirez, K.A. Rockett, J. Sayed, I.A. Shahin, A.A. Umaru, S. Widener, R. Angela, M.H. Kandawasvika, G. BATS Consortium

Subjects
hemic and lymphatic diseases
Abstract

BACKGROUND Evidence is urgently needed to support treatment decisions for children with multisystem inflammatory syndrome (MIS-C) associated with severe acute respiratory syndrome coronavirus 2. METHODS We performed an international observational cohort study of clinical and outcome data regarding suspected MIS-C that had been uploaded by physicians onto a Web-based database. We used inverse-probability weighting and generalized linear models to evaluate intravenous immune globulin (IVIG) as a reference, as compared with IVIG plus glucocorticoids and glucocorticoids alone. There were two primary outcomes: the first was a composite of inotropic support or mechanical ventilation by day 2 or later or death; the second was a reduction in disease severity on an ordinal scale by day 2. Secondary outcomes included treatment escalation and the time until a reduction in organ failure and inflammation. RESULTS Data were available regarding the course of treatment for 614 children from 32 countries from June 2020 through February 2021; 490 met the World Health Organization criteria for MIS-C. Of the 614 children with suspected MIS-C, 246 received primary treatment with IVIG alone, 208 with IVIG plus glucocorticoids, and 99 with glucocorticoids alone; 22 children received other treatment combinations, including biologic agents, and 39 received no immunomodulatory therapy. Receipt of inotropic or ventilatory support or death occurred in 56 patients who received IVIG plus glucocorticoids (adjusted odds ratio for the comparison with IVIG alone, 0.77; 95% confidence interval [CI], 0.33 to 1.82) and in 17 patients who received glucocorticoids alone (adjusted odds ratio, 0.54; 95% CI, 0.22 to 1.33). The adjusted odds ratios for a reduction in disease severity were similar in the two groups, as compared with IVIG alone (0.90 for IVIG plus glucocorticoids and 0.93 for glucocorticoids alone). The time until a reduction in disease severity was similar in the three groups. CONCLUSIONS We found no evidence that recovery from MIS-C differed after primary treatment with IVIG alone, IVIG plus glucocorticoids, or glucocorticoids alone, although significant differences may emerge as more data accrue. Copyright © 2021 Massachusetts Medical Society.

Published
2021

11. Pericarditis as the main clinical manifestation of covid-19 in adolescents

Author

Dimopoulou, D. Spyridis, N. Dasoula, F. Krepis, P. Eleftheriou, E. Liaska, M. Servos, G. Maritsi, D. Tsolia, M.

Abstract

Children and adolescents with severe acute respiratory syndrome coronavirus 2 infection usually have a milder illness, lower mortality rates and may manifest different clinical entities compared with adults. Acute effusive pericarditis is a rare clinical manifestation in patients with COVID-19, especially among those without concurrent pulmonary disease or myocardial injury. We present 2 cases of acute pericarditis, in the absence of initial respiratory or other symptoms, in adolescents with COVID-19. Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.

Published
2021

13. Treatment of Multisystem Inflammatory Syndrome in Children

Author

McArdle, A.J. Vito, O. Patel, H. Seaby, E.G. Shah, P. Wilson, C. Broderick, C. Nijman, R. Tremoulet, A.H. Munblit, D. Ulloa-Gutierrez, R. Carter, M.J. De, T. Hoggart, C. Whittaker, E. Herberg, J.A. Kaforou, M. Cunnington, A.J. Levin, M. Vazquez, J.A. Carmona, R. Perez, L. Rubinos, M. Veliz, N. Yori, S. Haerynck, F. Hoste, L. Leal, I.A. Da Silva, A.R.A. Silva, A.E.A. Barchik, A. Barreiro, S.T.A. Cochrane, N. Teixeira, C.H. Arauj, J.M. Ossa, R.A.P.-D.L. Vieira, C.S. Dimitrova, A. Ganeva, M. Stefanov, S. Telcharova-Mihaylovska, A. Biggs, C.M. Scuccimarri, R. Withington, D. Raul, B.B. Ampuero, C. Aravena, J. Casanova, D. Cruces, P. Diaz, F. Garcia-Salum, T. Godoy, L. Medina, R.A. Galaz, G.V. Avila-Aguero, M.L. Brenes-Chacon, H. Ivankovich-Escoto, G. Yock-Corrales, A. Badib, A. Badreldin, K. Elkhashab, Y. Heshmat, H. Heinonen, S. Angoulvant, F. Belot, A. Ouldali, N. Beske, F. Heep, A. Masjosthusmann, K. Reiter, K. Heuvel, I.V.D. Both, U.V. Agrafiotou, A. Antachopoulos, C. Eleftheriou, I. Farmaki, E. Fotis, L. Kafetzis, D. Lampidi, S. Liakopoulou, T. Maritsi, D. Michailidou, E. Milioudi, M. Mparmpounaki, I. Papadimitriou, E. Papaevangelou, V. Roilides, E. Tsiatsiou, O. Tsolas, G. Tsolia, M. Vantsi, P. Pineda, L.Y.B. Aguilar, K.L.B. Quintero, E.M.C. Ip, P. Kwan, M.Y.W. Kwok, J. Lau, Y.L. To, K. Wong, J.S.C. David, M. Farkas, D. Kalcakosz, S. Szekeres, K. Zsigmond, B. Aslam, N. Andreozzi, L. Bianco, F. Bucciarelli, V. Buonsenso, D. Cimaz, R. D'Argenio, P. Dellepiane, R.M. Fabi, M. Mastrolia, M.V. Mauro, A. Mazza, A. Romani, L. Simonini, G. Tipo, V. Valentini, P. Verdoni, L. Reel, B. Pace, D. Torpiano, P. Flores, M.F. Domínguez, M.G. Vargas, A.L.G. Hernandez, L.L. Figueroa, R.P.M. Gaxiola, G.P. Valadez, J. Klevberg, S. Knudsen, P.K. Maseide, P.H. Carrera, J.M. Castano, E.G. Timana, C.A.D. Leon, T.D. Estripeaut, D. Levy, J. Norero, X. Record, J. Rojas-Bonilla, M. Iramain, R. Hernandez, R. Huaman, G. Munaico, M. Peralta, C. Seminario, D. Yarleque, E.H.Z. Gadzinska, J. Man and McArdle, A.J. Vito, O. Patel, H. Seaby, E.G. Shah, P. Wilson, C. Broderick, C. Nijman, R. Tremoulet, A.H. Munblit, D. Ulloa-Gutierrez, R. Carter, M.J. De, T. Hoggart, C. Whittaker, E. Herberg, J.A. Kaforou, M. Cunnington, A.J. Levin, M. Vazquez, J.A. Carmona, R. Perez, L. Rubinos, M. Veliz, N. Yori, S. Haerynck, F. Hoste, L. Leal, I.A. Da Silva, A.R.A. Silva, A.E.A. Barchik, A. Barreiro, S.T.A. Cochrane, N. Teixeira, C.H. Arauj, J.M. Ossa, R.A.P.-D.L. Vieira, C.S. Dimitrova, A. Ganeva, M. Stefanov, S. Telcharova-Mihaylovska, A. Biggs, C.M. Scuccimarri, R. Withington, D. Raul, B.B. Ampuero, C. Aravena, J. Casanova, D. Cruces, P. Diaz, F. Garcia-Salum, T. Godoy, L. Medina, R.A. Galaz, G.V. Avila-Aguero, M.L. Brenes-Chacon, H. Ivankovich-Escoto, G. Yock-Corrales, A. Badib, A. Badreldin, K. Elkhashab, Y. Heshmat, H. Heinonen, S. Angoulvant, F. Belot, A. Ouldali, N. Beske, F. Heep, A. Masjosthusmann, K. Reiter, K. Heuvel, I.V.D. Both, U.V. Agrafiotou, A. Antachopoulos, C. Eleftheriou, I. Farmaki, E. Fotis, L. Kafetzis, D. Lampidi, S. Liakopoulou, T. Maritsi, D. Michailidou, E. Milioudi, M. Mparmpounaki, I. Papadimitriou, E. Papaevangelou, V. Roilides, E. Tsiatsiou, O. Tsolas, G. Tsolia, M. Vantsi, P. Pineda, L.Y.B. Aguilar, K.L.B. Quintero, E.M.C. Ip, P. Kwan, M.Y.W. Kwok, J. Lau, Y.L. To, K. Wong, J.S.C. David, M. Farkas, D. Kalcakosz, S. Szekeres, K. Zsigmond, B. Aslam, N. Andreozzi, L. Bianco, F. Bucciarelli, V. Buonsenso, D. Cimaz, R. D'Argenio, P. Dellepiane, R.M. Fabi, M. Mastrolia, M.V. Mauro, A. Mazza, A. Romani, L. Simonini, G. Tipo, V. Valentini, P. Verdoni, L. Reel, B. Pace, D. Torpiano, P. Flores, M.F. Domínguez, M.G. Vargas, A.L.G. Hernandez, L.L. Figueroa, R.P.M. Gaxiola, G.P. Valadez, J. Klevberg, S. Knudsen, P.K. Maseide, P.H. Carrera, J.M. Castano, E.G. Timana, C.A.D. Leon, T.D. Estripeaut, D. Levy, J. Norero, X. Record, J. Rojas-Bonilla, M. Iramain, R. Hernandez, R. Huaman, G. Munaico, M. Peralta, C. Seminario, D. Yarleque, E.H.Z. Gadzinska, J. Man

Abstract

BACKGROUND Evidence is urgently needed to support treatment decisions for children with multisystem inflammatory syndrome (MIS-C) associated with severe acute respiratory syndrome coronavirus 2. METHODS We performed an international observational cohort study of clinical and outcome data regarding suspected MIS-C that had been uploaded by physicians onto a Web-based database. We used inverse-probability weighting and generalized linear models to evaluate intravenous immune globulin (IVIG) as a reference, as compared with IVIG plus glucocorticoids and glucocorticoids alone. There were two primary outcomes: the first was a composite of inotropic support or mechanical ventilation by day 2 or later or death; the second was a reduction in disease severity on an ordinal scale by day 2. Secondary outcomes included treatment escalation and the time until a reduction in organ failure and inflammation. RESULTS Data were available regarding the course of treatment for 614 children from 32 countries from June 2020 through February 2021; 490 met the World Health Organization criteria for MIS-C. Of the 614 children with suspected MIS-C, 246 received primary treatment with IVIG alone, 208 with IVIG plus glucocorticoids, and 99 with glucocorticoids alone; 22 children received other treatment combinations, including biologic agents, and 39 received no immunomodulatory therapy. Receipt of inotropic or ventilatory support or death occurred in 56 patients who received IVIG plus glucocorticoids (adjusted odds ratio for the comparison with IVIG alone, 0.77; 95% confidence interval [CI], 0.33 to 1.82) and in 17 patients who received glucocorticoids alone (adjusted odds ratio, 0.54; 95% CI, 0.22 to 1.33). The adjusted odds ratios for a reduction in disease severity were similar in the two groups, as compared with IVIG alone (0.90 for IVIG plus glucocorticoids and 0.93 for glucocorticoids alone). The time until a reduction in disease severity was similar in the three groups. CONCLUSIONS We

Published
2021

15. Live attenuated MMR/V booster vaccines in children with rheumatic diseases on immunosuppressive therapy are safe: Multicenter, retrospective data collection

Author

Uziel, Y. Moshe, V. Onozo, B. Kulcsár, A. Tróbert-Sipos, D. Akikusa, J.D. Salviato Pileggi, G. Maritsi, D. Kasapcopur, O. Rodrigues, M. Smerla, R. Rigante, D. Makay, B. Atsali, E. Wulffraat, N. Toplak, N. for the PReS working party of Vaccination Study Group

Abstract

Purpose: To collect retrospective data of patients with Juvenile Idiopathic Arthritis (JIA) and other rheumatic diseases who received live attenuated booster measles-mumps-rubella (MMR) or measles-mumps-rubella-varicella (MMR/V) during treatment with immunosuppressive therapy. Results: Data from 13 pediatric rheumatology centers in 10 countries, including 234 patients, were collected. Mean age at diagnosis was 5 ± 2.7 years, 67% were girls. Among them, 211 (90.2%) had JIA and 110 (47%) were in remission on medication. Disease activity was low in 37%, high in 8%, and moderate in 8%. One hundred-twenty-four received MMR/V booster while on methotrexate (MTX); 3 reported local mild adverse events (AE). Among 62 on MTX + biologics and 9 patients who received a combination of 2 disease modifying antirheumatic drugs (DMARDs), 9 reported mild AE. Among 39 on biologics, 1 reported fever one day after booster vaccination. No vaccine-related infection of measles, rubella, mumps or varicella was reported, none of the patients developed disease flare, including those with high disease activity. Conclusions: In this retrospective study, live-attenuated MMR/V booster vaccines were safe for children with rheumatic diseases, on immunosuppressive therapies. This strengthens the Paediatric Rheumatology European Society (PReS) recommendation that vaccination with live attenuated vaccines in patients on immunosuppressive therapies can be considered individually, weighing the benefit of vaccination against the risk of inducing infection through vaccination. These data provide the basis for a prospective data collection study, planned by the PReS vaccination study group. © 2020 Elsevier Ltd

Published
2020

16. Aldosterone synthase deficiency type II: An unusual presentation of the first Greek case reported with confirmed genetic analysis

Author

Papailiou, S. Vlachopapadopoulou, E.A. Sertedaki, A. Maritsi, D. Syggelos, N. Syggelou, A.

Abstract

Objective. Aldosterone synthase deficiency (ASD) is a rare, autosomal recessive inherited disease with an overall clinical phenotype of failure to thrive, vomiting, severe dehydration, hyperkalemia, and hyponatremia. Mutations in the CYP11B2 gene encoding aldosterone synthase are responsible for the occurrence of ASD. Defects in CYP11B2 gene have only been reported in a limited number of cases worldwide. Due to this potential life-threatening risk, comprehensive hormonal investigation followed by genetic confirmation is essential for the clinical management of offsprings. Case presentation. We herein describe an unusual case of ASD type II in a neonate with faltering growth as a single presenting symptom. To our knowledge, this is the first Greek case of ASD type II reported with confirmed genetic analysis. Next generation sequencing of her DNA revealed the homozygous mutation p.T185I (ACC-ATC) (c.554C>T) (g.7757C>T) in exon 3 of the CYP11B2 gene in the neonate, inherited from both parents who were heterozygotes for the mutation. Conclusions. Physicians handling neonates with faltering growth, particularly in the initial six weeks of life, should be suspicious of mineralocorticoid insufficiency either as isolated hypoaldosteronism or in the context of congenital adrenal hyperplasia. Essential investigations should be performed and appropriate treatment should be administered promptly without awaiting for the hormonal profile results. Interpretation of the clinical picture and the hormonal profile will guide the analysis of candidate genes. Primary selective hypoaldosteronism is a rare, life threatening disease, but still with an unknown overall population impact. Thus, reporting cases with confirmed gene mutations is of major importance. © 2020 Stayroula Papailiou et al., published by Sciendo 2020.

Published
2020

17. BCG vaccine protection against TB infection among children older than 5 years in close contact with an infectious adult TB case

Author

Syggelou, A. Spyridis, N. Benetatou, K. Kourkouni, E. Kourlaba, G. Tsagaraki, M. Maritsi, D. Eleftheriou, I. Tsolia, M. and Syggelou, A. Spyridis, N. Benetatou, K. Kourkouni, E. Kourlaba, G. Tsagaraki, M. Maritsi, D. Eleftheriou, I. Tsolia, M.

Abstract

The Bacille Calmette–Guérin (BCG) vaccine has been shown to provide considerable protection against miliary or meningeal tuberculosis (TB), but whether it prevents other forms of disease remains controversial. Recent evidence has shown that the BCG vaccine also provides protection against latent TB infection (LTBI). The aim of the current study was to examine whether BCG has a protective role against LTBI among children in close contact with an adult index case in a low TB endemicity setting with the use of the QuantiFERON-TB Gold In-Tube test (QFT-GIT). A cross-sectional study was conducted over a 10-year period among children referred to our outpatient TB clinic with a history of close contact with an adult with pulmonary TB. All subjects had a QFT-GIT performed. In total, 207 children > 5 to 16 years of age with known recent exposure were enrolled. BCG-vaccinated subjects had a 59% lower risk of presenting with LTBI after close contact with an adult index case compared with unvaccinated subjects (OR = 0.41, 95% CI: 0.23– 0.73, p = 0.002). After adjustment for possible confounders, the protective effect of prior BCG immunization was estimated at 68% (OR = 0.32, 95% CI: 0.15–0.66, p = 0.002). Other risk factors for LTBI included a history of migration (OR = 2.27, 95% CI: 1.13–4.53, p = 0.021) and transmission of infection to other exposed child contacts (OR = 4.62, 95% CI: 2.27–9.39, p = 0.001). We were able to determine a strong protective role of BCG vaccination among children older than 5 years, immunized at school entry, who had close contact with an adult infectious TB case. © 2020 by the authors. Licensee MDPI, Basel, Switzerland.

Published
2020

18. Opportunistic infections in immunosuppressed patients with juvenile idiopathic arthritis: Analysis by the Pharmachild Safety Adjudication Committee

Author

Giancane, G. Swart, J.F. Castagnola, E. Groll, A.H. Horneff, G. Huppertz, H.-I. Lovell, D.J. Wolfs, T. Herlin, T. Dolezalova, P. Sanner, H. Susic, G. Sztajnbok, F. Maritsi, D. Constantin, T. Vargova, V. Sawhney, S. Rygg, M. Oliveira, S.K. Cattalini, M. Bovis, F. Bagnasco, F. Pistorio, A. Martini, A. Wulffraat, N. Ruperto, N. Cuttica, R. Garay, S.M. Brunner, J. Emminger, W. Appenzeller, S. Len, C. Saad Magalhaes, C. Telcharova-Mihaylovska, A. Harjacek, M. Jelusic, M. Estmann, A. Nielsen, S. Herrera Mora, C. Gervais, E. Koné-Paut, I. Quartier, P. Foeldvari, I. Horneff, G. Lutz, T. Minden, K. Tzaribachev, N. Trachana, M. Tsitsami, E. Vougiouka, O. Orban, I. Harel, L. Hashkes, P. Uziel, Y. Cimaz, R. Civino, A. Consolini, R. D'Angelo, G. De Benedetti, F. Filocamo, G. Fueri, E. Gallizzi, R. Maggio, M.C. Magnolia, M.G. Miniaci, A. Montin, D. Olivieri, A.N. Pastore, S. Rigante, D. Zulian, F. Rumba-Rozenfelde, I. Stanevicha, V. Panaviene, V. Rodriguez Lozano, A.L. Rubio-Perez, N. Vega Cornejo, G. Hoppenreijs, E. Kamphuis, S. Flato, B. Nordal, E.B. Abdwani, R. Miraval, T. Paz Gastanaga, M.E. Smolewska, E. Ailioaie, C. Cochino, A.-V. Laday, M. Lazar, C. Alexeeva, E. Chasnyk, V. Keltsev, V. Suwairi, W.M.S. Vijatov-Djuric, G. Vojinovic, J. Arkachaisri, T. Koskova, E. Avcin, T. Ally, M. Van Rensburg, C.J. Louw, I. Lopez, J.A. Boteanu, A.L. Calvo Penades, I. De Inocencio, J. Mesa-Del-Castillo, P. Moreno, E. Remesal, A. Hofer, M. Gok, F. Ozen, S. Ramanan, A. Pallotti, C. Villa, L. and Giancane, G. Swart, J.F. Castagnola, E. Groll, A.H. Horneff, G. Huppertz, H.-I. Lovell, D.J. Wolfs, T. Herlin, T. Dolezalova, P. Sanner, H. Susic, G. Sztajnbok, F. Maritsi, D. Constantin, T. Vargova, V. Sawhney, S. Rygg, M. Oliveira, S.K. Cattalini, M. Bovis, F. Bagnasco, F. Pistorio, A. Martini, A. Wulffraat, N. Ruperto, N. Cuttica, R. Garay, S.M. Brunner, J. Emminger, W. Appenzeller, S. Len, C. Saad Magalhaes, C. Telcharova-Mihaylovska, A. Harjacek, M. Jelusic, M. Estmann, A. Nielsen, S. Herrera Mora, C. Gervais, E. Koné-Paut, I. Quartier, P. Foeldvari, I. Horneff, G. Lutz, T. Minden, K. Tzaribachev, N. Trachana, M. Tsitsami, E. Vougiouka, O. Orban, I. Harel, L. Hashkes, P. Uziel, Y. Cimaz, R. Civino, A. Consolini, R. D'Angelo, G. De Benedetti, F. Filocamo, G. Fueri, E. Gallizzi, R. Maggio, M.C. Magnolia, M.G. Miniaci, A. Montin, D. Olivieri, A.N. Pastore, S. Rigante, D. Zulian, F. Rumba-Rozenfelde, I. Stanevicha, V. Panaviene, V. Rodriguez Lozano, A.L. Rubio-Perez, N. Vega Cornejo, G. Hoppenreijs, E. Kamphuis, S. Flato, B. Nordal, E.B. Abdwani, R. Miraval, T. Paz Gastanaga, M.E. Smolewska, E. Ailioaie, C. Cochino, A.-V. Laday, M. Lazar, C. Alexeeva, E. Chasnyk, V. Keltsev, V. Suwairi, W.M.S. Vijatov-Djuric, G. Vojinovic, J. Arkachaisri, T. Koskova, E. Avcin, T. Ally, M. Van Rensburg, C.J. Louw, I. Lopez, J.A. Boteanu, A.L. Calvo Penades, I. De Inocencio, J. Mesa-Del-Castillo, P. Moreno, E. Remesal, A. Hofer, M. Gok, F. Ozen, S. Ramanan, A. Pallotti, C. Villa, L.

Abstract

Background: To derive a list of opportunistic infections (OI) through the analysis of the juvenile idiopathic arthritis (JIA) patients in the Pharmachild registry by an independent Safety Adjudication Committee (SAC). Methods: The SAC (3 pediatric rheumatologists and 2 pediatric infectious disease specialists) elaborated and approved by consensus a provisional list of OI for use in JIA. Through a 5 step-procedure, all the severe and serious infections, classified as per MedDRA dictionary and retrieved in the Pharmachild registry, were evaluated by the SAC by answering six questions and adjudicated with the agreement of 3/5 specialists. A final evidence-based list of OI resulted by matching the adjudicated infections with the provisional list of OI. Results: A total of 772 infectious events in 572 eligible patients, of which 335 serious/severe/very severe non-OI and 437 OI (any intensity/severity), according to the provisional list, were retrieved. Six hundred eighty-two of 772 (88.3%) were adjudicated as infections, of them 603/682 (88.4%) as common and 119/682 (17.4%) as OI by the SAC. Matching these 119 opportunistic events with the provisional list, 106 were confirmed by the SAC as OI, and among them infections by herpes viruses were the most frequent (68%), followed by tuberculosis (27.4%). The remaining events were divided in the groups of non-OI and possible/patient and/or pathogen-related OI. Conclusions: We found a significant number of OI in JIA patients on immunosuppressive therapy. The proposed list of OI, created by consensus and validated in the Pharmachild cohort, could facilitate comparison among future pharmacovigilance studies. Trial registration: Clinicaltrials.gov NCT 01399281; ENCePP seal: awarded on 25 November 2011. © 2020 The Author(s).

Published
2020

19. Live attenuated MMR/V booster vaccines in children with rheumatic diseases on immunosuppressive therapy are safe: Multicenter, retrospective data collection

Author

Uziel, Y, Moshe, V, Onozo, B, Kulcsár, A, Tróbert-Sipos, D, Akikusa, Jd, Salviato Pileggi, G, Maritsi, D, Kasapcopur, O, Rodrigues, M, Smerla, R, Rigante, Donato, Makay, B, Atsali, E, Wulffraat, N, Toplak, N, Rigante D (ORCID:0000-0001-7032-7779), Uziel, Y, Moshe, V, Onozo, B, Kulcsár, A, Tróbert-Sipos, D, Akikusa, Jd, Salviato Pileggi, G, Maritsi, D, Kasapcopur, O, Rodrigues, M, Smerla, R, Rigante, Donato, Makay, B, Atsali, E, Wulffraat, N, Toplak, N, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Purpose: To collect retrospective data of patients with Juvenile Idiopathic Arthritis (JIA) and other rheumatic diseases who received live attenuated booster measles-mumps-rubella (MMR) or measles-mumps-rubellavaricella (MMR/V) during treatment with immunosuppressive therapy. Results: Data from 13 pediatric rheumatology centers in 10 countries, including 234 patients, were collected. Mean age at diagnosis was 5 ± 2.7 years, 67% were girls. Among them, 211 (90.2%) had JIA and 110 (47%) were in remission on medication. Disease activity was low in 37%, high in 8%, and moderate in 8%. One hundred-twenty-four receivedMMR/V booster while on methotrexate (MTX); 3 reported local mild adverse events (AE). Among 62 on MTX + biologics and 9 patients who received a combination of 2 disease modifying antirheumatic drugs (DMARDs), 9 reported mild AE. Among 39 on biologics, 1 reported fever one day after booster vaccination. No vaccine-related infection of measles, rubella, mumps or varicella was reported, none of the patients developed disease flare, including those with high disease activity. Conclusions: In this retrospective study, live-attenuatedMMR/V booster vaccines were safe for children with rheumatic diseases, on immunosuppressive therapies. This strengthens the Paediatric Rheumatology European Society (PReS) recommendation that vaccination with live attenuated vaccines in patients on immunosuppressive therapies can be considered individually, weighing the benefit of vaccination against the risk of inducing infection through vaccination. These data provide the basis for a prospective data collection study, planned by the PReS vaccination study group.

Published
2020

20. A novel de novo KCNQ2 mutation in a child with treatment-resistant early-onset epileptic encephalopathy

Author

Benetou, C. Papailiou, S. Maritsi, D. Anagnostopoulou, K. Kontos, H. Vartzelis, G.

Abstract

Mutations in KCNQ2 gene, encoding for voltage-gated K+ channel subunit, may result in a wide spectrum of early-onset epileptic disorders. The phenotype of the disease varies from “benign familial neonatal seizures” to “severe epileptic encephalopathies”. In this report, we present a novel mutation [namely: c.683A>G (p.His228Arg)], as a presumable cause of severe infantile-onset neonatal seizures, in a 3-month old boy. The seizures have been poorly responsive to various pharmacological treatments, with phenytoin and carbamazepine presenting with the most favourable results so far. The study of our patient could help to further clarify the clinical manifestations of KCNQ2 mutations, revealing a previously unreported mutation. © 2019, Turkish Journal of Pediatrics. All rights reserved.

Published
2019

22. OP0058 DEVELOPMENT OF INFLAMMATORY BOWEL DISEASE DURING TREATMENT WITH ETANERCEPT IN PATIENTSWITH JUVENILE IDIOPATHIC ARTHRITIS

Author

Krol, Roline, primary, Swart, Joost F., additional, Giancane, Gabriella, additional, Roock, Sytze De, additional, Herlin, Troels, additional, Dolezalova, Pavla, additional, Sanner, Helga, additional, Susic, Gordana, additional, Sztajnbok, Flávio R., additional, Maritsi, D, additional, Constantin, Tamas, additional, Vargova, V, additional, Sawhney, Sujata, additional, Rygg, Marite, additional, Oliveira, Sheila Knupp D.E., additional, Cattalini, Marco, additional, Nordal, Ellen, additional, Magalhaes, Claudia, additional, Martini, Alberto, additional, Wulffraat, Nico, additional, and Ruperto, Nicolino, additional

Published
2019
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24. Effect of Biologic Therapy on Clinical and Laboratory Features of Macrophage Activation Syndrome Associated With Systemic Juvenile Idiopathic Arthritis

Author

Schulert, G.S. Minoia, F. Bohnsack, J. Cron, R.Q. Hashad, S. KonÉ-Paut, I. Kostik, M. Lovell, D. Maritsi, D. Nigrovic, P.A. Pal, P. Ravelli, A. Shimizu, M. Stanevicha, V. Vastert, S. Woerner, A. de Benedetti, F. Grom, A.A.

Subjects
musculoskeletal diseases
Abstract

Objective: To assess performance of the 2016 macrophage activation syndrome (MAS) classification criteria for patients with systemic juvenile idiopathic arthritis (JIA) who develop MAS while treated with biologic medications. Methods: A systematic literature review was performed to identify patients with MAS while being treated with interleukin (IL)-1 and IL-6 blocking agents. Clinical and laboratory information was compared to a large previously compiled historical cohort. Results: Eighteen publications were identified, and after removing duplicates, 35 patients treated with canakinumab and 49 patients with tocilizumab were available for analysis; 5 anakinra-treated patients were excluded due to limited numbers. MAS classification criteria were less likely to classify tocilizumab-treated patients as having MAS compared to the historical cohort or canakinumab-treated patients (56.7%, 78.5%, and 84%, respectively; P < 0.01). Patients who developed MAS while treated with canakinumab trended towards lower ferritin at MAS onset than the historical cohort (4,050 versus 5,353 ng/ml; P = 0.18) but had no differences in other cardinal clinical or laboratory features. In comparison, patients who developed MAS while treated with tocilizumab were less likely febrile and had notably lower ferritin levels (1,152 versus 5,353 ng/ml; P < 0.001). Other features of MAS were more pronounced in patients treated with tocilizumab, including lower platelet counts, lower fibrinogen, and higher aspartate aminotransferase levels. Mortality rates for patients with MAS treated with tocilizumab or canakinumab were not significantly different from the historical cohort. Conclusion: These findings show substantial alterations in MAS features that may limit utility of defined criteria for diagnosis of systemic JIA patients treated with biologic agents. © 2017, American College of Rheumatology

Published
2018

25. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Author

Ravelli, A, Minoia, F, Davì, S, Horne, A, Bovis, F, Pistorio, A, Aricò, M, Avcin, T, Behrens, Em, De Benedetti, F, Filipovic, A, Grom, Aa, Henter, J-i, Ilowite, Nte, Jordan, Mb, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, Dj, Miettunen, P, Nichols, Ke, Ozen, S, Schmid, Jp, Ramanan, Av, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, Hi, Martini, A, Ruperto, N, Cron, Rq, Angioloni, S, Pallotti, C, Pesce, M, Rinaldi, M, Villa, L, Abinun, M, Aggarwal, A, Akikusa, J, Al-mayouf, Sm, Alessio, M, Anton, J, Apaz, Mt, Astigarraga, I, Ayaz, Na, Barone, P, Bica, B, Bolt, I, Breda, L, Chasnyk, V, Cimaz, R, Corona, F, Cuttica, R, D'Angelo, G, Davidsone, Z, De Cunto, C, De Inocencio, J, Eisenstein, E, Enciso, S, Espada, G, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, Ml, Gao, Y-j, Griffin, T, Hashad, S, Hennon, T, Horneff, G, Huasong, Z, Huber, A, Insalaco, A, Ioseliani, M, Jelusic-drazic, M, Jeng, M, Kapovic, A, Kasapcopur, O, Kone-paut, I, De Oliveira Skf, Lattanzi, B, Lepore, L, Li, C, Lipton, Jm, Magni-manzoni, S, Maritsi, D, Mccurdy, D, Merino, R, Mulaosmanovic, V, Nielsen, S, Pal, P, Prahalad, S, Rigante, Donato, Rumba-rozenfelde, I, Magalhaes, Cs, Sanner, H, Sawhney, S, Sewairi, Wm, Shakoory, B, Shenoi, S, Clovis, As, Stanevicha, V, Stine, Kc, Susic, G, Sztajnbok, F, Takei, S, Tezer, H, Trauzeddel, R, Tsitsami, E, Unsal, E, Vougiouka, O, Weaver, Lk, Weiss, J, Weitzman, S, On Behalf Of The Pediatric Rheumatology International Trials Organization, Zletni M., The Childhood Arthritis & Rheumatology Research Alliance, The Pediatric Rheumatology Collaborative Study Group And The Histiocyte Society, Ravelli, A., Minoia, F., Davi, S., Horne, A., Bovis, F., Pistorio, A., Arico, M., Avcin, T., Behrens, E. M., De Benedetti, F., Filipovic, A., Grom, A. A., Henter, J. -I., Ilowite, N. T., Jordan, M. B., Khubchandani, R., Kitoh, T., Lehmberg, K., Lovell, D. J., Miettunen, P., Nichols, K. E., Ozen, S., Schmid, J. P., Ramanan, A. V., Russo, R., Schneider, R., Sterba, G., Uziel, Y., Wallace, C., Wouters, C., Wulffraat, N., Demirkaya, E., Brunner, H. I., Martini, A., Ruperto, N., Cron, R. Q., Angioloni, S., Pallotti, C., Pesce, M., Rinaldi, M., Villa, L., Abinun, M., Aggarwal, A., Akikusa, J., Al-Mayouf, S. M., Alessio, M., Anton, J., Apaz, M. T., Astigarraga, I., Ayaz, N. A., Barone, P., Bica, B., Bolt, I., Breda, L., Chasnyk, V., Cimaz, R., Corona, F., Cuttica, R., D'Angelo, G., Davidsone, Z., De Cunto, C., De Inocencio, J., Eisenstein, E., Enciso, S., Espada, G., Fischbach, M., Frosch, M., Gallizzi, R., Gamir, M. L., Gao, Y. -J., Griffin, T., Hashad, S., Hennon, T., Horneff, G., Huasong, Z., Huber, A., Insalaco, A., Ioseliani, M., Jelusic-Drazic, M., Jeng, M., Kapovic, A., Kasapcopur, O., Kone-Paut, I., De Oliveira, S. K. F., Lattanzi, B., Lepore, L., Li, C., Lipton, J. M., Magni-Manzoni, S., Maritsi, D., Mccurdy, D., Merino, R., Mulaosmanovic, V., Nielsen, S., Pal, P., Prahalad, S., Rigante, D., Rumba-Rozenfelde, I., Magalhaes, C. S., Sanner, H., Sawhney, S., Sewairi, W. M., Shakoory, B., Shenoi, S., Clovis, A. S., Stanevicha, V., Stine, K. C., Susic, G., Sztajnbok, F., Takei, S., Tezer, H., Trauzeddel, R., Tsitsami, E., Unsal, E., Vougiouka, O., Weaver, L. K., Weiss, J., Weitzman, S., Zletni, M., and Çocuk Sağlığı ve Hastalıkları

Subjects
medicine.medical_specialty, systemic juvenile idiopathic arthritis, Epidemiology, Immunology, Arthritis, Bioinformatics, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Internal medicine, medicine, Journal Article, Immunology and Allergy, 030212 general & internal medicine, Juvenile Idiopathic Arthritis, Prospective cohort study, 030203 arthritis & rheumatology, medicine.diagnostic_test, business.industry, Paediatric Rheumatology, medicine.disease, Outcomes research, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Macrophage activation syndrome, Erythrocyte sedimentation rate, Absolute neutrophil count, sense organs, business
Abstract

OBJECTIVE: To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA).METHODS: A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of experts was first asked to evaluate 115 profiles of patients with MAS, which included the values of laboratory tests at the pre-MAS visit and at MAS onset, and the change in values between the two time points. The experts were asked to choose the 5 laboratory tests in which change was most important for the diagnosis of MAS and to rank the 5 selected tests in order of importance. The relevance of change in laboratory parameters was further discussed and ranked by the same experts at a consensus conference.RESULTS: Platelet count was the most frequently selected test, followed by ferritin level, aspartate aminotransferase (AST), white cell count, neutrophil count, and fibrinogen and erythrocyte sedimentation rate. Ferritin was most frequently assigned the highest score. At the end of the process, platelet count, ferritin level and AST were the laboratory tests in which the experts found change over time to be most important.CONCLUSIONS: We identified the laboratory tests in which change over time is most valuable for the early diagnosis of MAS in sJIA. The dynamics of laboratory values during the course of MAS should be further scrutinised in a prospective study in order to establish the optimal cut-off values for their variation.

Published
2016
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26. Clinical features, treatment, and outcome of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis: a multinational, multicenter study of 362 patients

Author

Minoia, F, Davì, S, Horne, A, Demirkaya, E, Bovis, Francesca, Li, C, Lehmberg, K, Weitzman, S, Insalaco, A, Wouters, C, Shenoi, S, Espada, G, Ozen, S, Anton, J, Khubchandani, R, Russo, R, Pal, P, Kasapcopur, O, Miettunen, P, Maritsi, D, Merino, R, Shakoory, B, Alessio, M, Chasnyk, V, Sanner, H, Gao, Yj, Huasong, Z, Kitoh, T, Avcin, T, Fischbach, M, Frosch, M, Grom, A, Huber, A, Jelusic, M, Sawhney, S, Uziel, Y, Ruperto, N, Martini, Alberto, Cron, Rq, Ravelli, Angelo, Minoia, F., Davi, S., Horne, A., Demirkaya, E., Bovis, F., Li, C., Lehmberg, K., Weitzman, S., Insalaco, A., Wouters, C., Shenoi, S., Espada, G., Ozen, S., Anton, J., Khubchandani, R., Russo, R., Pal, P., Kasapcopur, O., Miettunen, P., Maritsi, D., Merino, R., Shakoory, B., Alessio, M., Chasnyk, V., Sanner, H., Gao, Y. -J., Huasong, Z., Kitoh, T., Avcin, T., Fischbach, M., Frosch, M., Grom, A., Huber, A., Jelusic, M., Sawhney, S., Uziel, Y., Ruperto, N., Martini, A., Cron, R. Q., and Ravelli, A.

Subjects
musculoskeletal diseases, Male, Biological Products, Fever, International Cooperation, Macrophage Activation Syndrome, Arthritis, Juvenile, Cohort Studies, Survival Rate, Intensive Care Units, Treatment Outcome, Adrenal Cortex Hormones, Child, Preschool, Splenomegaly, Cyclosporine, Prevalence, Humans, Female, Child, Etoposide, Hepatomegaly, Retrospective Studies
Abstract

Objective To describe the clinical, laboratory, and histopathologic features, current treatment, and outcome of patients with macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (JIA). Methods In this multinational, multicenter study, pediatric rheumatologists and hemato-oncologists entered patient data collected retrospectively into a web-based database. Results A total of 362 patients, 22% of whom had MAS at the onset of systemic JIA, were included in the study by 95 investigators from 33 countries. The most frequent clinical manifestations were fever (96%), hepatomegaly (70%), and splenomegaly (58%). Central nervous system dysfunction and hemorrhages were recorded in 35% and 20% of the patients, respectively. Platelet count and liver transaminase, ferritin, lactate dehydrogenase, triglyceride, and d-dimer levels were the sole laboratory biomarkers showing a percentage change of >50% between the pre-MAS visit and MAS onset. Evidence of macrophage hemophagocytosis was found in 60% of the patients who underwent bone marrow aspiration. MAS occurred most frequently in the setting of active underlying disease, in the absence of a specific trigger. Nearly all patients were given corticosteroids, and 61% received cyclosporine. Biologic medications and etoposide were given to 15% and 12% of the patients, respectively. Approximately one-third of the patients required admission to the intensive care unit (ICU), and the mortality rate was 8%. Conclusion This study provides information on the clinical spectrum and current management of systemic JIA-associated MAS through the analysis of a very large patient sample. MAS remains a serious condition, as a sizeable proportion of patients required admission to the ICU or died.

Published
2014

28. Expert consensus on dynamics of laboratory tests for diagnosis of macrophage activation syndrome complicating systemic juvenile idiopathic arthritis

Author

Ravelli, A, Minoia, F, Davì, S, Horne, A, Bovis, F, Pistorio, A, Aricò, M, Avcin, T, Behrens, Em, De Benedetti, F, Filipovic, A, Grom, Aa, Henter, J-i, Ilowite, Nte, Jordan, Mb, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, Dj, Miettunen, P, Nichols, Ke, Ozen, S, Schmid, Jp, Ramanan, Av, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, Hi, Martini, A, Ruperto, N, Cron, Rq, Angioloni, S, Pallotti, C, Pesce, M, Rinaldi, M, Villa, L, Abinun, M, Aggarwal, A, Akikusa, J, Al-mayouf, Sm, Alessio, M, Anton, J, Apaz, Mt, Astigarraga, I, Ayaz, Na, Barone, P, Bica, B, Bolt, I, Breda, L, Chasnyk, V, Cimaz, R, Corona, F, Cuttica, R, D'Angelo, G, Davidsone, Z, De Cunto, C, De Inocencio, J, Eisenstein, E, Enciso, S, Espada, G, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, Ml, Gao, Y-j, Griffin, T, Hashad, S, Hennon, T, Horneff, G, Huasong, Z, Huber, A, Insalaco, A, Ioseliani, M, Jelusic-drazic, M, Jeng, M, Kapovic, A, Kasapcopur, O, Kone-paut, I, De Oliveira, Skf, Lattanzi, B, Lepore, L, Li, C, Lipton, Jm, Magni-manzoni, S, Maritsi, D, Mccurdy, D, Merino, R, Mulaosmanovic, V, Nielsen, S, Pal, P, Prahalad, S, Rigante, Donato, Rumba-rozenfelde, I, Magalhaes, C, Sanner, H, Sawhney, S, Sewairi, Wm, Shakoory, B, Shenoi, S, Clovis, A, Stanevicha, V, Stine, Kc, Susic, G, Sztajnbok, F, Takei, S, Tezer, H, Trauzeddel, R, Tsitsami, E, Unsal, E, Vougiouka, O, Weaver, Lk, Weiss, J, Weitzman, S, Zletni M., On Behalf Of The Pediatric Rheumatology International Trials Organization, The Childhood Arthritis &amp, Rheumatology Research, Alliance, The Pediatric Rheumatology Collaborative Study Group And The Histiocyte, Society, Rigante D (ORCID:0000-0001-7032-7779), Ravelli, A, Minoia, F, Davì, S, Horne, A, Bovis, F, Pistorio, A, Aricò, M, Avcin, T, Behrens, Em, De Benedetti, F, Filipovic, A, Grom, Aa, Henter, J-i, Ilowite, Nte, Jordan, Mb, Khubchandani, R, Kitoh, T, Lehmberg, K, Lovell, Dj, Miettunen, P, Nichols, Ke, Ozen, S, Schmid, Jp, Ramanan, Av, Russo, R, Schneider, R, Sterba, G, Uziel, Y, Wallace, C, Wouters, C, Wulffraat, N, Demirkaya, E, Brunner, Hi, Martini, A, Ruperto, N, Cron, Rq, Angioloni, S, Pallotti, C, Pesce, M, Rinaldi, M, Villa, L, Abinun, M, Aggarwal, A, Akikusa, J, Al-mayouf, Sm, Alessio, M, Anton, J, Apaz, Mt, Astigarraga, I, Ayaz, Na, Barone, P, Bica, B, Bolt, I, Breda, L, Chasnyk, V, Cimaz, R, Corona, F, Cuttica, R, D'Angelo, G, Davidsone, Z, De Cunto, C, De Inocencio, J, Eisenstein, E, Enciso, S, Espada, G, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, Ml, Gao, Y-j, Griffin, T, Hashad, S, Hennon, T, Horneff, G, Huasong, Z, Huber, A, Insalaco, A, Ioseliani, M, Jelusic-drazic, M, Jeng, M, Kapovic, A, Kasapcopur, O, Kone-paut, I, De Oliveira, Skf, Lattanzi, B, Lepore, L, Li, C, Lipton, Jm, Magni-manzoni, S, Maritsi, D, Mccurdy, D, Merino, R, Mulaosmanovic, V, Nielsen, S, Pal, P, Prahalad, S, Rigante, Donato, Rumba-rozenfelde, I, Magalhaes, C, Sanner, H, Sawhney, S, Sewairi, Wm, Shakoory, B, Shenoi, S, Clovis, A, Stanevicha, V, Stine, Kc, Susic, G, Sztajnbok, F, Takei, S, Tezer, H, Trauzeddel, R, Tsitsami, E, Unsal, E, Vougiouka, O, Weaver, Lk, Weiss, J, Weitzman, S, Zletni M., On Behalf Of The Pediatric Rheumatology International Trials Organization, The Childhood Arthritis &amp, Rheumatology Research, Alliance, The Pediatric Rheumatology Collaborative Study Group And The Histiocyte, Society, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: To identify which laboratory tests that change over time are most valuable for the timely diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (sJIA). Methods: A multistep process, based on a combination of expert consensus and analysis of real patient data, was conducted. A panel of experts was first asked to evaluate 115 profiles of patients with MAS, which included the values of laboratory tests at the pre-MAS visit and at MAS onset, and the change in values between the two time points. The experts were asked to choose the 5 laboratory tests in which change was most important for the diagnosis of MAS and to rank the 5 selected tests in order of importance. The relevance of change in laboratory parameters was further discussed and ranked by the same experts at a consensus conference. Results: Platelet count was the most frequently selected test, followed by ferritin level, aspartate aminotransferase (AST), white cell count, neutrophil count, and fibrinogen and erythrocyte sedimentation rate. Ferritin was most frequently assigned the highest score. At the end of the process, platelet count, ferritin level and AST were the laboratory tests in which the experts found change over time to be most important. Conclusions: We identified the laboratory tests in which change over time is most valuable for the early diagnosis of MAS in sJIA. The dynamics of laboratory values during the course of MAS should be further scrutinised in a prospective study in order to establish the optimal cut-off values for their variation.

Published
2017

29. Development and initial validation of the macrophage activation syndrome/primary hemophagocytic lymphohistiocytosis score, a diagnostic tool that differentiates primary hemophagocytic lymphohistiocytosis from macrophage activation syndrome

Author

Minoia, F, Bovis, F, Davì, S, Insalaco, A, Lehmberg, K, Shenoi, S, Weitzman, S, Espada, G, Gao, Yj, Anton, J, Kitoh, T, Kasapcopur, O, Sanner, H, Merino, R, Astigarraga, I, Alessio, M, Jeng, M, Chasnyk, V, Nichols, Ke, Huasong, Z, Li, C, Micalizzi, C, Ruperto, N, Martini, A, Cron, Rq, Ravelli, A, Horne, A, Abinun, M, Aggarwal, A, Akikusa, J, Al Mayouf, S, Apaz, Mt, Avcin, T, Ayaz, Na, Barone, P, Bica, B, Bolt, I, Breda, L, Cimaz, R, Corona, F, Cuttica, R, Davidsone, Z, De Cunto, C, De Inocencio, J, Demirkaya, E, Eisenstein, Em, Enciso, S, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, Ml, Griffin, T, Grom, A, Hashad, S, Hennon, T, Henter, Ji, Horneff, G, Huber, A, Ilowite, N, Ioseliani, M, Kapović, Am, Khubchandani, R, Koné Paut, I, de Oliveira, Skf, Lattanzi, B, Lepore, L, Lipton, Jm, Magni Manzoni, S, Maritsi, D, Mccurdy, D, Miettunen, P, Mulaosmanovic, V, Nielsen, S, Ozen, S, Pal, P, Prahalad, S, Rigante, Donato, Rumba Rozenfelde, I, Russo, R, Magalhães, C, Sewairi, Wm, Artur Silva, C, Stanevicha, V, Sterba, G, Stine, Kc, Susic, G, Sztajnbok, F, Takei, S, Trauzeddel, R, Tsitsami, E, Unsal, E, Uziel, Y, Vougiouka, O, Wallace, Ca, Weaver, L, Weiss J, E, Wouters, C, Wulffraat, N, Zletni, M, Aricò, M, Egeler, Rm, Filipovich, Ah, Gadner, H, Imashuku, S, Janka, G, Ladisch, S, Mcclain, Kl, Webb, D., Rigante, Donato (ORCID:0000-0001-7032-7779), Minoia, F, Bovis, F, Davì, S, Insalaco, A, Lehmberg, K, Shenoi, S, Weitzman, S, Espada, G, Gao, Yj, Anton, J, Kitoh, T, Kasapcopur, O, Sanner, H, Merino, R, Astigarraga, I, Alessio, M, Jeng, M, Chasnyk, V, Nichols, Ke, Huasong, Z, Li, C, Micalizzi, C, Ruperto, N, Martini, A, Cron, Rq, Ravelli, A, Horne, A, Abinun, M, Aggarwal, A, Akikusa, J, Al Mayouf, S, Apaz, Mt, Avcin, T, Ayaz, Na, Barone, P, Bica, B, Bolt, I, Breda, L, Cimaz, R, Corona, F, Cuttica, R, Davidsone, Z, De Cunto, C, De Inocencio, J, Demirkaya, E, Eisenstein, Em, Enciso, S, Fischbach, M, Frosch, M, Gallizzi, R, Gamir, Ml, Griffin, T, Grom, A, Hashad, S, Hennon, T, Henter, Ji, Horneff, G, Huber, A, Ilowite, N, Ioseliani, M, Kapović, Am, Khubchandani, R, Koné Paut, I, de Oliveira, Skf, Lattanzi, B, Lepore, L, Lipton, Jm, Magni Manzoni, S, Maritsi, D, Mccurdy, D, Miettunen, P, Mulaosmanovic, V, Nielsen, S, Ozen, S, Pal, P, Prahalad, S, Rigante, Donato, Rumba Rozenfelde, I, Russo, R, Magalhães, C, Sewairi, Wm, Artur Silva, C, Stanevicha, V, Sterba, G, Stine, Kc, Susic, G, Sztajnbok, F, Takei, S, Trauzeddel, R, Tsitsami, E, Unsal, E, Uziel, Y, Vougiouka, O, Wallace, Ca, Weaver, L, Weiss J, E, Wouters, C, Wulffraat, N, Zletni, M, Aricò, M, Egeler, Rm, Filipovich, Ah, Gadner, H, Imashuku, S, Janka, G, Ladisch, S, Mcclain, Kl, Webb, D., and Rigante, Donato (ORCID:0000-0001-7032-7779)

Abstract

OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample. Variables that entered the best fitted model of logistic regression were assigned a score, based on their statistical weight. The MAS/HLH (MH) score was made up with the individual scores of selected variables. The cutoff in the MH score that discriminated pHLH from MAS best was calculated by means of receiver operating characteristic curve analysis. Score performance was examined in both developmental and validation samples. RESULTS: Six variables composed the MH score: age at onset, neutrophil count, fibrinogen, splenomegaly, platelet count, and hemoglobin. The MH score ranged from 0 to 123, and its median value was 97 (1st-3rd quartile 75-123) and 12 (1st-3rd quartile 11-34) in pHLH and MAS, respectively. The probability of a diagnosis of pHLH ranged from <1% for a score of <11 to >99% for a score of ≥123. A cutoff value of ≥60 revealed the best performance in discriminating pHLH from MAS. CONCLUSION: The MH score is a powerful tool that may aid practitioners to identify patients who are more likely to have pHLH and, thus, could be prioritized for functional and genetic testing.

Published
2017

32. The addition of one or more biologics to methotrexate in children with juvenile idiopathic arthritis increases the incidence of infections and serious adverse events.:The 5882 Pharmachild cohort

Author

Swart, J, Pistorio, A, Bovis, F, Alekseeva, E, Hofer, M., Nielsen, S., Anton, J, Consolaro, A, Panaviene, V, Stanevicha, V, Trachana, M, Ailioaie, C, Quartier, P., de Benedetti, F, Tsitsami, E, Flato, B, Dolezalova, P, Constantin, T., Herlin, Troels, Kamphuis, S, Sawhney, S, Maritsi, D, Vargova, V, Villa, L, Palotti, C, Ravelli, A, Martini, A, Wulffraat, N, and Ruperto, N

Published
2015

35. OP0062 The Addition of One or More Biologics to Methotrexate in Children with Juvenile Idiopathic Arthritis Increases the Incidence of Infections and Serious Adverse Events. The 5882 Pharmachild Cohort

Author

Swart, J., primary, Pistorio, A., additional, Bovis, F., additional, Alekseeva, E., additional, Hofer, M., additional, Nielsen, S., additional, Anton, J., additional, Consolaro, A., additional, Panaviene, V., additional, Stanevicha, V., additional, Trachana, M., additional, Ailioaie, C., additional, Quartier, P., additional, De Benedetti, F., additional, Tsitsami, E., additional, Flato, B., additional, Dolezalova, P., additional, Constantin, T., additional, Herlin, T., additional, Kamphuis, S., additional, Sawhney, S., additional, Maritsi, D., additional, Vargova, V., additional, Villa, L., additional, Pallotti, C., additional, Ravelli, A., additional, Martini, A., additional, Wulffraat, N., additional, and Ruperto, N., additional

Published
2015
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36. What's new in p53?

Author

Maritsi, D., Stagikas, D., Charalabopoulos, K., and Batistatou, A.

Abstract

p53 is the main intrinsic factor inducing apoptosis by recognizing the external stimuli and activating the p53 responsive genes to an irreversible series of events. P53 activates the transcription of specific proapoptotic genes called p53 target genes. A growing number of p53 responsive genes have been identified and numerous studies have demonstrated that p53 proapoptotic factors such as Noxa, Puma and Perp play cell type specific roles in p53's mediated response to certain stimuli. Perp (p53 apoptosis effector related to PMP-22) is a direct proapoptotic target gene encoding a tetraspan protein. Perp is highly expressed in cells undergoing apoptosis compared to cells under G1 arrest and its overexpression is sufficient to cause cell death in fibroblasts. Noxa is another member of the preapoptotic p53 genes family. When expressed Noxa acts in a BH3 motif-dependent localization to mitochondria, causing structural changes, activation of caspase 9 and release of cytochrome c from mitochondria to cytosol. Puma (p53 mutant of apoptosis) is another critical mediator of p53-dependent apoptosis. P53 binds to Puma-promoter gene sites, leading to puma production. The mtCLIC, a member of intracellular chloride channels, is a cytoplasmic and mitochondrial protein positively regulated by p53. Caspase 10 is induced in p53-dependent manner leading to cellular apoptosis. Other newly announced factors are also involved in p53-regulated apoptosis such as brain-specific angiogenesis inhibitor-1 (BSAI1), MSOD and GPX genes. A global discussion on this topic is attempted in the present review article. Hippokratia

Published
2006

37. What's new in p53

Author

Maritsi, D, Stagikas, D, Charalabopoulos, K, and Batistatou, A

Subjects
Review Article
Abstract

p53 is the main intrinsic factor inducing apoptosis by recognizing the external stimuli and activating the p53 responsive genes to an irreversible series of events. P53 activates the transcription of specific proapoptotic genes called p53 target genes. A growing number of p53 responsive genes have been identified and numerous studies have demonstrated that p53 proapoptotic factors such as Noxa, Puma and Perp play cell type specific roles in p53's mediated response to certain stimuli. Perp (p53 apoptosis effector related to PMP-22) is a direct proapoptotic target gene encoding a tetraspan protein. Perp is highly expressed in cells undergoing apoptosis compared to cells under G1 arrest and its overexpression is sufficient to cause cell death in fibroblasts. Noxa is another member of the preapoptotic p53 genes family. When expressed Noxa acts in a BH3 motif-dependent localization to mitochondria, causing structural changes, activation of caspase 9 and release of cytochrome c from mitochondria to cytosol. Puma (p53 mutant of apoptosis) is another critical mediator of p53-dependent apoptosis. P53 binds to Puma-promoter gene sites, leading to puma production. The mtCLIC, a member of intracellular chloride channels, is a cytoplasmic and mitochondrial protein positively regulated by p53. Caspase 10 is induced in p53-dependent manner leading to cellular apoptosis. Other newly announced factors are also involved in p53-regulated apoptosis such as brain-specific angiogenesis inhibitor - 1 (BSAI1), MSOD and GPX genes. A global discussion on this topic is attempted in the present review article.

Published
2006

41. Decreased antibodies against rubella in previously vaccinated treatment-naïve childhood systemic lupus erythematous patients: a prospective case-control study.

Author

Maritsi, DN, Coffin, S, Onoufriou, M, Spyridis, N, Tsolia, MN, Maritsi, D N, and Tsolia, M N

Subjects
SYSTEMIC lupus erythematosus, AUTOIMMUNE diseases, IMMUNOLOGIC diseases, IMMUNOSUPPRESSIVE agents, RUBELLA, IMMUNIZATION, LONGITUDINAL method, RNA viruses, RUBELLA vaccines, VIRAL antibodies, CASE-control method, PREVENTION
Abstract

The article discusses the systemic lupus erythematosus (SLE) which is a multisystem autoimmune disease primarily affecting young females and that patients are susceptible to infections because of their defective immune system and the immunosuppressive treatment they receive. Also discussed is the study that determined the immune status against rubella in previously vaccinated childhood SLE patients before the start of treatment.

Published
2019
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47. Development and initial validation of the macrophage activation syndrome/primary hemophagocytic lymphohistiocytosis score, a diagnostic tool that differentiates primary hemophagocytic lymphohistiocytosis from macrophage activation syndrome

Author

Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E. Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q. Cron, Angelo Ravelli, AnnaCarin Horne, Mario Abinun, Amita Aggarwal, Jonathan Akikusa, Sulaiman Al-Mayouf, Maria Teresa Apaz, Tadej Avcin, Nuray Aktay Ayaz, Patrizia Barone, Bianca Bica, Isabel Bolt, Luciana Breda, Rolando Cimaz, Fabrizia Corona, Ruben Cuttica, Zane Davidsone, Carmen De Cunto, Jaime De Inocencio, Erkan Demirkaya, Eli M. Eisenstein, Sandra Enciso, Michel Fischbach, Michael Frosch, Romina Gallizzi, Maria Luz Gamir, Thomas Griffin, Alexei Grom, Soad Hashad, Teresa Hennon, Jan-Inge Henter, Gerd Horneff, Adam Huber, Norman Ilowite, Maka Ioseliani, Agneza Marija Kapović, Raju Khubchandani, Isabelle Koné-Paut, Sheila Knupp Feitosa de Oliveira, Bianca Lattanzi, Loredana Lepore, Jeffrey M. Lipton, Silvia Magni-Manzoni, Despoina Maritsi, Deborah McCurdy, Paivi Miettunen, Velma Mulaosmanovic, Susan Nielsen, Seza Ozen, Priyankar Pal, Sampath Prahalad, Donato Rigante, Ingrida Rumba-Rozenfelde, Ricardo Russo, Claudia Saad Magalhães, Wafaa Mohamed Saad Sewairi, Clovis Artur Silva, Valda Stanevicha, Gary Sterba, Kimo C. Stine, Gordana Susic, Flavio Sztajnbok, Syuji Takei, Ralf Trauzeddel, Elena Tsitsami, Erbil Unsal, Yosef Uziel, Olga Vougiouka, Carol A. Wallace, Lehn Weaver, Jennifer E. Weiss, Carine Wouters, Nico Wulffraat, Mabruka Zletni, Maurizio Arico, R. Maarten Egeler, Alexandra H. Filipovich, Helmut Gadner, Shinsaku Imashuku, Gritta Janka, Stephan Ladisch, Ken L. McClain, David Webb, Minoia, F., Bovis, F., Davi, S., Insalaco, A., Lehmberg, K., Shenoi, S., Weitzman, S., Espada, G., Gao, Y. -J., Anton, J., Kitoh, T., Kasapcopur, O., Sanner, H., Merino, R., Astigarraga, I., Alessio, M., Jeng, M., Chasnyk, V., Nichols, K. E., Huasong, Z., Li, C., Micalizzi, C., Ruperto, N., Martini, A., Cron, R. Q., Ravelli, A., Horne, A., Abinun, M., Aggarwal, A., Akikusa, J., Al-Mayouf, S., Apaz, M. T., Avcin, T., Ayaz, N. A., Barone, P., Bica, B., Bolt, I., Breda, L., Cimaz, R., Corona, F., Cuttica, R., Davidsone, Z., De Cunto, C., De Inocencio, J., Demirkaya, E., Eisenstein, E. M., Enciso, S., Fischbach, M., Frosch, M., Gallizzi, R., Gamir, M. L., Griffin, T., Grom, A., Hashad, S., Hennon, T., Henter, J. -I., Horneff, G., Huber, A., Ilowite, N., Ioseliani, M., Kapovic, A. M., Khubchandani, R., Kone-Paut, I., de Oliveira, S. K. F., Lattanzi, B., Lepore, L., Lipton, J. M., Magni-Manzoni, S., Maritsi, D., Mccurdy, D., Miettunen, P., Mulaosmanovic, V., Nielsen, S., Ozen, S., Pal, P., Prahalad, S., Rigante, D., Rumba-Rozenfelde, I., Russo, R., Magalhaes, C. S., Sewairi, W. M. S., Artur Silva, C., Stanevicha, V., Sterba, G., Stine, K. C., Susic, G., Sztajnbok, F., Takei, S., Trauzeddel, R., Tsitsami, E., Unsal, E., Uziel, Y., Vougiouka, O., Wallace, C. A., Weaver, L., E. Weiss, J., Wouters, C., Wulffraat, N., Zletni, M., Arico, M., Egeler, R. M., Filipovich, A. H., Gadner, H., Imashuku, S., Janka, G., Ladisch, S., Mcclain, K. L., and Webb, D.

Subjects
Male, 0301 basic medicine, Hemophagocytic, Logistic regression, Pediatrics, hemophagocytic syndrome, 0302 clinical medicine, diagnostic score, Diagnosis, Medicine, Cutoff, Child, primary hemophagocytic lymphohistiocytosi, Lymphohistiocytosis, education.field_of_study, primary hemophagocytic lymphohistiocytosis, Perinatology and Child Health, Quartile, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Macrophage activation syndrome, Child, Preschool, macrophage activation syndrome, Absolute neutrophil count, Female, Human, medicine.medical_specialty, Adolescent, Population, Lymphohistiocytosis, Hemophagocytic, Diagnosis, Differential, 03 medical and health sciences, Internal medicine, Humans, Preschool, education, 030203 arthritis & rheumatology, Receiver operating characteristic, business.industry, Infant, Reproducibility of Results, medicine.disease, Surgery, 030104 developmental biology, Macrophage Activation Syndrome, Pediatrics, Perinatology and Child Health, Differential, Differential diagnosis, business
Abstract

OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample. Variables that entered the best fitted model of logistic regression were assigned a score, based on their statistical weight. The MAS/HLH (MH) score was made up with the individual scores of selected variables. The cutoff in the MH score that discriminated pHLH from MAS best was calculated by means of receiver operating characteristic curve analysis. Score performance was examined in both developmental and validation samples. RESULTS: Six variables composed the MH score: age at onset, neutrophil count, fibrinogen, splenomegaly, platelet count, and hemoglobin. The MH score ranged from 0 to 123, and its median value was 97 (1st-3rd quartile 75-123) and 12 (1st-3rd quartile 11-34) in pHLH and MAS, respectively. The probability of a diagnosis of pHLH ranged from 99% for a score of =123. A cutoff value of =60 revealed the best performance in discriminating pHLH from MAS. CONCLUSION: The MH score is a powerful tool that may aid practitioners to identify patients who are more likely to have pHLH and, thus, could be prioritized for functional and genetic testing.

Published
2017

48. Influenza vaccine uptake in juvenile idiopathic arthritis: a multi-centre cross-sectional study.

Author

Maritsi D, Dasoula F, Ziv A, Bizjak M, Balažiová B, Matošević M, Yildiz M, Alpert N, Lamot L, Kasapcopur O, Dallos T, Uziel Y, Toplak N, and Heshin-Bekenstein M

Subjects
Humans, Cross-Sectional Studies, Male, Female, Child, Child, Preschool, Health Knowledge, Attitudes, Practice, Adolescent, Vaccination statistics & numerical data, Vaccination Coverage statistics & numerical data, Arthritis, Juvenile, Influenza Vaccines administration & dosage, Influenza, Human prevention & control
Abstract

While most countries provide safe and effective influenza vaccines for at-risk groups, influenza vaccine coverage among children with rheumatic diseases remains uncertain. This study investigated influenza vaccination rates in children with juvenile idiopathic arthritis (JIA) during the 2019-2020 season and assessed the knowledge and attitudes of caregivers of children with JIA regarding influenza vaccination. The secondary aims were to identify barriers to vaccination and explore strategies to improve vaccination rates. A multi-centre, cross-sectional anonymous survey was conducted in 7 countries during the 2019-2020 influenza season to assess the uptake history of influenza vaccination. Among 287 participants, only 87 (30%) children with JIA received the influenza vaccine during the 2019-2020 season. Children who were more likely to be vaccinated were those with systemic juvenile idiopathic arthritis (sJIA), a history of previous vaccination and those aware of the vaccination recommendations. Conversely, children who previously experienced adverse vaccine-related events reported the lowest uptake. The primary reason for non-vaccination was lack of awareness about the necessity of influenza vaccination.  Conclusion: Despite variations among countries, the uptake of influenza vaccines remains low in children with JIA. Improving awareness among families about the importance of influenza vaccination may increase vaccination rates in children with rheumatic diseases. What is Known: • Rheumatic children are at increased risk for influenza infection due to immunosuppressive therapy and immune dysregulation. • Influenza vaccine is formally recommended to children with rheumatic diseases. What is New: • This multicentre study showed that influenza vaccine uptake rates remain suboptimal among children with Juvenile Idiopathic Arthritis despite formal recommendations. • Factors like previous experience with vaccination and information provided by medical professionals via different ways play essential roles in increasing vaccination rates and can contribute to improved health outcomes for these vulnerable children., (© 2024. The Author(s).)

Published
2024
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49. Multisystem inflammatory syndrome in children (MIS-C): A nationwide collaborative study in the Greek population.

Author

Lampidi S, Maritsi D, Charakida M, Eleftheriou I, Farmaki E, Spyridis N, Charisi K, Vantsi P, Filippatos F, Skourti K, Papadopoulou-Alataki E, Papadopoulou-Legbelou K, Kampouridou P, Grivea IN, Vergadi E, Gkentzi D, Dimou D, Koletsi P, Fotis L, Liakopoulou T, Agrafiotou A, Kourtesi K, Tsolas G, Kafetzis D, Papaevangelou V, Dimitriou G, Galanakis E, Syrogiannopoulos GA, Spoulou V, Michos A, Roilides E, and Tsolia MN

Subjects
Child, Male, Humans, Greece, Retrospective Studies, Disease Progression, Adrenal Cortex Hormones, Myocarditis, COVID-19 complications, COVID-19 epidemiology, COVID-19 therapy, Pericarditis, Acute Kidney Injury, Systemic Inflammatory Response Syndrome
Abstract

Multisystem inflammatory syndrome in children (MIS-C) is a rare but severe hyperinflammatory condition that may occur following SARS-CoV-2 infection. This retrospective, descriptive study of children hospitalized with multisystem inflammatory syndrome in children (MIS-C) in 12 tertiary care centers from 3/11/2020 to 12/31/2021. Demographics, clinical and laboratory characteristics, treatment and outcomes are described. Among 145 patients (95 males, median age 8.2 years) included, 123 met the WHO criteria for MIS-C, while 112 (77%) had serological evidence of SARS-CoV-2 infection. Fever was present in 99%, gastrointestinal symptoms in 77%, mucocutaneous involvement in 68% and respiratory symptoms in 28%. Fifty-five patients (38%) developed myocarditis, 29 (20%) pericarditis and 19 (13%) coronary aneurysms. Among the above cases 11/55 (20%), 1/29 (3.4%) and 5/19 (26.3%), respectively, cardiac complications had not fully resolved at discharge. Underlying comorbidities were reported in 18%. Median CRP value was 155 mg/l, ferritin 535 ng/ml, PCT 1.6 ng/ml and WBC 14.2 × 10 9 /mm 3 . Most patients had elevated troponin (41.3%) and/or NT-pro-BNP (49.6%). Intravenous immunoglobulin plus corticosteroids were used in 117/145 (80.6%), monotherapy with IVIG alone in 13/145 (8.9%) and with corticosteroids alone in 2/145 (1.3%). Anti-IL1 treatment was added in 15 patients (10.3%). Thirty-three patients (23%) were admitted to the PICU, 14% developed shock and 1 required ECMO. Mortality rate was 0.68%. The incidence of MIS-C was estimated at 0.69/1000 SARS-CoV-2 infections. Patients who presented with shock had higher levels of NT-pro-BNP compared to those who did not (p < 0.001). Acute kidney injury and/or myocarditis were associated with higher risk of developing shock., Conclusion: MIS-C is a novel, infrequent but serious disease entity. Cardiac manifestations included myocarditis and pericarditis, which resolved in most patients before discharge. Timely initiation of immunomodulatory therapy was shown to be effective. NT-pro-BNP levels may provide a better prediction and monitoring of the disease course. Further research is required to elucidate the pathogenesis, risk factors and optimal management, and long-term outcomes of this clinical entity., What Is Known: • MIS-C is an infrequent but serious disease entity. • Patients with MIS-C present with multi-organ dysfunction, primarily involving the gastrointestinal and cardiovascular systems., What Is New: • NT-pro-BNP levels may provide a better prediction and monitoring of the disease course. • Acute kidney injury and/or myocarditis were associated with higher risk of developing shock., (© 2024. The Author(s).)

Published
2024
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50. Antiphospholipid syndrome after streptococcal toxic shock syndrome later diagnosed as Behcet's disease.

Author

Kourti A, Mentesidou L, Michalopoulou A, Maritsi D, and Bachou T

Subjects
Male, Humans, Antibodies, Antiphospholipid, Behcet Syndrome complications, Behcet Syndrome diagnosis, Antiphospholipid Syndrome complications, Antiphospholipid Syndrome diagnosis, Shock, Septic diagnosis, Shock, Septic etiology, Streptococcal Infections complications, Streptococcal Infections diagnosis
Abstract

Streptococcal toxic shock syndrome is a severe complication of group A streptococci. The production of antiphospholipid antibodies has been associated with streptococcal infections and with autoimmune diseases. Furthermore, streptococcal infections could be a trigger of Behcet's disease. We report a case of a boy who presented antiphospholipid syndrome after streptococcal toxic shock syndrome later he was diagnosed with Behcet's disease., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Published
2023
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