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1. Versorgungsrealität der stationären vasoaktiven Therapie mit Prostazyklinderivaten bei Patienten mit akralen Durchblutungsstörungen bei systemischer Sklerose in Deutschland

Author

Juche, A., Siegert, E., Mueller-Ladner, U., Riemekasten, G., Günther, C., Kötter, I., Henes, J., Blank, N., Voll, R. E., Ehrchen, J., Schmalzing, M., Susok, L., Schmeiser, T., Sunderkoetter, C., Distler, J., Worm, M., Kreuter, A., Horváth, O. N., Schön, M. P., Korsten, P., Zeidler, G., Pfeiffer, C., Krieg, T., Hunzelmann, N., and Moinzadeh, P.

Published
2020
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6. Rituximab is more effective than second anti-TNF therapy in rheumatoid arthritis patients and previous TNFα blocker failure

Author

Kekow J, Mueller-Ladner U, and Schulze-Koops H

Subjects
Medicine (General), R5-920
Abstract

Joern Kekow,1 Ulf Mueller-Ladner,2 Hendrik Schulze-Koops31Clinic of Rheumatology and Orthopedics, Otto-von-Guericke University of Magdeburg, Vogelsang-Gommern; 2Department of Rheumatology and Clinical Immunology, Kerckhoff Clinic, Bad Nauheim; 3Division of Rheumatology, University of Munich, Munich, GermanyPurpose: To assess the efficacy of one course of rituximab (two 1-g doses) compared to an alternative tumor necrosis factor-α (TNFα) blocker in rheumatoid arthritis patients who had experienced one previous TNFα blocker failure (eg, etanercept, adalimumab, or infliximab).Patients and methods: The efficacy of both treatments was studied in this retrospective, multicenter, noninterventional cohort study with 196 patients. All patients had active rheumatoid arthritis defined by a Disease Activity Score-28 of ≥3.2 despite having TNFα blocker therapy, and were followed over 6.6 months on average after switching to rituximab versus a second TNFα blocker (ie, switching to etanercept, adalimumab, or infliximab) at baseline.Results: At baseline, both cohorts showed similar demographic and disease-related characteristics (including Disease Activity Score-28). At the end of observation, mean Disease Activity Score-28 was significantly lower after treatment with rituximab than with a second TNFα blocker (-1.64 [95% confidence interval: -1.92; -1.36] versus -1.19 [95% confidence interval: -1.42; -0.96], P = 0.013). This difference between the two groups was even more pronounced when patients were seropositive for rheumatoid factor (-1.66 versus -1.17, P = 0.018) and anti-cyclic citrullinated peptide antibodies (-1.75 versus -1.06, P = 0.002). More rituximab-treated patients achieved good European League Against Rheumatism response than TNFα blocker-treated patients (30% versus 15%), and less patients were nonresponders (22% versus 35%) according to European League Against Rheumatism criteria (P = 0.022, chi-squared test).Conclusion: Treatment with rituximab was more effective than a second TNFα blocker therapy in rheumatoid arthritis patients after failure of the first TNFα blocker. It was found that anti-cyclic citrullinated peptide antibodies may be a useful predictive biomarker for response to rituximab in patients with TNFα blocker treatment failure.Keywords: rheumatoid arthritis, rituximab, anti-cyclic citrullinated peptide antibodies, rheumatoid factor

Published
2012

7. EULAR points to consider for the management of difficult-to-treat rheumatoid arthritis

Author

Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Pchelnikova, P., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, J.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Dumas, S., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Pchelnikova, P., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, J.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Dumas, S., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., and van Laar, J.M.

Abstract

Objective: To develop evidence- based European Alliance of Associations for Rheumatology (EULAR) points to consider (PtCs) for the management of difficult- to- treat rheumatoid arthritis (D2T RA). Methods: An EULAR Task Force was established comprising 34 individuals: 26 rheumatologists, patient partners and rheumatology experienced health professionals. Two systematic literature reviews addressed clinical questions around diagnostic challenges, and pharmacological and non- pharmacological therapeutic strategies in D2T RA. PtCs were formulated based on the identified evidence and expert opinion. Strength of recommendations (SoR, scale A–D: A typically consistent level 1 studies and D level 5 evidence or inconsistent studies) and level of agreement (LoA, scale 0–10: 0 completely disagree and 10 completely agree) of the PtCs were determined by the Task Force members. Results: Two overarching principles and 11 PtCs were defined concerning diagnostic confirmation of RA, evaluation of inflammatory disease activity, pharmacological and non- pharmacological interventions, treatment adherence, functional disability, pain, fatigue, goal setting and self- efficacy and the impact of comorbidities. The SoR varied from level C to level D. The mean LoA with the overarching principles and PtCs was generally high (8.4–9.6). Conclusions: These PtCs for D2T RA can serve as a clinical roadmap to support healthcare professionals and patients to deliver holistic management and more personalised pharmacological and non- pharmacological therapeutic strategies. High- quality evidence was scarce. A research agenda was created to guide future research

Published
2022

9. EULAR points to consider for the management of difficult-to-treat rheumatoid arthritis

Author

Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Pchelnikova, P., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, J.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Dumas, S., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Pchelnikova, P., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, J.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Dumas, S., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., and van Laar, J.M.

Published
2022

11. Vaccination in adult patients with auto-immune inflammatory rheumatic diseases: A systematic literature review for the European League Against Rheumatism evidence-based recommendations for vaccination in adult patients with auto-immune inflammatory rheumatic diseases

Author

van Assen, S., Elkayam, O., Agmon-Levin, N., Cervera, R., Doran, M.F., Dougados, M., Emery, P., Geborek, P., Ioannidis, J.P.A., Jayne, D.R.W., Kallenberg, C.G.M., Müller-Ladner, U., Shoenfeld, Y., Stojanovich, L., Valesini, G., Wulffraat, N.M., and Bijl, M.

Published
2011
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12. The Predict Study: low risk for digital ulcer development in patients with systemic sclerosis with increasing disease duration and lack of topoisomerase-1 antibodies

Author

Hunzelmann, N., Riemekasten, G., Becker, M. O., Moinzadeh, P., Kreuter, A., Melchers, I., Mueller-Ladner, U., Meier, F., Worm, M., Lee, H., Herrgott, I., Pfeiffer, C., Fierlbeck, G., Henes, J., Juche, A., Zeidler, G., Mensing, H., Günther, C., Sárdy, M., Burkhardt, H., Koehm, M., Kuhr, K., Krieg, T., and Sunderkötter, C.

Published
2016
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15. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author

Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, Cavagna, L, Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., and Cavagna L.

Abstract

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.

Published
2019

16. IgG4-related diseases: State of the art on clinical practice guidelines

Author

Iaccarino, L, Talarico, R, Scire, C, Amoura, Z, Burmester, G, Doria, A, Faiz, K, Frank, C, Hachulla, E, Hie, M, Launay, D, Montecucco, C, Monti, S, Mouthon, L, Tincani, A, Toniati, P, Van Hagen, P, Van Vollenhoven, R, Bombardieri, S, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Alexander, T, Iaccarino L., Talarico R., Scire C. A., Amoura Z., Burmester G., Doria A., Faiz K., Frank C., Hachulla E., Hie M., Launay D., Montecucco C., Monti S., Mouthon L., Tincani A., Toniati P., Van Hagen P. M., Van Vollenhoven R. F., Bombardieri S., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., Alexander T., Iaccarino, L, Talarico, R, Scire, C, Amoura, Z, Burmester, G, Doria, A, Faiz, K, Frank, C, Hachulla, E, Hie, M, Launay, D, Montecucco, C, Monti, S, Mouthon, L, Tincani, A, Toniati, P, Van Hagen, P, Van Vollenhoven, R, Bombardieri, S, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Alexander, T, Iaccarino L., Talarico R., Scire C. A., Amoura Z., Burmester G., Doria A., Faiz K., Frank C., Hachulla E., Hie M., Launay D., Montecucco C., Monti S., Mouthon L., Tincani A., Toniati P., Van Hagen P. M., Van Vollenhoven R. F., Bombardieri S., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., and Alexander T.

Abstract

Immunoglobulin G4-related diseases (IgG4-RD) are a group of chronic relapsing-remitting inflammatory conditions, characterised by tissue infiltration with lymphocytes and IgG4-secreting plasma cells, fibrosis and a usually favourable response to steroids. In this narrative review, we summarise the results of a systematic literature research, which was performed as part of the European Reference Network ReCONNET, aimed at evaluating existing clinical practice guidelines (CPGs) and recommendations in IgG4-RD. From 167 publications initially obtained from a systematic literature search, only one was identified as a systematic multispecialist, evidence-based, consensus guidance statement on diagnosis and treatment of IgG4-RD, which may be recommended for use as CPG in IgG4-RD. With the recognition of a limited evidence based in this increasingly recognised disease, the group discussion has identified the following unmet needs: lack of shared classification criteria, absence of formal guidelines on diagnosis, no evidence-based therapeutic recommendations and lack of activity and damage indices. Areas of unmet needs include the difficulties in diagnosis, management and monitoring and the scarcity of expert centres.

Published
2019

17. EULAR definition of difficult-to-treat rheumatoid arthritis

Author

Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Leerstoel Geenen, and Clinical Psychology (overkoepelend voor heel KP)

Subjects
musculoskeletal diseases, medicine.medical_specialty, rheumatoid, Advisory Committees, immune system diseases, Immunology, Drug Resistance, Arthritis, Biochemistry, General Biochemistry, Genetics and Molecular Biology, Healthcare improvement science Radboud Institute for Health Sciences [Radboudumc 18], Arthritis, Rheumatoid, All institutes and research themes of the Radboud University Medical Center, Quality of life, Rheumatology, Stakeholder Participation, Synovitis, Terminology as Topic, Dmard therapy, medicine, Humans, Immunology and Allergy, Treatment Failure, Intensive care medicine, skin and connective tissue diseases, Glucocorticoids, Biological Products, business.industry, Task force, Biochemistry, Genetics and Molecular Biology(all), Recommendation, medicine.disease, Clinical trial, Europe, arthritis, Rheumatoid arthritis, Antirheumatic Agents, Practice Guidelines as Topic, Disease Progression, Drug Therapy, Combination, business, synovitis, Rheumatism, Genetics and Molecular Biology(all)
Abstract

BackgroundDespite treatment according to the current management recommendations, a significant proportion of patients with rheumatoid arthritis (RA) remain symptomatic. These patients can be considered to have ‘difficult-to-treat RA’. However, uniform terminology and an appropriate definition are lacking.ObjectiveThe Task Force in charge of the “Development of EULAR recommendations for the comprehensive management of difficult-to-treat rheumatoid arthritis” aims to create recommendations for this underserved patient group. Herein, we present the definition of difficult-to-treat RA, as the first step.MethodsThe Steering Committee drafted a definition with suggested terminology based on an international survey among rheumatologists. This was discussed and amended by the Task Force, including rheumatologists, nurses, health professionals and patients, at a face-to-face meeting until sufficient agreement was reached (assessed through voting).ResultsThe following three criteria were agreed by all Task Force members as mandatory elements of the definition of difficult-to-treat RA: (1) Treatment according to European League Against Rheumatism (EULAR) recommendation and failure of ≥2 biological disease-modifying antirheumatic drugs (DMARDs)/targeted synthetic DMARDs (with different mechanisms of action) after failing conventional synthetic DMARD therapy (unless contraindicated); (2) presence of at least one of the following: at least moderate disease activity; signs and/or symptoms suggestive of active disease; inability to taper glucocorticoid treatment; rapid radiographic progression; RA symptoms that are causing a reduction in quality of life; and (3) the management of signs and/or symptoms is perceived as problematic by the rheumatologist and/or the patient.ConclusionsThe proposed EULAR definition for difficult-to-treat RA can be used in clinical practice, clinical trials and can form a basis for future research.

Published
2021

18. EULAR definition of difficult-to-treat rheumatoid arthritis

Author

Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., van Laar, J.M., Leerstoel Geenen, Clinical Psychology (overkoepelend voor heel KP), Nagy, G., Roodenrijs, N.M., Welsing, P.M., Kedves, M., Hamar, A., van der Goes, M.C., Kent, A., Bakkers, M., Blaas, E., Senolt, L., Szekanecz, Z., Choy, E., Dougados, M., Jacobs, J.W., Geenen, R., Bijlsma, H.W., Zink, A., Aletaha, D., Schoneveld, L., van Riel, P., Gutermann, L., Prior, Y., Nikiphorou, E., Ferraccioli, G., Schett, G., Hyrich, K.L., Mueller-Ladner, U., Buch, M.H., McInnes, I.B., van der Heijde, D., and van Laar, J.M.

Published
2021

19. LONG-TERM OUTCOME OF SSC ASSOCIATED ILD: IMPROVED SURVIVAL IN PPI TREATED PATIENTS

Author

Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Kathrin, K., Henes, J., Siegert, E., Riemekasten, G., Blank, N., Pfeiffer, C., Mueller-Ladner, U., Kreuter, A., Korsten, P., Juche, A., Schmalzing, M., Worm, M., Jandova, I., Susok, L., Schmeiser, T., Guenther, C., Keyszer, G., Ehrchen, J., Ramming, A., Koetter, I., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2021

20. Systemic sclerosis: State of the art on clinical practice guidelines

Author

Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., Matucci-Cerinic M., Smith, V, Scire, C, Talarico, R, Airo, P, Alexander, T, Allanore, Y, Bruni, C, Codullo, V, Dalm, V, De Vries-Bouwstra, J, Della Rossa, A, Distler, O, Galetti, I, Launay, D, Lepri, G, Mathian, A, Mouthon, L, Ruaro, B, Sulli, A, Tincani, A, Vandecasteele, E, Vanhaecke, A, Vanthuyne, M, Van Den Hoogen, F, Van Vollenhoven, R, Voskuyl, A, Zanatta, E, Bombardieri, S, Burmester, G, Eurico, F, Frank, C, Hachulla, E, Houssiau, F, Mueller-Ladner, U, Schneider, M, Van Laar, J, Vieira, A, Cutolo, M, Mosca, M, Matucci-Cerinic, M, Smith V., Scire C. A., Talarico R., Airo P., Alexander T., Allanore Y., Bruni C., Codullo V., Dalm V., De Vries-Bouwstra J., Della Rossa A., Distler O., Galetti I., Launay D., Lepri G., Mathian A., Mouthon L., Ruaro B., Sulli A., Tincani A., Vandecasteele E., Vanhaecke A., Vanthuyne M., Van Den Hoogen F., Van Vollenhoven R., Voskuyl A. E., Zanatta E., Bombardieri S., Burmester G., Eurico F. J., Frank C., Hachulla E., Houssiau F., Mueller-Ladner U., Schneider M., Van Laar J. M., Vieira A., Cutolo M., Mosca M., and Matucci-Cerinic M.

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018

21. Systemic lupus erythematosus: State of the art on clinical practice guidelines

Author

Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., Costedoat-Chalumeau N., Tamirou, F, Arnaud, L, Talarico, R, Scire, C, Alexander, T, Amoura, Z, Avcin, T, Bortoluzzi, A, Cervera, R, Conti, F, Cornet, A, Devilliers, H, Doria, A, Frassi, M, Fredi, M, Govoni, M, Houssiau, F, Llado, A, Macieira, C, Martin, T, Massaro, L, Moraes-Fontes, M, Pamfil, C, Paolino, S, Tani, C, Tas, S, Tektonidou, M, Tincani, A, Van Vollenhoven, R, Bombardieri, S, Burmester, G, Eurico, F, Galetti, I, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Cutolo, M, Mosca, M, Costedoat-Chalumeau, N, Tamirou F., Arnaud L., Talarico R., Scire C. A., Alexander T., Amoura Z., Avcin T., Bortoluzzi A., Cervera R., Conti F., Cornet A., Devilliers H., Doria A., Frassi M., Fredi M., Govoni M., Houssiau F., Llado A., Macieira C., Martin T., Massaro L., Moraes-Fontes M. F., Pamfil C., Paolino S., Tani C., Tas S. W., Tektonidou M., Tincani A., Van Vollenhoven R. F., Bombardieri S., Burmester G., Eurico F. J., Galetti I., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Cutolo M., Mosca M., and Costedoat-Chalumeau N.

Abstract

Systemic lupus erythematosus (SLE) is the paradigm of systemic autoimmune diseases characterised by a wide spectrum of clinical manifestations with an unpredictable relapsing-remitting course. The aim of the present work was to identify current available clinical practice guidelines (CPGs) for SLE, to provide their review and to identify physicians' and patients' unmet needs. Twenty-Three original guidelines published between 2004 and 2017 were identified. Many aspects of disease management are covered, including global disease management, lupus nephritis and neuropsychiatric involvement, management of pregnancies, vaccinations and comorbidities monitoring. Unmet needs relate with disease management of some clinical manifestations and adherence to treatment. Many patient's unmet needs have been identified starting with faster diagnosis, need for more therapeutic options, guidelines on lifestyle issues, attention to quality of life and adequate education.

Published
2018

26. Long term outcomes of immunmodulatory drugs in SSc-ILD - data rom the German SSc network

Author

Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2020

27. Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network

Author

Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., Hunzelmann, N., Kreuter, M., Bonella, F., Blank, N., Siegert, E., Henes, J., Worm, M., Sunderkoetter, C., Schmalzing, M., Kreuter, A., Guenther, C., Susok, L., Zeidler, G., Koetter, I, Mueller-Ladner, U., Krieg, T., Juche, A., Schmeiser, T., Riemekasten, G., Aberer, E., Gaebelein-Wissing, N., Distler, J. H. W., Sardy, M., Pfeiffer, C., Kuhr, K., Lorenz, H. M., Moinzadeh, P., and Hunzelmann, N.

Published
2020

28. Large Variability of Frequency and Type of Physical Therapy in Patients in the German Network for Systemic Sclerosis

Author

Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., Hunzelmann, N., Belz, D., Moinzadeh, P., Riemekasten, G., Henes, J., Mueller-Ladner, U., Blank, N., Koetter, I, Siegert, E., Pfeiffer, C., Schmalzing, M., Zeidler, G., Schmeiser, T., Worm, M., Guenther, C., Susok, L., Kreuter, A., Sunderkoetter, C., Juche, A., Aberer, E., Gaebelein-Wissing, N., Ramming, A., Kuhr, K., and Hunzelmann, N.

Abstract

Objective To determine the type and frequency of physical therapy (PT) prescribed by physicians for patients in the registry of the German Network for Systemic Sclerosis. Methods The data for 4,252 patients were analyzed using descriptive statistics, chi-square tests, and odds ratios (ORs). Results Overall, 37.4% of patients (1,590 of 4,252) receivedPTat the end of a yearly follow-up. The most frequently used type ofPTwas lymphatic drainage (n = 1,061, 36.8%), followed by exercise therapy (n = 1,047, 36.3%) and heat therapy (n = 689, 23.9%). More than three-fourths of treated patients (82%) received 1 or 2 different forms ofPTsimultaneously. The prescription ofPTwas associated with the extent of skin fibrosis as measured by the modified Rodnan skin thickness score (<10 [41.8% of patients], 11-20 [55.8% of patients], and >21 [63.9% of patients];P< 0.001). Patients with musculoskeletal involvement (e.g., arthritis, muscle weakness, joint contractures, tendon friction rubs) had a higher chance of receivingPTthan patients without these symptoms, with correspondingORs ranging from 1.96 (95% confidence interval [95%CI] 1.69-2.28) for joint contractures to 3.83 (95%CI2.89-5.08) for arthritis. When comparing the type ofPTprescription across the initial and all follow-up visits from 2003 to 2017, significant alterations with a decreasing frequency of patients receivingPTcould be observed (P= 0.001). Conclusion To our knowledge, this is the first study reporting the use ofPTin patients with systemic sclerosis (SSc) in a large cohort. AlthoughSSc is characterized by considerable disability and restriction of motion, PT.

Published
2020

29. Treatment of systemic sclerosis-associated interstitial lung disease

Author

Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., Distler, J., Prasse, A., Bonella, F., Mueller-Ladner, U., Witte, T., Hunzelmann, N., and Distler, J.

Abstract

Background Systemic sclerosis (SSc) is a fibrosing autoimmune disease of the connective tissue. In addition to skin fibrosis, pulmonary involvement and interstitial lung disease (ILD) in particular are the most common and severe manifestations of SSc. The disease is associated with a substantial risk of morbidity and mortality, especially in progressive ILD. In the last 5 years new treatment concepts for SSc-ILD have been investigated in numerous clinical studies. Material and methods This review is based on a literature search in PubMed, focusing on the most relevant papers published up to the end of 2018 with the keywords SSc and treatment. Results The treatment of SSc-ILD has changed over the last few years due to the results of many clinical studies. The updated guidelines of the European League Against Rheumatism (EULAR) recommend the use of cyclophosphamide or hematopoietic stem cell transplantation. Data for a positive influence on SSc-ILD are also available for mycophenolate, tocilizumab and anabasum. Because of the pathophysiological similarities to idiopathic pulmonary fibrosis, the use of the antifibrotic agents nintedanib and pirfenidone is currently being investigated in randomized, multicenter clinical trials and could be a novel and promising therapeutic strategy. Conclusion Current drug studies may provide innovative therapeutic perspectives for SSc-ILD and could significantly improve the prognosis of affected patients in the future.

Published
2020

31. Idiopathic inflammatory myopathies: Narrative review of unmet needs in clinical practice guidelines

Author

Meyer A., Scire C. A., Talarico R., Alexander T., Amoura Z., Avcin T., Barsotti S., Beretta L., Blagojevic J., Burmester G., Cavazzana I., Cherrin P., Damian L., Doria A., Fonseca J. E., Furini F., Galetti I., Houssiau F., Krieg T., Maddalena L., Launay D., Campanilho-Marques R., Martin T., Matucci-Cerinic M., Moinzadeh P., Montecucco C., Moraes-Fontes M. F., Mouthon L., Neri R., Paolino S., Piette Y., Rednic S., Tamirou F., Tincani A., Toplak N., Bombardieri S., Hachulla E., Mueller-Ladner U., Schneider M., Smith V., Vieira A., Cutolo M., Mosca M., Cavagna L., Meyer, A, Scire, C, Talarico, R, Alexander, T, Amoura, Z, Avcin, T, Barsotti, S, Beretta, L, Blagojevic, J, Burmester, G, Cavazzana, I, Cherrin, P, Damian, L, Doria, A, Fonseca, J, Furini, F, Galetti, I, Houssiau, F, Krieg, T, Maddalena, L, Launay, D, Campanilho-Marques, R, Martin, T, Matucci-Cerinic, M, Moinzadeh, P, Montecucco, C, Moraes-Fontes, M, Mouthon, L, Neri, R, Paolino, S, Piette, Y, Rednic, S, Tamirou, F, Tincani, A, Toplak, N, Bombardieri, S, Hachulla, E, Mueller-Ladner, U, Schneider, M, Smith, V, Vieira, A, Cutolo, M, Mosca, M, and Cavagna, L

Subjects
myositi
Abstract

Idiopathic inflammatory myopathies (IIMs) encompass a heterogeneous group of rare autoimmune diseases characterised by muscle weakness and inflammation, but in antisynthetase syndrome arthritis and interstitial lung disease are more frequent and often inaugurate the disease. Clinical practice guidelines (CPGs) have been proposed for IIMs, but they are sparse and heterogeneous. This work aimed at identifying: i) current available CPGs for IIMs, ii) patients ' and clinicians' unmet needs not covered by CPGs. It has been performed in the framework of the European Reference Network on rare and complex connective tissue and musculoskeletal diseases (ReCONNET), a network of centre of expertise and patients funded by the European Union's Health Programme. Fourteen original CPGs were identified, notably recommending that: i) extra-muscular involvements should be assessed; ii) corticosteroids and methotrexate or azathioprine are first-line therapies of IIMs. ii) IVIG is a treatment of resistant-DM that may be also used in other resistant-IIMs; iii) physical therapy and sun protection (in DM patients) are part of the treatment; v) tumour screening for patients with DM include imaging of chest, abdomen, pelvis and breast (in woman) along with colonoscopy (in patients over 50 years); vi) disease activity and damages should be monitor using standardised and validated tools. Yet, only half of these CPGs were evidence-based. Crucial unmet needs were identified both by patients and clinicians. In particular, there was a lack of large multidisciplinary working group and of patients ' preferences. The following fields were not or inappropriately targeted: diagnosis; management of extra-muscular involvements other than skin; co-morbidities and severe manifestations.

Published
2018

32. Does anti-acid treatment influence disease progression in SSc-ILD? Data form the German SSc-network

Author

Kreuter, M, additional, Bonella, F, additional, Blank, N, additional, Siegert, E, additional, Henes, J, additional, Worm, M, additional, Sunderkoetter, C, additional, Schmalzing, M, additional, Kreuter, A, additional, Guenther, C, additional, Susok, L, additional, Zeidler, G, additional, Koetter, I, additional, Mueller-Ladner, U, additional, Krieg, T, additional, Juche, A, additional, Schmeiser, T, additional, Riemekasten, G, additional, Aberer, E, additional, Gaebelein-Wissing, N, additional, Distler, JHW, additional, Sárdy, M, additional, Pfeiffer, C, additional, Kuhr, K, additional, Lorenz, HM, additional, Moinzadeh, P, additional, and Hunzelmann, N, additional

Published
2020
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33. Long term outcomes of immunmodulatory drugs in SSc-ILD – data rom the German SSc network

Author

Kreuter, M, additional, Bonella, F, additional, Blank, N, additional, Siegert, E, additional, Henes, J, additional, Worm, M, additional, Sunderkoetter, C, additional, Schmalzing, M, additional, Kreuter, A, additional, Guenther, C, additional, Susok, L, additional, Zeidler, G, additional, Koetter, I, additional, Mueller-Ladner, U, additional, Krieg, T, additional, Juche, A, additional, Schmeiser, T, additional, Riemekasten, G, additional, Aberer, E, additional, Gaebelein-Wissing, N, additional, Distler, JHW, additional, Sárdy, M, additional, Pfeiffer, C, additional, Kuhr, K, additional, Lorenz, HM, additional, Moinzadeh, P, additional, and Hunzelmann, N, additional

Published
2020
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34. Characteristics of difficult-to-treat rheumatoid arthritis: results of an international survey

Author

Roodenrijs, N.M.T., Hair, M.J.H. de, Goes, M.C. van der, Jacobs, J.W.G., Welsing, P.M.J., Heijde, D. van der, Aletaha, D., Dougados, M., Hyrich, K.L., McInnes, I.B., Mueller-Ladner, U., Senolt, L., Szekanecz, Z., Laar, J.M. van, Nagy, G., and EULAR Recommendations

Subjects
musculoskeletal diseases, 0301 basic medicine, medicine.medical_specialty, Immunology, Comorbidity, Disease, Klinikai orvostudományok, General Biochemistry, Genetics and Molecular Biology, Arthritis, Rheumatoid, Disease activity, 03 medical and health sciences, 0302 clinical medicine, Rheumatology, Prednisone, Surveys and Questionnaires, Humans, Immunology and Allergy, Medicine, Intensive care medicine, 030203 arthritis & rheumatology, Polypharmacy, business.industry, International survey, Orvostudományok, medicine.disease, 030104 developmental biology, Current management, Rheumatoid arthritis, Rheumatologists, business, Rheumatism, medicine.drug
Abstract

ObjectivesPatients with difficult-to-treat rheumatoid arthritis (RA) remain symptomatic despite treatment according to current European League Against Rheumatism (EULAR) management recommendations. These focus on early phases of the disease and pharmacological management. We aimed to identify characteristics of difficult-to-treat RA and issues to be addressed in its workup and management that are not covered by current management recommendations.MethodsAn international survey was conducted among rheumatologists with multiple-choice questions on disease characteristics of difficult-to-treat RA. Using open questions, additional items to be addressed and items missing in current management recommendations were identified.Results410 respondents completed the survey: 50% selected disease activity score assessing 28 joints >3.2 OR presence of signs suggestive of active disease as characteristics of difficult-to-treat RA; 42% selected fatigue; 48% selected failure to ≥2 conventional synthetic disease-modifying antirheumatic drugs (DMARDs) AND ≥2 biological/targeted synthetic DMARDs; 89% selected inability to taper glucocorticoids below 5 mg or 10 mg prednisone equivalent daily. Interfering comorbidities, extra-articular manifestations and polypharmacy were identified as important issues missing in current management recommendations.ConclusionsThere is wide variation in concepts of difficult-to-treat RA. Several important issues regarding these patients are not addressed by current EULAR recommendations.

Published
2018

36. SAT0506 Ssc in older age: frequent and with a different phenotype. data of the german network for systemic sclerosis

Author

Moinzadeh, P., primary, Riemekasten, G., additional, Blank, N., additional, Henes, J., additional, Koetter, I., additional, Siegert, E., additional, Pfeiffer, C., additional, Zeidler, G., additional, Schmalzing, M., additional, Guenther, C., additional, Susok, L., additional, Worm, M., additional, Kreuter, A., additional, Sunderkoetter, C., additional, Mueller-Ladner, U., additional, Juche, A., additional, Aberer, E., additional, Schmeiser, T., additional, Krieg, T., additional, Kuhr, K., additional, and Hunzelmann, N., additional

Published
2018
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43. Systemic sclerosis: State of the art on clinical practice guidelines

Author

Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), Matucci-Cerinic, M. (Marco), Smith, V. (Vanessa), Scirè, C.A. (Carlo Alberto), Talarico, R. (Rosaria), Airo, P. (Paolo), Alexander, T. (Tobias), Allanore, Y. (Yannick), Bruni, C. (Cosimo), Codullo, V. (Veronica), Dalm, V.A.S.H. (Virgil), Vries-Bouwstra, J.K. (Jeska) de, Della Rossa, A. (Alessandra), Distler, O. (Oliver), Galetti, I. (Ilaria), Launay, D. (David), Lepri, G. (Gemma), Mathian, A. (Alexis), Mouthon, L. (Luc), Ruaro, B. (Barbara), Sulli, A. (Alberto), Tincani, A. (Angela), Vandecasteele, E. (Els), Vanhaecke, A. (Amber), Vanthuyne, M. (Marie), Hoogen, F.H.J. van den, Van Vollenhoven, R.F. (Ronald F.), Voskuyl, R.A. (Robert), Zanatta, E. (Elisabetta), Bombardieri, S. (Stefano), Burmester, G.R. (Gerd), Eurico, F.J. (Fonseca João), Frank, C. (Charissa), Hachulla, E. (Eric), Houssiau, F. (Frederic), Mueller-Ladner, U. (Ulf), Schneider, M. (Matthias), Van Laar, J.M. (Jacob M), Vieira, A. (Ana), Cutolo, M. (Maurizio), Mosca, M. (Marta), and Matucci-Cerinic, M. (Marco)

Abstract

Systemic sclerosis (SSc) is an orphan disease characterised by autoimmunity, fibrosis of the skin and internal organs, and vasculopathy. SSc may be associated with high morbidity and mortality. In this narrative review we summarise the results of a systematic literature research, which was performed as part of the European Reference Network on Rare and Complex Connective Tissue and Musculoskeletal Diseases project, aimed at evaluating existing clinical practice guidelines or recommendations. Only in the domains 'Vascular & Ulcers' (ie, non-pharmacological approach to digital ulcer), 'PAH' (ie, screening and treatment), 'Treatment' and 'Juveniles' (ie, evaluation of juveniles with Raynaud's phenomenon) evidence-based and consensus-based guidelines could be included. Hence there is a preponderance of unmet needs in SSc referring to the diagnosis and (non-)pharmacological treatment of several SSc-specific complications. Patients with SSc experience significant uncertainty concerning SSc-related taxonomy, management (both pharmacological and nonpharmacological) and education. Day-to-day impact of the disease (loss of self-esteem, fatigue, sexual dysfunction, and occupational, nutritional and relational problems) is underestimated and needs evaluation.

Published
2018
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44. There is a need for new systemic sclerosis subset criteria. A content analytic approach

Author

Johnson, S. R., Soowamber, M. L., Fransen, J., Khanna, D., Van den Hoogen, F., Baron, M., Matucci-Cerinic, M., Denton, C. P., Medsger, T. A., Jr., Carreira, P. E., Riemekasten, G., Distler, J., Gabrielli, A., Steen, V., Chung, L., Silver, R., Varga, J., Mueller-Ladner, U., Vonk, M. C., Walker, U. A., Wollheim, F. A., Herrick, A., Furst, D. E., Czirjak, L., Kowal-Bielecka, O., Del Galdo, F., Cutolo, M., Hunzelmann, N., Murray, C. D., Foeldvari, I., Mouthon, L., Damjanov, N., Kahaleh, B., Frech, T., Assassi, S., Saketkoo, L. A., Pope, J. E., Johnson, S. R., Soowamber, M. L., Fransen, J., Khanna, D., Van den Hoogen, F., Baron, M., Matucci-Cerinic, M., Denton, C. P., Medsger, T. A., Jr., Carreira, P. E., Riemekasten, G., Distler, J., Gabrielli, A., Steen, V., Chung, L., Silver, R., Varga, J., Mueller-Ladner, U., Vonk, M. C., Walker, U. A., Wollheim, F. A., Herrick, A., Furst, D. E., Czirjak, L., Kowal-Bielecka, O., Del Galdo, F., Cutolo, M., Hunzelmann, N., Murray, C. D., Foeldvari, I., Mouthon, L., Damjanov, N., Kahaleh, B., Frech, T., Assassi, S., Saketkoo, L. A., and Pope, J. E.

Abstract

Objectives. Systemic sclerosis (SSc) is heterogenous. The objectives of this study were to evaluate the purpose, strengths and limitations of existing SSc subset criteria, and identify ideas among experts about subsets. Methods. We conducted semi-structured interviews with randomly sampled international SSc experts. The interview transcripts underwent an iterative process with text deconstructed to single thought units until a saturated conceptual framework with coding was achieved and respondent occurrence tabulated. Serial cross-referential analyses of clusters were developed. Results. Thirty experts from 13 countries were included; 67% were male, 63% were from Europe and 37% from North America; median experience of 22.5years, with a median of 55 new SSc patients annually. Three thematic clusters regarding subsetting were identified: research and communication; management; and prognosis (prediction of internal organ involvement, survival). The strength of the limited/diffuse system was its ease of use, however 10% stated this system had marginal value. Shortcomings of the diffuse/limited classification were the risk of misclassification, predictions/generalizations did not always hold true, and that the elbow or knee threshold was arbitrary. Eighty-seven percent use more than 2 subsets including: SSc sine scleroderma, overlap conditions, antibody-determined subsets, speed of progression, and age of onset (juvenile, elderly). Conclusions. We have synthesized an international view of the construct of SSc subsets in the modern era. We found a number of factors underlying the construct of SSc subsets. Considerations for the next phase include rate of change and hierarchal clustering (e.g. limited/diffuse, then by antibodies).

Published
2018

45. SSC IN OLDER AGE: FREQUENT AND WITH A DIFFERENT PHENOTYPE. DATA OF THE GERMAN NETWORK FOR SYSTEMIC SCLEROSIS

Author

Moinzadeh, P., Riemekasten, G., Blank, N., Henes, J., Koetter, I., Siegert, E., Pfeiffer, C., Zeidler, G., Schmalzing, M., Guenther, C., Susok, L., Worm, M., Kreuter, A., Sunderkoetter, C., Mueller-Ladner, U., Juche, A., Aberer, E., Schmeiser, T., Krieg, T., Kuhr, K., Hunzelmann, N., Moinzadeh, P., Riemekasten, G., Blank, N., Henes, J., Koetter, I., Siegert, E., Pfeiffer, C., Zeidler, G., Schmalzing, M., Guenther, C., Susok, L., Worm, M., Kreuter, A., Sunderkoetter, C., Mueller-Ladner, U., Juche, A., Aberer, E., Schmeiser, T., Krieg, T., Kuhr, K., and Hunzelmann, N.

Published
2018

46. Diffusing capacity and clinical characteristics of patients with systemic sclerosis – data from the german network for systemic sclerosis

Author

Distler, JHW, Blank, N, Zeidler, G, Moinzadeh, P, Hunzelmann, N, Bonella, F, Kuhr, K, Riemekasten, G, Kreuter, M, Pfeiffer, C, Gaebelein-Wissing, N, Gunther, C, Sunderkoetter, C, Mueller-Ladner, U, Henes, J, Susok, L, Sárdy, M, Aberer, E, Schmeiser, T, Schmalzing, M, Worm, M, Siegert, E, Koetter, I, Krieg, T, Juche, A, and Kreuter, A

Subjects
Medizin
Published
2017

47. Inhibition of joint damage and improved clinical outcomes with rituximab plus methotrexate in early active rheumatoid arthritis: the IMAGE trial

Author

Tak, Pp, Rigby, Wf, Rubbert Roth, A, Peterfy, Cg, van Vollenhoven RF, Stohl, W, Hessey, E, Chen, A, Tyrrell, H, Shaw, Tm, Aelion J, IMAGE I. n. v. e. s. t. i. g. a. t. o. r. s., Afif, N, Ahmadi, F, Aires, F, Alanis, E, Alonso, Cs, Alten, Rh, Alvaro Gracia JM, Ashrafzadeh, A, Ballina, J, Bambara, Lm, Bao, C, Bell, M, Berney, S, Bessette, L, Birbara, C, Boling, E, Bourgeois, P, Braun, J, Briones, H, Brzezicki, J, Burgos Vargos, R, Burmester, G, Burnett, M, Busch, H, Cabello, E, Calvo, A, Cantagrel, A, Cantini, F, Zea, Ac, Carreño Perez, L, Chavez, J, Shim, Sc, Chindalore, V, Chiriac, R, Codding, C, Danda, D, Del Guidice, J, De Vita, S, Digiovanni, R, Dikranian, A, Eider, W, Fantini, F, Ferraccioli, G, Fietchner, J, Filipowicz Sosnowska, A, Finnanger, B, Fiocco, G, Fleck, M, Fleischmann, R, Fraser, A, Gaudin, P, Gauler, G, Gaylis, N, Gerlag, Dm, Godde, J, Gomez Reino JJ, Gornisiewicz, M, Gough, W, Greenwald, M, Guerra, G, Hackshaw, K, Haentzschel, Hm, Hammond, T, Hazleman, Bl, Heilig, B, Herenius, Mm, Hilliquin, P, Holt, D, Huang, F, Huff, J, Huizinga, T, Isaacs, J, Jaffer, A, Amante, Ej, Jeka, S, Jimenez, R, Jones, G, Jones, R, Kaine, J, Kashif, A, Kaufmann, C, Kay, J, Khraishi, M, Kivitz, A, Klinkhoff, A, Kraag, G, Krystufkova, O, Kucharz, E, Lawson, J, Leirisalo Repo, M, Levin, R, Liang, G, Liang, P, Limonta, M, Lowenstein, M, Rodriguez Lozano, C, Lue, C, Mahowald, M, Maradiaga, M, Maricic, M, Mariette, X, Martin, L, Massarotti, E, Matucci Cerinic, M, Montecucco, Cm, Mazurov, V, Mcnally, J, Mehta, D, Meyer, O, Misra, R, Moreland, Lw, Mueller Ladner, U, Myerson, G, Nasonov, E, Navarra, S, Navarro, F, Neal, N, Olech, E, Olsen, N, Pablos, Jl, Pacheco, C, Pal, S, Palomo, Er, Pandith, V, Penserga, Eg, Prupas, H, Radominski, S, Ramos Remus, C, Reid, D, Riordan, K, Rosenberg, D, Ruiz, A, Saadeh, C, Salvarani, Carlo, Samuels, A, Sanmarti, R, Sarzi Puttini, P, Saxe, P, Schechtman, J, Scoville, C, Sedlackova, M, Sedrish, M, Sejer Hansen, M, Sibilia, J, Siebert, S, Specker, C, Stern, S, Szechinski, J, Tahir, H, Taylor, A, Thompson, Pw, Tony, Hp, Tornero, J, Trapp, R, Tremblay, Jl, Valesini, G, Van Den Bosch, F, Wanchu, A, Wassenberg, S, Ximenes, Ac, Kim, Hy, Zanetakis, E, Zazueta, B, Zerbini, C., Faculteit der Geneeskunde, AII - Amsterdam institute for Infection and Immunity, Clinical Immunology and Rheumatology, and Other departments

Subjects
Adult, musculoskeletal diseases, medicine.medical_specialty, Adolescent, Immunology, 610 Medizin, Arthritis, Severity of Illness Index, Gastroenterology, Drug Administration Schedule, General Biochemistry, Genetics and Molecular Biology, law.invention, Arthritis, Rheumatoid, Antibodies, Monoclonal, Murine-Derived, Young Adult, Double-Blind Method, Rheumatology, Randomized controlled trial, law, immune system diseases, Internal medicine, medicine, Clinical endpoint, Humans, Immunology and Allergy, heterocyclic compounds, skin and connective tissue diseases, Aged, Aged, 80 and over, ddc:610, business.industry, Middle Aged, medicine.disease, Surgery, Clinical trial, Methotrexate, Treatment Outcome, Antirheumatic Agents, Rheumatoid arthritis, Disease Progression, Drug Therapy, Combination, Rituximab, business, medicine.drug
Abstract

Objectives: Rituximab is an effective treatment in patients with established rheumatoid arthritis (RA). The objective of the IMAGE study was to determine the efficacy of rituximab in the prevention of joint damage and its safety in combination with methotrexate (MTX) in patients initiating treatment with MTX. Methods: In this double-blind randomised controlled phase III study, 755 MTX-naïve patients with active RA were randomly assigned to MTX alone, rituximab 2×500 mg + MTX or rituximab 2×1000 mg + MTX. The primary end point at week 52 was the change in joint damage measured using a Genant-modified Sharp score. Results: 249, 249 and 250 patients were randomly assigned to MTX alone, rituximab 2×500 mg + MTX or rituximab 2×1000 mg + MTX, respectively. At week 52, treatment with rituximab 2×1000 mg + MTX compared with MTX alone was associated with a reduction in progression of joint damage (mean change in total modified Sharp score 0.359 vs 1.079; p=0.0004) and an improvement in clinical outcomes (ACR50 65% vs 42%; p

Published
2011
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49. Vasoactive Therapy in Systemic Sclerosis

Author

Moinzadeh, P., Riemekasten, G., Siegert, E., Fierlbeck, G., Henes, J., Blank, N., Melchers, I., Mueller-Ladner, U., Frerix, M., Kreuter, A., Tigges, C., Lahner, N., Susok, L., Guenther, C., Zeidler, G., Pfeiffer, C., Worm, M., Karrer, S., Aberer, E., Bretterklieber, A., Genth, E., Simon, J.C., Distler, J.H.W., Hein, R., Schneider, M., Seitz, C.S., Herink, C., Steinbrink, K., Sárdy, M., Varga, R., Mensing, H., Mensing, C., Lehmann, P., Neeck, G., Fiehn, C., Weber, M., Goebeler, M., Burkhardt, H., Buslau, M., Ahmadi-Simab, K., Himsel, A., Juche, A., Koetter, I., Kuhn, A., Sticherling, M., Hellmich, M., Kuhr, K., Krieg, T., Ehrchen, J., Sunderkoetter, C., Hunzelmann, N., and Publica

Abstract

Objective: Vasculopathy is a key factor in the pathophysiology of systemic sclerosis (SSc) and the main cause for Raynaud phenomenon (RP), digital ulcers (DU), and/or pulmonary arterial hypertension (PAH). It is so far unknown how patients with SSc are treated with vasoactive agents in daily practice. To determine to which extent patients with SSc were treated with different vasoactive agents, we used data from the German Network for Systemic Scleroderma registry. Methods: The data of 3248 patients with SSc were analyzed. Results: Patients were treated with vasoactive drugs in 61.1% of cases (1984/3248). Of these, 47.6% received calcium channel inhibitors, followed by 34.2% treated with angiotensin-converting enzyme (ACE) inhibitors, 21.1% treated with intravenous (IV) prostanoids, 10.1% with pentoxifylline, 8.8% with angiotensin 1 receptor antagonists (AT1RA), 8.7% with endothelin 1 receptor antagonists (ET1RA), 4.1% with phosphodiesterase type 5 (PDE5) inhibitors, and 5.3% with others. Patients with RP received vasoactive therapy in 63.3% of cases, with DU in 70.1%, and with PAH in 78.2% of cases. Logistic regression analysis revealed that patients with PAH were significantly more often treated with PDE5 inhibitors and ET1RA, and those with DU with ET1RA and IV prostanoids. In addition, 41.8% of patients were treated with ACE inhibitors and/or AT1RA. Patients registered after 2009 received significantly more often ET1RA, AT1RA, and IV prostanoids compared with patients registered prior to 2005. Conclusion: These data clearly indicate that many patients with SSc do not yet receive sufficient vasoactive therapy. Further, in recent years, a marked change of treatment regimens can be observed.

Published
2016

50. AB0181 Diffusing capacity and clinical characteristics of patients with systemic sclerosis – data from the german network for systemic sclerosis

Author

Moinzadeh, P, primary, Blank, N, additional, Siegert, E, additional, Henes, J, additional, Worm, M, additional, Sunderkoetter, C, additional, Schmalzing, M, additional, Kreuter, A, additional, Gunther, C, additional, Susok, L, additional, Zeidler, G, additional, Koetter, I, additional, Mueller-Ladner, U, additional, Krieg, T, additional, Juche, A, additional, Schmeiser, T, additional, Riemekasten, G, additional, Aberer, E, additional, Gaebelein-Wissing, N, additional, Distler, JHW, additional, Sárdy, M, additional, Pfeiffer, C, additional, Kuhr, K, additional, Hunzelmann, N, additional, Bonella, F, additional, and Kreuter, M, additional

Published
2017
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