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1. Prevalence and gender - specific analysis of a systemic sclerosis cohort in Latvia

Author

Kristine Ivanova, Olga Ribakova, Anna Mihailova, Evelina Mozeitovica, Anda Kadisa, Julija Zepa, Viktorija Kenina, Natalja Kurjane, and Inita Bulina

Subjects
Systemic sclerosis, Demography, Latvia, Prevalence of systemic sclerosis, Rhaynauds’s phenomen, Modified Rodnan skin score, Medicine
Abstract

Abstract Background Systemic sclerosis (SSc) is considered by many to be one of the most severe autoimmune rheumatic diseases with lower prevalence observed in Northern Europe. No previous studies on the prevalence of SSc in Latvia have been conducted and the aim was to study the demographic and clinical data of patients with SSc in northeastern Europe country. Methods This study was conducted in two main Latvian hospitals for adults and includes patients with SSc who were consulted between 2016 and 2021. Results During the study period, 159 patients with SSc were consulted. The point prevalence on 1 January 2021 was 84.0 per million. Female to male ratio was 4.67:1, and highest gender ratio was observed in the age group 70–79-year (6.75:1). Antinuclear antibodies were present in 82.58% of patients, without gender difference. Centromere pattern was more frequently observed in females (40.19% vs. 19.04%), in contrast to speckled pattern (50.98% vs. 57.14%). At disease onset females tended to be younger (46.51 ± 13.52) than males (50.5 ± 16.64). Males had more diffuse cutaneous subtype, interstitial lung disease, pulmonary hypertension and esophageal dysmotility. More than half of patients received treatment with glucocorticoids at any point of the disease (68.31%), without gender difference. Conclusions Systemic sclerosis is less common in Latvia than in other countries and regions. Due to its location, the data from Latvia are consistent with a north-south gradient in Europe. Gender ratio differences persisted in older age groups as well. Antinuclear antibodies presence did not differ between genders, but in female’s centromere pattern was much more likely to be present. Males had more severe disease course, but in both genders more than half of patients received treatment with GCs at any point of the disease.

Published
2024
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2. Prolonged COVID-19 symptom duration in people with systemic autoimmune rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

Author

Francis Berenbaum, Jasvinder A Singh, Ali Duarte-García, Jinoos Yazdany, Pedro M Machado, Namrata Singh, Deshire Alpizar-Rodriguez, Zachary S Wallace, Eimear Duff, Rebecca Grainger, Tamer A Gheita, Elizabeth R Graef, Jean W Liew, Michael S Putman, Julia F Simard, Emily Sirotich, Carly Harrison, Philip C Robinson, Sebastian E Sattui, Jeffrey A Sparks, Gary Foster, Suleman Bhana, Wendy Costello, Jonathan S Hausmann, Paul Sufka, Richard Conway, Akpabio Akpabio, Michal Nudel, Manuel F Ugarte-Gil, Michael DiIorio, Mitchell Levine, Evelyn Hsieh, Richard A Howard, John Wallace, Inita Bulina, Kevin Kennedy, Tarin T Moni, Aman Dev Singh, Lina El Kibbi, Chieh Lo, David FL Liew, Monique Gore-Massy, Maggie J Larché, More A Kodhek, Nadine Lalonde, Laura-Ann Tomasella, Richard P Beesley, and Eugenia Yupei Chock

Subjects
Medicine
Abstract

Objective We investigated prolonged COVID-19 symptom duration, defined as lasting 28 days or longer, among people with systemic autoimmune rheumatic diseases (SARDs).Methods We analysed data from the COVID-19 Global Rheumatology Alliance Vaccine Survey (2 April 2021–15 October 2021) to identify people with SARDs reporting test-confirmed COVID-19. Participants reported COVID-19 severity and symptom duration, sociodemographics and clinical characteristics. We reported the proportion experiencing prolonged symptom duration and investigated associations with baseline characteristics using logistic regression.Results We identified 441 respondents with SARDs and COVID-19 (mean age 48.2 years, 83.7% female, 39.5% rheumatoid arthritis). The median COVID-19 symptom duration was 15 days (IQR 7, 25). Overall, 107 (24.2%) respondents had prolonged symptom duration (≥28 days); 42/429 (9.8%) reported symptoms lasting ≥90 days. Factors associated with higher odds of prolonged symptom duration included: hospitalisation for COVID-19 vs not hospitalised and mild acute symptoms (age-adjusted OR (aOR) 6.49, 95% CI 3.03 to 14.1), comorbidity count (aOR 1.11 per comorbidity, 95% CI 1.02 to 1.21) and osteoarthritis (aOR 2.11, 95% CI 1.01 to 4.27). COVID-19 onset in 2021 vs June 2020 or earlier was associated with lower odds of prolonged symptom duration (aOR 0.42, 95% CI 0.21 to 0.81).Conclusion Most people with SARDs had complete symptom resolution by day 15 after COVID-19 onset. However, about 1 in 4 experienced COVID-19 symptom duration 28 days or longer; 1 in 10 experienced symptoms 90 days or longer. Future studies are needed to investigate the possible relationships between immunomodulating medications, SARD type/flare, vaccine doses and novel viral variants with prolonged COVID-19 symptoms and other postacute sequelae of COVID-19 among people with SARDs.

Published
2022
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3. An overlook on the current registries for rare and complex connective tissue diseases and the future scenario of TogethERN ReCONNET

Author

Matilde Bandeira, Federica Di Cianni, Diana Marinello, Laurent Arnaud, Sara Cannizzo, Claudio Carta, Alain Cornet, Sara M. Barril, Inita Bulina, Alessandro Ferraris, João Fonseca, Andrea Gaglioti, Marteen Limper, Valentina Lorenzoni, Judith Majnik, Marco Matucci-Cerinic, Ilaria Palla, Simona Rednic, Matthias Schneider, Vanessa Smith, Alberto Sulli, Klaus Søndergaard, Simone Ticciati, Angela Tincani, Giuseppe Turchetti, Rosaria Talarico, Maurizio Cutolo, Marta Mosca, and Domenica Taruscio

Subjects
rare and complex connective tissue diseases, registries, ERN ReCONNET, TogethERN ReCONNET, European Reference Networks, Medicine (General), R5-920
Abstract

BackgroundPatient registries play a crucial role in supporting clinical practice, healthcare planning and medical research, offering a real-world picture on rare and complex connective tissue diseases (rCTDs). ERN ReCONNET launched the first European Registry Infrastructure with the aim to plan, upgrade and link registries for rCTDs, with the final goal to promote a harmonized data collection approach all over Europe for rCTDs.MethodsAn online survey addressed to healthcare professionals and patients' representatives active in the field of rCTDs was integrated by an extensive database search in order to build a mapping of existing registries for rCTDs.FindingsA total of 140 registries were found, 38 of which include multiple diseases. No disease-specific registry was identified for relapsing polychondritis, mixed connective tissue disease and undifferentiated connective tissue disease.DiscussionThis overview on the existing registries for rCTDs provides a useful starting point to identify the gaps and the strengths of registries on the coverage of rCTDs, and to develop a common data set and data collection approach for the establishment of the TogethERN ReCONNET Infrastructure.

Published
2022
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4. SARS-CoV-2 vaccine safety in adolescents with inflammatory rheumatic and musculoskeletal diseases and adults with juvenile idiopathic arthritis: data from the EULAR COVAX physician-reported registry

Author

Gözde Kübra Yardımcı, Eric Hachulla, Laure Gossec, Loreto Carmona, Patrick Durez, Pedro M Machado, Eric Toussirot, Umut Kalyoncu, Xavier Mariette, Simona Rednic, Viviane Queyrel, Raphaele Seror, Anja Strangfeld, Kimme L Hyrich, Saskia Lawson-Tovey, Julija Zepa, Nicolas Roux, Bernd Raffeiner, Daniel Clemente, Theodoros Dimitroulas, Inita Bulina, Emőke Šteňová, Eva Strakova, Pavol Masaryk, Elsa Mateus, Ana M. Rodrigues, Amelie Leurs, Anna Carbone, Jose AG Puerta, Natalia de la Torre Rubio, Rachel Jeffrey, Sharon Floyd, and Vanda Mylnarikova

Subjects
Medicine
Abstract

Background There is a lack of data on SARS-CoV-2 vaccination safety in children and young people (CYP) with rheumatic and musculoskeletal diseases (RMDs). Current vaccination guidance is based on data from adults with RMDs or CYP without RMDs.Objectives To describe the safety of SARS-COV-2 vaccination in adolescents with inflammatory RMDs and adults with juvenile idiopathic arthritis (JIA).Methods We described patient characteristics, flares and adverse events (AEs) in adolescent cases under 18 with inflammatory RMDs and adult cases aged 18 or above with JIA submitted to the European Alliance of Associations for Rheumatology COVAX registry.Results A total of 110 cases were reported to the registry. Thirty-six adolescent cases were reported from four countries, most with JIA (42%). Over half (56%) reported early reactogenic-like AEs. One mild polyarthralgia flare and one serious AE of special interest (malaise) were reported. No CYP reported SARS-CoV-2 infection postvaccination.Seventy-four adult JIA cases were reported from 11 countries. Almost two-thirds (62%) reported early reactogenic-like AEs and two flares were reported (mild polyarthralgia and moderate uveitis). No serious AEs of special interest were reported among adults with JIA. Three female patients aged 20–30 years were diagnosed with SARS-CoV-2 postvaccination; all fully recovered.Conclusions This is an important contribution to research on SARS-CoV-2 vaccine safety in adolescents with RMDs and adults with JIA. It is important to note the low frequency of disease flares, serious AEs and SARS-CoV-2 reinfection seen in both populations, although the dataset is limited by its size.

Published
2022
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5. SARS-CoV-2 breakthrough infections among vaccinated individuals with rheumatic disease: results from the COVID-19 Global Rheumatology Alliance provider registry

Author

Gözde Kübra Yardımcı, Laure Gossec, Loreto Carmona, Zara Izadi, Jinoos Yazdany, Pedro M Machado, Anja Strangfeld, Rebecca Grainger, Emily Sirotich, Carly Harrison, Philip C Robinson, Jean Liew, Milena Gianfrancesco, Kimme L Hyrich, Lindsay Jacobsohn, Saskia Lawson-Tovey, Stephanie Rush, Suleman Bhana, Paul Sufka, Julija Zepa, Abdurrahman Tufan, Martin Schäfer, Claire Cook, Fatemah Abutiban, Jeffrey Sparks, Leanna Wise, Zachary Wallace, Naomi J Patel, Samar Al Emadi, Inita Bulina, Christine Graver, Emily Kowalski, Jonathan Hausmann, Clairissa Ja, Elsa Frãzao-Mateus, Frances Stafford, Dfiza Dey, Genevieve Katigbak, Lauren Kaufman, Marco Ulises Martínez-Martínez, Greta Reyes-Cordero, Evelyn Salido, Ellison Smith, David Snow, and Monique Gore-Massy

Subjects
Medicine
Published
2022
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6. Early experience of COVID-19 vaccination in adults with systemic rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

Author

Jasvinder A Singh, Jinoos Yazdany, Namrata Singh, Zachary S Wallace, Bruce Miller, Maggie Larche, Lisa G Rider, Eimear Duff, Rebecca Grainger, Tamer A Gheita, Jean W Liew, Emily Sirotich, Carly Harrison, Philip C Robinson, Kristen J Young, Jeffrey A Sparks, Gary Foster, Suleman Bhana, Wendy Costello, Jonathan S Hausmann, Paul Sufka, Alfred Hyoungju Kim, Akpabio Akpabio, Michal Nudel, Catherine L Hill, Serena Mingolla, Karen L Durrant, Mitchell Levine, Evelyn Hsieh, Richard A Howard, John Wallace, Inita Bulina, Adam Kilian, Sebastian Eduardo Sattui, Kevin Kennedy, Michael Putman, Tarin T Moni, Aman Dev Singh, Lina El Kibbi, David F L Liew, Chieh Lo, and Candace A Palmerlee

Subjects
Medicine
Published
2021
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7. COVID-19 vaccine perceptions and uptake: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

Author

Michael Putman, Kevin Kennedy, Emily Sirotich, Jean W Liew, Sebastian E Sattui, Tarin T Moni, Akpabio A Akpabio, Deshire Alpizar-Rodriguez, Saskya Angevare, Richard P Beesley, Francis Berenbaum, Inita Bulina, Yu Pei Eugenia Chock, Richard Conway, Ali Duarte-García, Aman Dev Singh, Eimear Duff, Karen L Durrant, Tamer A Gheita, Catherine L Hill, Richard Howard, Bimba F Hoyer, Evelyn Hsieh, Lina el Kibbi, Adam Kilian, Alfred H J Kim, David F L Liew, Chieh Lo, Elsa F Mateus, Bruce Miller, Serena Mingolla, Michal Nudel, Jasvinder A Singh, Namrata Singh, Manuel F Ugarte-Gil, John Wallace, Kristen J Young, Erick Adrian Zamora-Tehozol, Suleman Bhana, Wendy Costello, Rebecca Grainger, Pedro M Machado, Philip C Robinson, Paul Sufka, Zachary S Wallace, Jinoos Yazdany, Carly Harrison, Maggie J Larché, Mitchell Levine, Gary Foster, Lehana Thabane, Jonathan S Hausmann, Jeffrey A Sparks, and Julia F Simard

Subjects
Rheumatology, Immunology, Immunology and Allergy
Published
2022
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8. P61 SARS-CoV-2 vaccine safety in adolescents with inflammatory rheumatic and musculoskeletal diseases and adults with juvenile idiopathic arthritis: data from the EULAR COVAX physician-reported registry

Author

Saskia Lawson-Tovey, Pedro Machado, Anja Strangfeld, Elsa Mateus, Laure Gossec, Loreto Carmona, Bernd Raffeiner, Inita Bulina, Daniel Clemente, Julija Zepa, Ana Rodrigues, Xavier Mariette, and Kimme Hyrich

Subjects
Rheumatology
Abstract

Introduction/Background There is a lack of data on Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) vaccination safety in children and young people (CYP) with rheumatic and musculoskeletal diseases (RMDs) as they were excluded from initial vaccine trials. Vaccination guidance is based on data from adults with or CYP without RMDs. Description/Method Our objective was to describe the safety of SARS-COV-2 vaccination in adolescents with inflammatory RMDs and adults with JIA. We described patient characteristics, flares, and adverse events in adolescent cases under 18 with inflammatory RMDs and adult cases aged 18 or above with JIA submitted to the European Alliance of Associations for Rheumatology (EULAR) COVAX registry. Discussion/Results Thirty-six adolescent cases were reported from 4 countries, mostly female (58%) with JIA (42%: 28% non-systemic JIA, 14% systemic JIA) and a median age of 15 [IQR: 14.5, 17]. Most were in remission (64%) or had minimal (22%) disease activity at the time of vaccination. Over half of the adolescent group (56%) reported early reactogenic-like AEs. One mild polyarthralgia flare and one serious AE of special interest (malaise) were reported. No CYP reported SARS-CoV-2 infection post-vaccination. No cases of paediatric inflammatory multi-system syndrome or myocarditis adverse events were reported. Seventy-four adult JIA cases were reported from 11 countries; 73% were female with a median age of 26 [IQR: 23, 31]. Eight-five percent had ns-JIA and 15% had s-JIA. Almost two thirds (62%) reported early reactogenic-like AEs and two flares were reported (mild polyarthralgia and moderate uveitis). No serious AEs of special interest were reported among adults with JIA. Three 20-30 year old females were diagnosed with SARS-CoV-2 post-vaccination; all fully recovered. Key learning points/Conclusion In this observational registry dataset, SARS-CoV-2 vaccines appeared safe in adolescents with RMDs and adults with JIA, with a low frequency of disease flares, serious AEs, and SARS-CoV-2 re-infection seen in both populations.

Published
2022
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9. Environmental and societal factors associated with COVID-19-related death in people with rheumatic disease: an observational study

Author

Zara Izadi, Milena A Gianfrancesco, Gabriela Schmajuk, Lindsay Jacobsohn, Patricia Katz, Stephanie Rush, Clairissa Ja, Tiffany Taylor, Kie Shidara, Maria I Danila, Katherine D Wysham, Anja Strangfeld, Elsa F Mateus, Kimme L Hyrich, Laure Gossec, Loreto Carmona, Saskia Lawson-Tovey, Lianne Kearsley-Fleet, Martin Schaefer, Samar Al-Emadi, Jeffrey A Sparks, Tiffany Y-T Hsu, Naomi J Patel, Leanna Wise, Emily Gilbert, Alí Duarte-García, Maria O Valenzuela-Almada, Manuel F Ugarte-Gil, Lotta Ljung, Carlo A Scirè, Greta Carrara, Eric Hachulla, Christophe Richez, Patrice Cacoub, Thierry Thomas, Maria J Santos, Miguel Bernardes, Rebecca Hasseli, Anne Regierer, Hendrik Schulze-Koops, Ulf Müller-Ladner, Guillermo Pons-Estel, Romina Tanten, Romina E Nieto, Cecilia N Pisoni, Yohana S Tissera, Ricardo Xavier, Claudia D Lopes Marques, Gecilmara C S Pileggi, Philip C Robinson, Pedro M Machado, Emily Sirotich, Jean W Liew, Jonathan S Hausmann, Paul Sufka, Rebecca Grainger, Suleman Bhana, Monique Gore-Massy, Zachary S Wallace, Jinoos Yazdany, Brahim Dahou, Gimena Gómez, Karen Roberts, Roberto M Baez, Vanessa V Castro Coello, María J Haye Salinas, Federico N Maldonado, Alvaro A Reyes, Gelsomina Alle, Hernán Maldonado Ficco, Romina Nieto, Carla Gobbi, Yohana Tissera, Cecilia Pisoni, Alba Paula, Juan A Albiero, Maria M Schmid, Micaela Cosatti, Maria J Gamba, Carlevaris Leandro, María A Cusa, Noelia German, Veronica Bellomio, Lorena Takashima, Mariana Pera, Karina Cogo, Maria S Gálvez Elkin, María A Medina, Veronica Savio, Romina Rojas Tessel, Rodolfo P Alamino, Marina L Werner, Sofía Ornella, Luciana Casalla, Maria de la Vega, María Severina, Mercedes García, Luciana Gonzalez Lucero, Cecilia Romeo, Sebastián Moyano, Tatiana Barbich, Ana Bertoli, Andrea Baños, Sandra Petruzzelli, Carla Matellan, Silvana Conti, Maria A Lazaro, Gustavo F Rodriguez Gil, Fabian Risueño, Maria I Quaglia, Julia Scafati, Natalia L Cuchiaro, Jonathan E Rebak, Susana I Pineda, María E Calvo, Eugenia Picco, Josefina G Yanzi, Pablo Maid, Debora Guaglianone, Julieta S Morbiducci, Sabrina Porta, Natalia Herscovich, José L Velasco Zamora, Boris Kisluk, Maria S Castaños Menescardi, Rosana Gallo, María V Martire, Carla Maldini, Cecilia Goizueta, Sabrina S de la Vega Fernandez, Carolina Aeschlimann, Gisela Subils, Eva Rath, Yves Piette, Mieke Devinck, Bea Maeyaert, Francinne Machado Ribeiro, Sandra L Euzebio Ribeiro, Marcelo Pinheiro, Sebastián Ibáñez, Anne-Marie Chassin Trubert, Lingli Dong, Lui Cajas, Marko Barešić, Branimir Anić, Melanie-Ivana Ćulo, Tea A Pavelić, Kristina K Stranski, Boris Karanovic, Jiri Vencovsky, Marta Píchová, Maria Filkova, Hesham Hamoud, Dimitrios Vassilopoulos, Gabriela M Guzman Melgar, Ho So, Márta Király, Mahdi Vojdanian, Alexandra Balbir Gurman, Fatemah Abutiban, Julija Zepa, Inita Bulina, Loreta Bukauskiene, Beatriz E Zazueta Montiel, Angel A Castillo Ortiz, Erick Zamora Tehozol, David Vega Morales, Diana Cervántes Rosete, Eduardo Martín Nares, Tatiana S Rodriguez Reyna, Marina Rull Gabayet, Deshiré Alpízar Rodríguez, Fedra Irazoque, Xochitl Jimenez, Lenny Geurts van Bon, Theo Zijlstra, Monique Hoekstra, Nasra Al Adhoubi, Babur Salim, Enrique Giraldo, Ariel Salinas, Manuel Ugarte Gil, Jarosław Nowakowski, Richard Conway, Rachael Flood, Geraldine McCarthy, Ioana Felea, Ileana Filipescu, Simona Rednic, Laura Groseanu, Maria M Tamas, Vanda Mlynarikova, Martina Skamlova, Martin Zlnay, Dagmar Mičeková, Lubica Capova, Zelmira Macejova, Emőke Šteňová, Helena Raffayova, Gabriela Belakova, Eva Strakova, Marieta Senčarová, Soňa Žlnayová, Anna Sabová, Daniela Spisakova, Mária Oetterová, Olga Lukacova, Martina Bakosova, Alojzija Hocevar, Natalia de la Torre Rubio, Juan J Alegre Sancho, Montserrat Corteguera Coro, Juan C Cobeta Garcia, Maria C Torres Martin, Jose Campos, Jose A Gomez Puerta, Gozd K Yardimci, Servet Akar, Ozan C Icacan, Selda ÇELİK, Viktoriia Vasylets, Su-Ann Yeoh, Claire Vandevelde, Sasha Dunt, Jane Leeder, Elizabeth Macphie, Rosaria Salerno, Christine Graver, Katie Williams, Sheila O'Reilly, Kirsty Devine, Jennifer Tyler, Elizabeth Warner, James Pilcher, Samir Patel, Elena Nikiphorou, Laura Chadwick, Caroline M Jones, Beverley Harrison, Lucy Thornton, Diana O'Kane, Lucia Fusi, Audrey Low, Sarah Horton, Shraddha Jatwani, Sara Baig, Hammad Bajwa, Vernon Berglund, Angela Dahle, Walter Dorman, Jody Hargrove, Maren Hilton, Nicholas Lebedoff, Susan Leonard, Jennifer Morgan, Emily Pfeifer, Archibald Skemp, Jeffrey Wilson, Anne Wolff, Eduardo Cepeda, Derrick Todd, Denise Hare, Cassandra Calabrese, Christopher Adams, Arezou Khosroshahi, Adam Kilian, Douglas White, Melanie Winter, Theodore Fields, Caroline Siegel, Nicole Daver, Melissa Harvey, Neil Kramer, Concetta Lamore, Suneya Hogarty, Karen Yeter, Faizah Siddique, Byung Ban, Tamar Tanner, Eric Ruderman, William Davis, Robert Quinet, Evangeline Scopelitis, Karen Toribio, Tameka Webb Detiege, Jerald Zakem, Khurram Abbass, Gilbert Kepecs, Lilliam Miranda, Michael Guma, Ammar Haikal, Sushama Mody, Daric Mueller, Arundathi Jayatilleke, JoAnn Zell, Alison Bays, Kathryn Dao, Fatemeh Ezzati, Deborah Parks, David Karp, Guillermo Quiceno, Izadi, Z, Gianfrancesco, M, Schmajuk, G, Jacobsohn, L, Katz, P, Rush, S, Ja, C, Taylor, T, Shidara, K, Danila, M, Wysham, K, Strangfeld, A, Mateus, E, Hyrich, K, Gossec, L, Carmona, L, Lawson-Tovey, S, Kearsley-Fleet, L, Schaefer, M, Al-Emadi, S, Sparks, J, Hsu, T, Patel, N, Wise, L, Gilbert, E, Duarte-Garcia, A, Valenzuela-Almada, M, Ugarte-Gil, M, Ljung, L, Scire, C, Carrara, G, Hachulla, E, Richez, C, Cacoub, P, Thomas, T, Santos, M, Bernardes, M, Hasseli, R, Regierer, A, Schulze-Koops, H, Muller-Ladner, U, Pons-Estel, G, Tanten, R, Nieto, R, Pisoni, C, Tissera, Y, Xavier, R, Lopes Marques, C, Pileggi, G, Robinson, P, Machado, P, Sirotich, E, Liew, J, Hausmann, J, Sufka, P, Grainger, R, Bhana, S, Gore-Massy, M, Wallace, Z, Yazdany, J, Dahou, B, Gomez, G, Roberts, K, Baez, R, Castro Coello, V, Haye Salinas, M, Maldonado, F, Reyes, A, Alle, G, Maldonado Ficco, H, Gobbi, C, Paula, A, Albiero, J, Schmid, M, Cosatti, M, Gamba, M, Leandro, C, Cusa, M, German, N, Bellomio, V, Takashima, L, Pera, M, Cogo, K, Galvez Elkin, M, Medina, M, Savio, V, Rojas Tessel, R, Alamino, R, Werner, M, Ornella, S, Casalla, L, de la Vega, M, Severina, M, Garcia, M, Gonzalez Lucero, L, Romeo, C, Moyano, S, Barbich, T, Bertoli, A, Banos, A, Petruzzelli, S, Matellan, C, Conti, S, Lazaro, M, Rodriguez Gil, G, Risueno, F, Quaglia, M, Scafati, J, Cuchiaro, N, Rebak, J, Pineda, S, Calvo, M, Picco, E, Yanzi, J, Maid, P, Guaglianone, D, Morbiducci, J, Porta, S, Herscovich, N, Velasco Zamora, J, Kisluk, B, Castanos Menescardi, M, Gallo, R, Martire, M, Maldini, C, Goizueta, C, de la Vega Fernandez, S, Aeschlimann, C, Subils, G, Rath, E, Piette, Y, Devinck, M, Maeyaert, B, Machado Ribeiro, F, Euzebio Ribeiro, S, Pinheiro, M, Ibanez, S, Chassin Trubert, A, Dong, L, Cajas, L, Baresic, M, Anic, B, Culo, M, Pavelic, T, Stranski, K, Karanovic, B, Vencovsky, J, Pichova, M, Filkova, M, Hamoud, H, Vassilopoulos, D, Guzman Melgar, G, So, H, Kiraly, M, Vojdanian, M, Balbir Gurman, A, Abutiban, F, Zepa, J, Bulina, I, Bukauskiene, L, Zazueta Montiel, B, Castillo Ortiz, A, Zamora Tehozol, E, Vega Morales, D, Cervantes Rosete, D, Martin Nares, E, Rodriguez Reyna, T, Rull Gabayet, M, Alpizar Rodriguez, D, Irazoque, F, Jimenez, X, Geurts van Bon, L, Zijlstra, T, Hoekstra, M, Al Adhoubi, N, Salim, B, Giraldo, E, Salinas, A, Ugarte Gil, M, Nowakowski, J, Conway, R, Flood, R, Mccarthy, G, Felea, I, Filipescu, I, Rednic, S, Groseanu, L, Tamas, M, Mlynarikova, V, Skamlova, M, Zlnay, M, Micekova, D, Capova, L, Macejova, Z, Stenova, E, Raffayova, H, Belakova, G, Strakova, E, Sencarova, M, Zlnayova, S, Sabova, A, Spisakova, D, Oetterova, M, Lukacova, O, Bakosova, M, Hocevar, A, de la Torre Rubio, N, Alegre Sancho, J, Corteguera Coro, M, Cobeta Garcia, J, Torres Martin, M, Campos, J, Gomez Puerta, J, Yardimci, G, Akar, S, Icacan, O, Celik, S, Vasylets, V, Yeoh, S, Vandevelde, C, Dunt, S, Leeder, J, Macphie, E, Salerno, R, Graver, C, Williams, K, O'Reilly, S, Devine, K, Tyler, J, Warner, E, Pilcher, J, Patel, S, Nikiphorou, E, Chadwick, L, Jones, C, Harrison, B, Thornton, L, O'Kane, D, Fusi, L, Low, A, Horton, S, Jatwani, S, Baig, S, Bajwa, H, Berglund, V, Dahle, A, Dorman, W, Hargrove, J, Hilton, M, Lebedoff, N, Leonard, S, Morgan, J, Pfeifer, E, Skemp, A, Wilson, J, Wolff, A, Cepeda, E, Todd, D, Hare, D, Calabrese, C, Adams, C, Khosroshahi, A, Kilian, A, White, D, Winter, M, Fields, T, Siegel, C, Daver, N, Harvey, M, Kramer, N, Lamore, C, Hogarty, S, Yeter, K, Siddique, F, Ban, B, Tanner, T, Ruderman, E, Davis, W, Quinet, R, Scopelitis, E, Toribio, K, Webb Detiege, T, Zakem, J, Abbass, K, Kepecs, G, Miranda, L, Guma, M, Haikal, A, Mody, S, Mueller, D, Jayatilleke, A, Zell, J, Bays, A, Dao, K, Ezzati, F, Parks, D, Karp, D, Quiceno, G, and Repositório da Universidade de Lisboa

Subjects
health care disparity, Immunology, air pollution, cohort analysi, mortality rate, Autoimmune Disease, Article, coronavirus disease 2019, biocontainment, Rheumatology, male, cardiovascular disease, environmental factor, death, geographic distribution, Immunology and Allergy, follow up, controlled study, human, population density, outcome assessment, rheumatic disease, diabetes mellitu, adult, Prevention, market, temperature, mortality, United Kingdom, Europe, aged, female, workplace, Good Health and Well Being, human development, particulate matter 2.5, statistical analysi, North America, incidence, life expectancy, social aspect, COVID-19 Global Rheumatology Alliance Registry, observational study, demographic
Abstract

Published by Elsevier Ltd., Background: Differences in the distribution of individual-level clinical risk factors across regions do not fully explain the observed global disparities in COVID-19 outcomes. We aimed to investigate the associations between environmental and societal factors and country-level variations in mortality attributed to COVID-19 among people with rheumatic disease globally. Methods: In this observational study, we derived individual-level data on adults (aged 18-99 years) with rheumatic disease and a confirmed status of their highest COVID-19 severity level from the COVID-19 Global Rheumatology Alliance (GRA) registry, collected between March 12, 2020, and Aug 27, 2021. Environmental and societal factors were obtained from publicly available sources. The primary endpoint was mortality attributed to COVID-19. We used a multivariable logistic regression to evaluate independent associations between environmental and societal factors and death, after controlling for individual-level risk factors. We used a series of nested mixed-effects models to establish whether environmental and societal factors sufficiently explained country-level variations in death. Findings: 14 044 patients from 23 countries were included in the analyses. 10 178 (72·5%) individuals were female and 3866 (27·5%) were male, with a mean age of 54·4 years (SD 15·6). Air pollution (odds ratio 1·10 per 10 μg/m3 [95% CI 1·01-1·17]; p=0·0105), proportion of the population aged 65 years or older (1·19 per 1% increase [1·10-1·30]; p, MAG is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K01 AR070585 and K24 AR074534 [JY]). KDW is supported by the Department of Veterans Affairs and the Rheumatology Research Foundation Scientist Development award. JAS is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (grant numbers K23 AR069688, R03 AR075886, L30 AR066953, P30 AR070253, and P30 AR072577), the Rheumatology Research Foundation (K Supplement Award and R Bridge Award), the Brigham Research Institute, and the R. Bruce and Joan M. Mickey Research Scholar Fund. NJP is supported by the National Institute of Arthritis and Musculoskeletal and Skin Diseases (T32-AR-007258). AD-G is supported by grants from the Centers for Disease Control and Prevention and the Rheumatology Research Foundation. RH was supported by the Justus-Liebig University Giessen Clinician Scientist Program in Biomedical Research to work on this registry. JY is supported by grants from the National Institutes of Health (K24 AR074534 and P30 AR070155).

Published
2022

10. Obstetric Outcomes in Women with Rheumatic Disease and COVID-19 in the Context of Vaccination Status

Author

Sinead Maguire, Samar Al-Emadi, Paula Alba, Mathia Cecilia Aguiar, Talal Al Lawati, Gelsomina Alle, Bonnie Bermas, Suleman Bhana, Anic Branimir, Inita Bulina, Megan Clowse, Karina Cogo, Iris Colunga, Claire Cook, Karen J Cortez, Kathryn Dao, Milena Gianfrancesco, Monique Gore-Massey, Laure Gossec, Rebecca Grainger, Jonathon Hausman, Tiffany Y T Hsu, Kimme Hyrich, Carolina Isnardi, Yumeko Kawano, Rachael Kilding, Daria A Kusevich, Saskia Lawson-Tovey, Jean Liew, Eoghan McCarthy, Anna Montgumery, Sebastian Moyano, Noreen Nasir, Ivan Padjen, Charalampos Papagoras, Naomi J Patel, Mariana Pera, Cecilia Pisoni, Guillermo Pons-Estel, Antonio L Quiambao, Rosana Quintana, Eric Ruderman, Sebastian Sattui, Veronica Savio, Savino Sciascia, Marieta Sencarova, Rosa Serrano Morales, Faizah Siddique, Emily Sirotich, Jeffrey Sparks, Anja Strangfeld, Paul Sufka, Helen Tanner, Yohana Tissera, Zachary Wallace, Marina L Werner, Leanna Wise, Angus B Worthing, JoAnn Zell, Julija Zepa, Pedro M Machado, Jinoos Yazdany, Philip Robinson, and Richard Conway

Subjects
Rheumatology, Pharmacology (medical)
Abstract

Objective To describe obstetric outcomes based on COVID-19 vaccination status, in women with rheumatic and musculoskeletal diseases (RMDs) who developed COVID-19 during pregnancy. Methods Data regarding pregnant women entered into the COVID-19 Global Rheumatology Alliance registry from 24 March 2020–25 February 2022 were analysed. Obstetric outcomes were stratified by number of COVID-19 vaccine doses received prior to COVID-19 infection in pregnancy. Descriptive differences between groups were tested using the chi-squared or Fisher’s exact test. Results There were 73 pregnancies in 73 women with RMD and COVID-19. Overall, 24.7% (18) of pregnancies were ongoing, while of the 55 completed pregnancies, 90.9% (50) of pregnancies resulted in livebirths. At the time of COVID-19 diagnosis, 60.3% (n = 44) of women were unvaccinated, 4.1% (n = 3) had received one vaccine dose while 35.6% (n = 26) had two or more doses. Although 83.6% (n = 61) of women required no treatment for COVID-19, 20.5% (n = 15) required hospital admission. COVID-19 resulted in delivery in 6.8% (n = 3) of unvaccinated women and 3.8% (n = 1) of fully vaccinated women. There was a greater number of preterm births (PTB) in unvaccinated women compared with fully vaccinated 29.5% (n = 13) vs 18.2% (n = 2). Conclusions In this descriptive study, unvaccinated pregnant women with RMD and COVID-19 had a greater number of PTB compared with those fully vaccinated against COVID-19. Additionally, the need for COVID-19 pharmacological treatment was uncommon in pregnant women with RMD regardless of vaccination status. These results support active promotion of COVID-19 vaccination in women with RMD who are pregnant or planning a pregnancy.

Published
2022

11. The added value of a European Reference Network on rare and complex connective tissue and musculoskeletal diseases: insights after the first 5 years of the ERN ReCONNET

Author

Rosaria Talarico, Silvia Aguilera, Tobias Alexander, Zahir Amoura, Janette Andersen, Laurent Arnaud, Tadej Avcin, Sara Marsal Barril, Lorenzo Beretta, Stefano Bombardieri, Alessandra Bortoluzzi, Coralie Bouillot, Inita Bulina, Gerd R. Burmester, Sara Cannizzo, Lorenzo Cavagna, Benjamin Chaigne, Alain Cornet, Paolo Corti, Nathalie Costedoat-Chalumeau, Zane Dāvidsone, Andrea Doria, Carol Fenech, Alessandro Ferraris, Rebecca Fischer-Betz, João Eurico Fonseca, Charissa Frank, Andrea Gaglioti, Ilaria Galetti, Vera Guimarães, Eric Hachulla, Monica Holmner, Frederic Houssiau, Luca Iaccarino, Søren Jacobsen, Maarten Limper, Fransiska Malfait, Xavier Mariette, Diana Marinello, Thierry Martin, Lisa Matthews, Marco Matucci-Cerinic, Alain Meyer, Jasminka Milas-Ahić, Pia Moinzadeh, Carlomaurizio Montecucco, Luc Mouthon, Ulf Müller-Ladner, György Nagy, Eunice Patarata, Margarita Pileckyte, Chris Pruunsild, Simona Rednic, Vasco C. Romão, Matthias Schneider, Carlo Alberto Scirè, Vanessa Smith, Alberto Sulli, Farah Tamirou, Chiara Tani, Domenica Taruscio, Anna V. Taulaigo, Angela Tincani, Simone Ticciati, Giuseppe Turchetti, P. Martin van Hagen, Jacob M. van Laar, Ana Viera, Jeska K. de Vries-Bouwstra, Johannes Zschocke, Maurizio Cutolo, Marta Mosca, Talarico, R, Aguilera, S, Alexander, T, Amoura, Z, Andersen, J, Arnaud, L, Avcin, T, Marsal Barril, S, Beretta, L, Bombardieri, S, Bortoluzzi, A, Bouillot, C, Bulina, I, Burmester, G, Cannizzo, S, Cavagna, L, Chaigne, B, Cornet, A, Corti, P, Costedoat-Chalumeau, N, Davidsone, Z, Doria, A, Fenech, C, Ferraris, A, Fischer-Betz, R, Fonseca, J, Frank, C, Gaglioti, A, Galetti, I, Guimaraes, V, Hachulla, E, Holmner, M, Houssiau, F, Iaccarino, L, Jacobsen, S, Limper, M, Malfait, F, Mariette, X, Marinello, D, Martin, T, Matthews, L, Matucci-Cerinic, M, Meyer, A, Milas-Ahic, J, Moinzadeh, P, Montecucco, C, Mouthon, L, Muller-Ladner, U, Nagy, G, Patarata, E, Pileckyte, M, Pruunsild, C, Rednic, S, Romao, V, Schneider, M, Scire, C, Smith, V, Sulli, A, Tamirou, F, Tani, C, Taruscio, D, Taulaigo, A, Tincani, A, Ticciati, S, Turchetti, G, van Hagen, P, van Laar, J, Vieira, A, de Vries-Bouwstra, J, Zschocke, J, Cutolo, M, Mosca, M, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, and UCL - (SLuc) Service de rhumatologie

Subjects
rare and complex diseases, Health Personnel, rare and complex disease, Immunology, patient care, European Reference Network, rheumatic and musculoskeletal diseases, rheumatic and musculoskeletal disease, Europe, European Reference Networks, Rare Diseases, Rheumatology, Connective Tissue, connective tissue disease, Immunology and Allergy, Humans, Musculoskeletal Diseases, connective tissue diseases, European Commission
Abstract

In order to address the main challenges related to the rare diseases (RDs) the European Commission launched the European Reference Networks (ERNs), virtual networks involving healthcare providers (HCPs) across Europe. The mission of the ERNs is to tackle low prevalence and RDs that require highly specialised treatment and a concentration of knowledge and resources. In fact, ERNs offer the potential to give patients and healthcare professionals across the EU access to the best expertise and timely exchange of lifesaving knowledge, trying to make the knowledge travelling more than patients. For this reason, ERNs were established as concrete European infrastructures, and this is particularly crucial in the framework of rare and complex diseases in which no country alone has the whole knowledge and capacity to treat all types of patients. It has been five years since their kick-off launch in Vilnius in 2017. The 24 ERNs have been intensively working on different transversal areas, including patient management, education, clinical practice guidelines, patients' care pathways and many other fundamental topics. The present work is therefore aimed not only at reporting a summary of the main activities and milestones reached so far, but also at celebrating the first 5 years of the ERN on Rare and Complex Connective Tissue and Musculo-skeletal Diseases (ReCONNET), in which the members of the network built together one of the 24 infrastructures that are hopefully going to change the scenario of rare diseases across the EU.

Published
2022
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12. Rare clinical manifestations in systemic lupus erythematosus: a review on frequency and clinical presentation

Author

Chiara Tani, Elena Elefante, Laurent Arnaud, Sofia C. Barreira, Inita Bulina, Lorenzo Cavagna, Nathalie Costedoat-Chalumeau, Andrea Doria, João Eurico Fonseca, Franco Franceschini, Micaela Fredi, Luca Iaccarino, Maarten Limper, Judit Majnik, Gyorgy Nagy, Cristina Pamfil, Simona Rednic, John A. Reynolds, Maria G. Tektonidou, Anne Troldborg, Giovanni Zanframundo, Marta Mosca, and Repositório da Universidade de Lisboa

Subjects
Lung Diseases, Lupus Erythematosus, Hypertension, Pulmonary, Humans, Hepatitis, Autoimmune, Lupus Erythematosus, Systemic, Systemic, Immunology, education, Pulmonary, Lupus Erythematosus, Systemic/complications, Hepatitis, Rheumatology, Hypertension, Immunology and Allergy, Autoimmune
Abstract

© Copyright Clinical and Experimental Rheumatology 2022, Objectives: The purpose of this study was to review the frequency and clinical presentation of the rarest clinical manifestations of systemic lupus erythematosus (SLE). Methods: A list of 6 rare SLE manifestations were defined: gastrointestinal, liver, pulmonary, cardiac, ocular and neurological manifestations. Each topic was assigned to a pair of authors to perform a literature search and article review. Results: In total, 149 articles were included in the literature review: 37 for gastrointestinal manifestations, 6 for liver manifestations, 27 for pulmonary manifestations, 50 for cardiac manifestations, 16 for ocular manifestations, 13 for neurological manifestations. Gastrointestinal disorders included several clinical presentations with variable frequency (from 0.5% to 10.7% of the cases); liver involvement included lupus-related hepatitis (9.3%) and autoimmune hepatitis (2.3%). The rarest pulmonary manifestations identified were shrinking lung syndrome, described in 1.5% of patients, while interstitial lung disease and lupus pneumonia were reported in 4% and 3% of patients respectively. Myocarditis and pulmonary hypertension were also rarely described in SLE patients although ranging from 0.4-16% and 1-14% respectively, depending on the methodology used for its identification. Ocular manifestations in SLE included some rare manifestations (reported in less than 5% of patients) and lupus retinopathy that is described in 1.2-28.8% of patients depending on methods of ascertainment. Aseptic meningitis and chorea were also confirmed as very rare manifestations being reported in less than 1% and in 0.3-2.4% of cases respectively. Conclusions: The results of this literature review provide the basis for a better understanding of some less-known manifestations of SLE and for stressing the need for a higher awareness in diagnostic and therapeutic protocols regarding these rare disease aspects.

Published
2021
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13. Outcomes of COVID-19 in patients with primary systemic vasculitis or polymyalgia rheumatica from the COVID-19 Global Rheumatology Alliance physician registry: a retrospective cohort study

Author

Maria Sol Castaños Menescardi, Mercedes García, Elena Nikiphorou, Ulf Müller-Ladner, Rita Pinheiro Torres, Cassandra Calabrese, Nasra Al-Adhoubi, Sandra Lucia Euzebio Ribeiro, Kaley Beins, Puja Mehta, José Luis Velasco Zamora, Lorena Takashima, Loreta Bukauskiene, Derrick Todd, Gozd Kubra Yardimci, Samia Araujo de Sousa Studart, Ariel Salinas, Babur Salim, Martin Schaefer, Sarah Horton, Bea Maeyaert, Andrea M Seet, Beverley Harrison, Vernon Berglund, Douglas White, Archibald Skemp, Vanessa Castro Coello, Susan Leonard, Guillermo Quiceno, Geraldine McCarthy, Neil Kramer, Reinhard E. Voll, Helena Raffayova, Lianne Kearsley-Fleet, Hernán Maldonado Ficco, Emily L Gilbert, Samir Patel, Arezou Khosroshahi, Boris Karanovic, Jaroslaw Nowakowski, Dagmar Miceková, Márta Király, Saskia Lawson-Tovey, Alison Bays, Katie Williams, Pablo Maid, Montserrat Corteguera Coro, Anna Anna Sabová, Noelia German, Rosana Gallo, María Alejandra Cusa, Philip Robinson, Natalia Herscovich, Branimir Anic, Lorna Neill, Sara Baig, James Pilcher, Lucia Fusi, Jerald Zakem, Eugenia Picco, Monique Hoekstra, Michael S. Putman, Veronica Bellomio, Martina Skamlova, Daniela Spisakova, Caroline Mulvaney Jones, Ezzati Fatemeh, Sona Žlnayová, Silvana Conti, Eva Strakova, Jose A Gomez Puerta, Kristin M D’Silva, Marko Barešic, Yohana Tissera, Roberto Miguel Baez, Theodore Fields, Rachael Flood, Josefina Gallino Yanzi, Fatemah Abutiban, Henrique Ataide Mariz, Martin Zlnay, Mariana Luís, Mariana Pera, Robert Quinet, Claire Vandevelde, Richard Conway, Lubica Capova, Rodolfo Perez Alamino, Romina Nieto, Deborah Parks, Denise Hare, Audrey Low, Faizah Siddique, Zachary S. Wallace, Tiffany Y-T Hsu, Tameka Webb-Detiege, Sheila O'Reilly, Tatiana Barbich, Theo Zijlstra, Kathryn Dao, Luca Quartuccio, Cecilia Pisoni, Maria Isabel Quaglia, Zelmira Macejova, Laure Gossec, Jean W Liew, JoAnn Zell, Tiffany Y.T. Hsu, Rebecca Hasseli, Zara Izadi, Francinne Machado Ribeiro, Christopher Adams, Laura Chadwick, Naomi J Patel, Maria Carmen Torres Martin, Emoke Štenová, Fedra Irazoque, Natalia Lili Cuchiaro, Selda Çelik, Maria Isabel Haye Salinas, Alexandra Balbir-Gurman, Lucy Thornton, Shraddha Jatwani, Jane Leeder, Jeffrey A. Sparks, Pedro Machado, Elizabeth Macphie, Alojzija Hocevar, Melanie Winter, Christopher Hill, Carolina Aeschlimann, Loreto Carmona, Viktoriia Vasylets, Jose Campos, Jiri Vencovsky, Romina Tanten, Karina Cogo, Emily Pfeifer, Zachary S Wallace, Erick Zamora Tehozol, David F L Liew, Christine Graver, Cecilia Romeo, Lenny Geurts-van Bon, Alvaro Andres Reyes Torres, Arundathi Jayatilleke, Lui Cajas, Ana Bertoli, María Victoria Martire, Pascal Chazerain, Boris Kisluk, Anne Wolff, Alba Paula, Maria Marcela Schmid, Marieta Sencarová, Manuel F. Ugarte-Gil, Concetta Lamore, Veronica Savio, Emily Sirotich, Nicole Daver, Inita Bulina, Maria Filkova, Samar Al-Emadi, Jennifer Tyler, Ho So, Anne-Marie Chassin-Trubert, Leanna Wise, Davide Rozza, Yves Piette, Sabrina Solange de la Vega Fernandez, Eva Rath, Angel Alejandro Castillo Ortiz, Simona Rednic, Stéphane Bally, Kimme L Hyrich, Eric Ruderman, Gabriela Maria Guzman Melgar, Diana Cervántes Rosete, Alí Duarte-García, Juan Carlos Cobeta Garcia, Vanda Mlynarikova, Byung Ban, David Karp, Juan José Alegre Sancho, Carlevaris Leandro, Tamar Tanner, Gisela Subils, Susana Isabel Pineda, Ileana Filipescu, Lilliam Miranda, Karen Toribio Toribio, Karen Roberts, María Severina, Maria Valenzuela Almada, Kristin M. D’Silva, Tea Ahel Pavelic, Federico Nicolas Maldonado, Lingli Dong, Kirsty Devine, Ammar Haikal, Sebastian E Sattui, María J. Haye Salinas, Karen Yeter, Jennifer Morgan, Maria Julieta Gamba, Carla Matellan, Juan Alejandro Albiero, Angela Dahle, Martina Bakosova, Julija Zepa, Luciana Casalla, Jonathan S. Hausmann, Andrea Baños, William Davis, Elsa F Mateus, Kristina Kovacevic Stranski, Lindsay Jacobsohn, Milena A. Gianfrancesco, Daric Mueller, Eduardo Cepeda, Tatiana Sofia Rodriguez-Reyna, Sarah L. Mackie, Mahdi Vojdanian, Julieta Silvana Morbiducci, Enrique Giraldo, Gustavo Fabián Rodriguez Gil, Ivana Romina Rojas Tessel, Laura Groseanu, Carla Gobbi, Anja Strangfeld, Maria Soledad Gálvez Elkin, Alexandre Tj Maria, Adam Kilian, Marina Laura Werner, Sebastián Ibáñez, Sushama Mody, Melissa Harvey, Sofía Ornella, Melanie-Ivana Culo, Gabriela Belakova, Luciana Gonzalez Lucero, Marcelo Pinheiro, Natalia de la Torre-Rubio, Sasha Dunt, Khurram Abbass, Jeffrey A Sparks, Beatriz Zaueta, Elizabeth Warner, Servet Akar, Maren Hilton, Evangeline Scopelitis, Julia Scafati, Jeffrey Wilson, Marta Píchová, Rosana Quintana, Mária Oetterová, Diana O'Kane, Paul Sufka, Jinoos Yazdany, Mieke Devinck, Eduardo Martín Nares, Michael Guma, Gimena Gomez, Nicholas Lebedoff, Su-Ann Yeoh, Suneya Hogarty, Sandra Petruzzelli, Ma. Alicia Lazaro, Marina Rull Gabayet, Nafice Costa Araujo, Bimba F Hoyer, Maria Magdelena Tamas, Cecilia Goizueta, María Alejandra Medina, David Vega, Xochitl Jimenez, Rebecca Grainger, Micaela Cosatti, Gilbert Kepecs, Jonathan Eliseo Rebak, Walter Dorman, Dimitrios Vassilopoulos, Ann Knight, Maria de la Vega, Deshiré Alpízar-Rodríguez, Caroline Siegel, Ozan Cemal Icacan, María Elena Calvo, Sabrina Porta, Hesham Hamoud, Sandra Lucia Euzebio Ribeirio, Maxime Samson, Suleman Bhana, Gelsomina Alle, Ioana Felea, Sebastián Moyano, Rosaria Salerno, Carla Maldini, Jody Hargrove, Brahim Dahou, Fabian Risueño, Debora Guaglianone, Olga Lukacova, and Hammad Bajwa

Subjects
Aging, medicine.medical_specialty, Immunology, Autoimmune Disease, Polymyalgia rheumatica, Rheumatology, Clinical Research, Internal medicine, medicine, Immunology and Allergy, Global Rheumatology Alliance, business.industry, Arthritis, Inflammatory and immune system, Evaluation of treatments and therapeutic interventions, Retrospective cohort study, Articles, Odds ratio, medicine.disease, Comorbidity, Giant cell arteritis, 6.1 Pharmaceuticals, business, Vasculitis, Systemic vasculitis
Abstract

Summary Background Patients with primary systemic vasculitis or polymyalgia rheumatica might be at a high risk for poor COVID-19 outcomes due to the treatments used, the potential organ damage cause by primary systemic vasculitis, and the demographic factors associated with these conditions. We therefore aimed to investigate factors associated with COVID-19 outcomes in patients with primary systemic vasculitis or polymyalgia rheumatica. Methods In this retrospective cohort study, adult patients (aged ≥18 years) diagnosed with COVID-19 between March 12, 2020, and April 12, 2021, who had a history of primary systemic vasculitis (antineutrophil cytoplasmic antibody [ANCA]-associated vasculitis, giant cell arteritis, Behcet's syndrome, or other vasculitis) or polymyalgia rheumatica, and were reported to the COVID-19 Global Rheumatology Alliance registry were included. To assess COVID-19 outcomes in patients, we used an ordinal COVID-19 severity scale, defined as: (1) no hospitalisation; (2) hospitalisation without supplemental oxygen; (3) hospitalisation with any supplemental oxygen or ventilation; or (4) death. Multivariable ordinal logistic regression analyses were used to estimate odds ratios (ORs), adjusting for age, sex, time period, number of comorbidities, smoking status, obesity, glucocorticoid use, disease activity, region, and medication category. Analyses were also stratified by type of rheumatic disease. Findings Of 1202 eligible patients identified in the registry, 733 (61·0%) were women and 469 (39·0%) were men, and their mean age was 63·8 years (SD 17·1). A total of 374 (31·1%) patients had polymyalgia rheumatica, 353 (29·4%) had ANCA-associated vasculitis, 183 (15·2%) had giant cell arteritis, 112 (9·3%) had Behcet's syndrome, and 180 (15·0%) had other vasculitis. Of 1020 (84·9%) patients with outcome data, 512 (50·2%) were not hospitalised, 114 (11·2%) were hospitalised and did not receive supplemental oxygen, 239 (23·4%) were hospitalised and received ventilation or supplemental oxygen, and 155 (15·2%) died. A higher odds of poor COVID-19 outcomes were observed in patients who were older (per each additional decade of life OR 1·44 [95% CI 1·31–1·57]), were male compared with female (1·38 [1·05–1·80]), had more comorbidities (per each additional comorbidity 1·39 [1·23–1·58]), were taking 10 mg/day or more of prednisolone compared with none (2·14 [1·50–3·04]), or had moderate, or high or severe disease activity compared with those who had disease remission or low disease activity (2·12 [1·49–3·02]). Risk factors varied among different disease subtypes. Interpretation Among patients with primary systemic vasculitis and polymyalgia rheumatica, severe COVID-19 outcomes were associated with variable and largely unmodifiable risk factors, such as age, sex, and number of comorbidities, as well as treatments, including high-dose glucocorticoids. Our results could be used to inform mitigation strategies for patients with these diseases. Funding American College of Rheumatology and the European Alliance of Associations for Rheumatology.

Published
2021
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14. Early experience of COVID-19 vaccination in adults with systemic rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

Author

Alfred H.J. Kim, Tamer A. Gheita, Lina El Kibbi, Akpabio Akanimo Akpabio, S. Mingolla, Philip Robinson, Gary Foster, Chieh Lo, Manuel F. Ugarte-Gil, Jean W. Liew, Jinoos Yazdany, Suleman Bhana, Michal Nudel, Christopher Hill, Lehana Thabane, Carly Harrison, Jonathan S. Hausmann, Jasvinder A. Singh, Kristen J. Young, Emily Sirotich, Richard A Howard, Adam Kilian, Tarin Moni, Paul Sufka, Julia F. Simard, Candace A Palmerlee, Bimba F. Hoyer, Pedro Machado, Jeffrey A. Sparks, Bruce Miller, Maggie Larché, Namrata Singh, Aman Dev Singh, Deshire Alpizar-Rodriguez, Eimear Duff, Mitchell Levine, Richard Conway, Evelyn Hsieh, Zachary S. Wallace, Sebastian E. Sattui, Lisa G. Rider, Kevin Kennedy, David F L Liew, Rebecca Grainger, Wendy Costello, Inita Bulina, K. Durrant, Michael S. Putman, John Wallace, Francis Berenbaum, and Medicine

Subjects
Adult, Male, medicine.medical_specialty, Pediatrics, Aging, COVID-19 Vaccines, Immunology, Population, Clinical Sciences, Infections, Autoimmune Disease, Vaccine Related, Rheumatology, Internal medicine, Rheumatic Diseases, Surveys and Questionnaires, medicine, Immunology and Allergy, Humans, autoimmune diseases, Adverse effect, education, education.field_of_study, business.industry, SARS-CoV-2, Arthritis, Prevention, Inflammatory and immune system, Vaccination, COVID-19, Middle Aged, Vaccine efficacy, medicine.disease, 3.4 Vaccines, Rheumatoid arthritis, 6.1 Pharmaceuticals, Medicine, Chills, Female, Immunization, medicine.symptom, business, Somnolence
Abstract

BackgroundWe describe the early experiences of adults with systemic rheumatic disease who received the COVID-19 vaccine.MethodsFrom 2 April to 30 April 2021, we conducted an online, international survey of adults with systemic rheumatic disease who received COVID-19 vaccination. We collected patient-reported data on clinician communication, beliefs and intent about discontinuing disease-modifying antirheumatic drugs (DMARDs) around the time of vaccination, and patient-reported adverse events after vaccination.ResultsWe analysed 2860 adults with systemic rheumatic diseases who received COVID-19 vaccination (mean age 55.3 years, 86.7% female, 86.3% white). Types of COVID-19 vaccines were Pfizer-BioNTech (53.2%), Oxford/AstraZeneca (22.6%), Moderna (21.3%), Janssen/Johnson & Johnson (1.7%) and others (1.2%). The most common rheumatic disease was rheumatoid arthritis (42.3%), and 81.2% of respondents were on a DMARD. The majority (81.9%) reported communicating with clinicians about vaccination. Most (66.9%) were willing to temporarily discontinue DMARDs to improve vaccine efficacy, although many (44.3%) were concerned about rheumatic disease flares. After vaccination, the most reported patient-reported adverse events were fatigue/somnolence (33.4%), headache (27.7%), muscle/joint pains (22.8%) and fever/chills (19.9%). Rheumatic disease flares that required medication changes occurred in 4.6%.ConclusionAmong adults with systemic rheumatic disease who received COVID-19 vaccination, patient-reported adverse events were typical of those reported in the general population. Most patients were willing to temporarily discontinue DMARDs to improve vaccine efficacy. The relatively low frequency of rheumatic disease flare requiring medications was reassuring.

Published
2021

15. Prolonged COVID-19 symptom duration in people with systemic autoimmune rheumatic diseases: results from the COVID-19 Global Rheumatology Alliance Vaccine Survey

Author

Michael DiIorio, Kevin Kennedy, Jean W Liew, Michael S Putman, Emily Sirotich, Sebastian E Sattui, Gary Foster, Carly Harrison, Maggie J Larché, Mitchell Levine, Tarin T Moni, Lehana Thabane, Suleman Bhana, Wendy Costello, Rebecca Grainger, Pedro M Machado, Philip C Robinson, Paul Sufka, Zachary S Wallace, Jinoos Yazdany, Monique Gore-Massy, Richard A Howard, More A Kodhek, Nadine Lalonde, Laura-Ann Tomasella, John Wallace, Akpabio Akpabio, Deshiré Alpízar-Rodríguez, Richard P Beesley, Francis Berenbaum, Inita Bulina, Eugenia Yupei Chock, Richard Conway, Alí Duarte-García, Eimear Duff, Tamer A Gheita, Elizabeth R Graef, Evelyn Hsieh, Lina El Kibbi, David FL Liew, Chieh Lo, Michal Nudel, Aman Dev Singh, Jasvinder A Singh, Namrata Singh, Manuel F Ugarte-Gil, Jonathan S Hausmann, Julia F Simard, and Jeffrey A Sparks

Subjects
Arthritis, Rheumatoid, Male, COVID-19 Vaccines, Rheumatology, Surveys and Questionnaires, Immunology, COVID-19, Humans, Immunology and Allergy, Female, Middle Aged
Abstract

ObjectiveWe investigated prolonged COVID-19 symptom duration, defined as lasting 28 days or longer, among people with systemic autoimmune rheumatic diseases (SARDs).MethodsWe analysed data from the COVID-19 Global Rheumatology Alliance Vaccine Survey (2 April 2021–15 October 2021) to identify people with SARDs reporting test-confirmed COVID-19. Participants reported COVID-19 severity and symptom duration, sociodemographics and clinical characteristics. We reported the proportion experiencing prolonged symptom duration and investigated associations with baseline characteristics using logistic regression.ResultsWe identified 441 respondents with SARDs and COVID-19 (mean age 48.2 years, 83.7% female, 39.5% rheumatoid arthritis). The median COVID-19 symptom duration was 15 days (IQR 7, 25). Overall, 107 (24.2%) respondents had prolonged symptom duration (≥28 days); 42/429 (9.8%) reported symptoms lasting ≥90 days. Factors associated with higher odds of prolonged symptom duration included: hospitalisation for COVID-19 vs not hospitalised and mild acute symptoms (age-adjusted OR (aOR) 6.49, 95% CI 3.03 to 14.1), comorbidity count (aOR 1.11 per comorbidity, 95% CI 1.02 to 1.21) and osteoarthritis (aOR 2.11, 95% CI 1.01 to 4.27). COVID-19 onset in 2021 vs June 2020 or earlier was associated with lower odds of prolonged symptom duration (aOR 0.42, 95% CI 0.21 to 0.81).ConclusionMost people with SARDs had complete symptom resolution by day 15 after COVID-19 onset. However, about 1 in 4 experienced COVID-19 symptom duration 28 days or longer; 1 in 10 experienced symptoms 90 days or longer. Future studies are needed to investigate the possible relationships between immunomodulating medications, SARD type/flare, vaccine doses and novel viral variants with prolonged COVID-19 symptoms and other postacute sequelae of COVID-19 among people with SARDs.

Published
2022
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16. Immediate effect of the COVID-19 pandemic on patient health, health-care use, and behaviours: results from an international survey of people with rheumatic diseases

Author

Jonathan S Hausmann, Kevin Kennedy, Julia F Simard, Jean W Liew, Jeffrey A Sparks, Tarin T Moni, Carly Harrison, Maggie J Larché, Mitchell Levine, Sebastian E Sattui, Teresa Semalulu, Gary Foster, Salman Surangiwala, Lehana Thabane, Richard P Beesley, Karen L Durrant, Elsa F Mateus, Serena Mingolla, Michal Nudel, Candace A Palmerlee, Dawn P Richards, David F L Liew, Catherine L Hill, Suleman Bhana, Wendy Costello, Rebecca Grainger, Pedro M Machado, Philip C Robinson, Paul Sufka, Zachary S Wallace, Jinoos Yazdany, Emily Sirotich, Philip C. Robinson, Jean W. Liew, Paul H. Sufka, Namrata Singh, Richard A. Howard, Alfred H.J. Kim, Tiffany Westrich-Robertson, Edmund Tsui, Ali Duarte-Garcia, Jeffrey A. Sparks, Herman Tam, Arundathi Jayatilleke, Maximilian F. Konig, Elizabeth R. Graef, Michael S. Putman, Reema H. Syed, Peter Korsten, Elsa Mateus, Sebastian E. Sattui, Zachary S. Wallace, Upton A. Laura, Kilian Adam, Yu Pei Eugenia Chock, Douglas W. White, Geraldine T. Zamora, Lisa S. Traboco, Aarat M. Patel, Manuel F. Ugarte-Gil, Milena A. Gianfrancesco, Isabelle Amigues, Catalina Sanchez-Alvarez, Laura Trupin, Lindsay R. Jacobsohn, Richard P. Beesley, Bimba F. Hoyer, Pedro M. Machado, Kavita Makan, Laure Gossec, Chaudhary Priyank, Jan Leipe, Beth Wallace, Sheila T. Angeles-Han, Ibrahim A. Almaghlouth, Wysham D. Katherine, Anthony S. Padula, Francis Berenbaum, Erin M. Treemarcki, Rashmi Sinha, Laura B. Lewandowski, Kate Webb, Kristen J. Young, Inita Bulina, Sebastian Herrera Uribe, Tamar B. Rubinstein, Marc W. Nolan, Elizabeth Y. Ang, Swamy R. Venuturupalli, Jonathan S. Hausmann, Maureen Dubreuil, Cecilia N. Pisoni, Micaela A. Cosatti, Jose Campos, Julia F. Simard, Richard Conway, Tiffany M. Peterson, Carly O. Harrison, Christele Felix, Dawn P. Richards, Laurie Proulx, Akpabio A. Akpabio, Angus B. Worthing, Lynn R. Laidlaw, Pankti Reid, Candace A. Palmerlee, Maria I. Danila, Lotfi-Emran Sahar, Ngo Q. Linh, Arnav Agarwal, Paul Studenic, David F.L. Liew, Maggie J. Larche, Serena A.M. Mingolla, Erick A. Zamora, Saskya S. Angevare, Rashmi R. Sinha, Karen L.W. Durrant, Andrea Peirce, Emily C. Somers, Laura C. Cappelli, Brittany A. Frankel, Bharat Kumar, Sonia D. Silinsky Krupnikova, Jorge A. Rosario Vega, Jourdan Frankovich, Ruth Fernandez-Ruiz, Marcela Posada Velásquez, Su-Ann Yeoh, Maria Marino, Chrisiaan Scott, Cecilia Rodríguez, Ana I. Martín Mancheño, Philip Seo, Rocío V. Gamboa-Cárdenas, Victor R. Pimentel-Quiroz, Cristina Reátegui-Sokolova, Mari Kihara, Chung M.A. Lin, Dheera Kattula, Girgis Laila, Loreto Carmona, John Wallace, Monique C. Gore-massy, Laura-Ann Tomasella, and Moré A. Kodek

Subjects
medicine.medical_specialty, business.industry, Public health, Immunology, Articles, medicine.disease, Mental health, Rheumatology, Rheumatoid arthritis, Family medicine, Internal medicine, Fibromyalgia, Patient experience, Pandemic, Health care, medicine, Immunology and Allergy, business
Abstract

Background: The impact and consequences of the COVID-19 pandemic on people with rheumatic disease are unclear. We developed the COVID-19 Global Rheumatology Alliance Patient Experience Survey to assess the effects of the COVID-19 pandemic on people with rheumatic disease worldwide. Methods: Survey questions were developed by key stakeholder groups and disseminated worldwide through social media, websites, and patient support organisations. Questions included demographics, rheumatic disease diagnosis, COVID-19 diagnosis, adoption of protective behaviours to mitigate COVID-19 exposure, medication access and changes, health-care access and communication with rheumatologists, and changes in employment or schooling. Adults age 18 years and older with inflammatory or autoimmune rheumatic diseases were eligible for inclusion. We included participants with and without a COVID-19 diagnosis. We excluded participants reporting only non-inflammatory rheumatic diseases such as fibromyalgia or osteoarthritis. Findings: 12 117 responses to the survey were received between April 3 and May 8, 2020, and of these, 10 407 respondents had included appropriate age data. We included complete responses from 9300 adults with rheumatic disease (mean age 46·1 years; 8375 [90·1%] women, 893 [9·6%] men, and 32 [0·3%] participants who identified as non-binary). 6273 (67·5%) of respondents identified as White, 1565 (16·8%) as Latin American, 198 (2·1%) as Black, 190 (2·0%) as Asian, and 42 (0·5%) as Native American or Aboriginal or First Nation. The most common rheumatic disease diagnoses included rheumatoid arthritis (3636 [39·1%] of 9300), systemic lupus erythematosus (2882 [31·0%]), and Sjogren's syndrome (1290 [13·9%]). Most respondents (6921 [82·0%] of 8441) continued their antirheumatic medications as prescribed. Almost all (9266 [99·7%] of 9297) respondents adopted protective behaviours to limit SARS-CoV-2 exposure. A change in employment status occurred in 2524 (27·1%) of 9300) of respondents, with a 13·6% decrease in the number in full-time employment (from 4066 to 3514). Interpretation: People with rheumatic disease maintained therapy and followed public health advice to mitigate the risks of COVID-19. Substantial employment status changes occurred, with potential implications for health-care access, medication affordability, mental health, and rheumatic disease activity. Funding: American College of Rheumatology.

Published
2021

17. SARS-CoV-2 vaccine safety in adolescents with inflammatory rheumatic and musculoskeletal diseases and adults with juvenile idiopathic arthritis: data from the EULAR COVAX physician-reported registry

Author

Saskia, Lawson-Tovey, Pedro M, Machado, Anja, Strangfeld, Elsa, Mateus, Laure, Gossec, Loreto, Carmona, Bernd, Raffeiner, Inita, Bulina, Daniel, Clemente, Julija, Zepa, Ana M, Rodrigues, Xavier, Mariette, Kimme L, Hyrich, Vanda, Mylnarikova, UCL - SSS/IREC/RUMA - Pôle de Pathologies rhumatismales, and UCL - (SLuc) Service de rhumatologie

Subjects
Adult, COVID-19 Vaccines, Adolescent, SARS-CoV-2, Immunology, COVID-19, Arthralgia, Arthritis, Juvenile, Rheumatology, Physicians, Humans, Immunology and Allergy, Female, Musculoskeletal Diseases, Registries, Child
Abstract

BackgroundThere is a lack of data on SARS-CoV-2 vaccination safety in children and young people (CYP) with rheumatic and musculoskeletal diseases (RMDs). Current vaccination guidance is based on data from adults with RMDs or CYP without RMDs.ObjectivesTo describe the safety of SARS-COV-2 vaccination in adolescents with inflammatory RMDs and adults with juvenile idiopathic arthritis (JIA).MethodsWe described patient characteristics, flares and adverse events (AEs) in adolescent cases under 18 with inflammatory RMDs and adult cases aged 18 or above with JIA submitted to the European Alliance of Associations for Rheumatology COVAX registry.ResultsA total of 110 cases were reported to the registry. Thirty-six adolescent cases were reported from four countries, most with JIA (42%). Over half (56%) reported early reactogenic-like AEs. One mild polyarthralgia flare and one serious AE of special interest (malaise) were reported. No CYP reported SARS-CoV-2 infection postvaccination.Seventy-four adult JIA cases were reported from 11 countries. Almost two-thirds (62%) reported early reactogenic-like AEs and two flares were reported (mild polyarthralgia and moderate uveitis). No serious AEs of special interest were reported among adults with JIA. Three female patients aged 20–30 years were diagnosed with SARS-CoV-2 postvaccination; all fully recovered.ConclusionsThis is an important contribution to research on SARS-CoV-2 vaccine safety in adolescents with RMDs and adults with JIA. It is important to note the low frequency of disease flares, serious AEs and SARS-CoV-2 reinfection seen in both populations, although the dataset is limited by its size.

Published
2022
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18. SARS-CoV-2 breakthrough infections among vaccinated individuals with rheumatic disease: results from the COVID-19 Global Rheumatology Alliance provider registry

Author

Jean Liew, Milena Gianfrancesco, Carly Harrison, Zara Izadi, Stephanie Rush, Saskia Lawson-Tovey, Lindsay Jacobsohn, Clairissa Ja, Kimme L Hyrich, Laure Gossec, Anja Strangfeld, Loreto Carmona, Martin Schäfer, Elsa Frãzao-Mateus, Inita Bulina, Frances Stafford, Abdurrahman Tufan, Christine Graver, Gözde Kübra Yardımcı, Julija Zepa, Samar Al Emadi, Claire Cook, Fatemah Abutiban, Dfiza Dey, Genevieve Katigbak, Lauren Kaufman, Emily Kowalski, Marco Ulises Martínez-Martínez, Naomi J Patel, Greta Reyes-Cordero, Evelyn Salido, Ellison Smith, David Snow, Jeffrey Sparks, Leanna Wise, Suleman Bhana, Monique Gore-Massy, Rebecca Grainger, Jonathan Hausmann, Emily Sirotich, Paul Sufka, Zachary Wallace, Pedro M Machado, Philip C Robinson, and Jinoos Yazdany

Subjects
Adult, Aged, 80 and over, Male, COVID-19 Vaccines, SARS-CoV-2, Immunology, COVID-19, Middle Aged, Rheumatology, Rheumatic Diseases, Humans, Immunology and Allergy, Female, Registries, Aged
Abstract

ObjectiveWhile COVID-19 vaccination prevents severe infections, poor immunogenicity in immunocompromised people threatens vaccine effectiveness. We analysed the clinical characteristics of patients with rheumatic disease who developed breakthrough COVID-19 after vaccination against SARS-CoV-2.MethodsWe included people partially or fully vaccinated against SARS-CoV-2 who developed COVID-19 between 5 January and 30 September 2021 and were reported to the Global Rheumatology Alliance registry. Breakthrough infections were defined as occurring ≥14 days after completion of the vaccination series, specifically 14 days after the second dose in a two-dose series or 14 days after a single-dose vaccine. We analysed patients’ demographic and clinical characteristics and COVID-19 symptoms and outcomes.ResultsSARS-CoV-2 infection was reported in 197 partially or fully vaccinated people with rheumatic disease (mean age 54 years, 77% female, 56% white). The majority (n=140/197, 71%) received messenger RNA vaccines. Among the fully vaccinated (n=87), infection occurred a mean of 112 (±60) days after the second vaccine dose. Among those fully vaccinated and hospitalised (n=22, age range 36–83 years), nine had used B cell-depleting therapy (BCDT), with six as monotherapy, at the time of vaccination. Three were on mycophenolate. The majority (n=14/22, 64%) were not taking systemic glucocorticoids. Eight patients had pre-existing lung disease and five patients died.ConclusionMore than half of fully vaccinated individuals with breakthrough infections requiring hospitalisation were on BCDT or mycophenolate. Further risk mitigation strategies are likely needed to protect this selected high-risk population.

Published
2022
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19. Neuroinflammatory disease as an isolated manifestation of hemophagocytic lymphohistiocytosis

Author

Salah Ali, Yenan T. Bryceson, Marina Garcia-Prat, Maximilian Heeg, Jeffrey J. Bednarski, Carsten Speckmann, Jelena Rascon, Viktorija Kenina, Annaliesse Blincoe, Melissa Hines, Rebecca A. Marsh, Elie Haddad, Julie-An Talano, Anne Lortie, Patrick Campbell, Natalja Kurjane, Geertje E. Legger, Amer Khojah, Fabien Touzot, yasmine El Chazli, Julia T. Warren, Erica G. Schmitt, Marisa Klein-Gitelman, Stephan Ehl, Laura C. Alonso, Austen Worth, Maria C. Putti, Joerg Krueger, Evangeline Wassmer, Jacques G. Rivière, Kai Lehmberg, Itziar Astigarraga, Gal Goldstein, Kuang-Yueh Chiang, Inita Bulina, Claire Booth, Arjan C. Lankester, Michael M. Henry, Sarah Maier, Marwa Abd El-Maksoud, and Steven M. Holland

Subjects
Male, PRF1, Biopsy, CHILDREN, Gastroenterology, Leukoencephalopathy, Immunology and Allergy, Age of Onset, Child, CNS disease, Hematopoietic Stem Cell Transplantation, NERVOUS-SYSTEM INVOLVEMENT, Familial Hemophagocytic Lymphohistiocytosis, Magnetic Resonance Imaging, PERFORIN, Phenotype, Child, Preschool, Disease Progression, Female, GENOTYPE-PHENOTYPE, Symptom Assessment, medicine.symptom, Encephalitis, Adult, medicine.medical_specialty, Ataxia, Adolescent, Genotype, Immunology, Neuroimaging, FREQUENCY, Lymphohistiocytosis, Hemophagocytic, Young Adult, MUNC13-4, Internal medicine, Familial hemophagocytic lymphohistiocytosis, medicine, Humans, Genetic Predisposition to Disease, UNC13D, Alleles, Cytopenia, Hemophagocytic lymphohistiocytosis, SPECTRUM, therapy, business.industry, MUTATIONS, Multiple sclerosis, Infant, medicine.disease, CNS inflammation, Mutation, ONSET, business, Biomarkers
Abstract

Isolated neuroinflammatory disease has been described in case reports of familial hemophagocytic lymphohistiocytosis (FHL), but the clinical spectrum of disease manifestations, response to therapy and prognosis remain poorly defined. We combined an international survey with a literature search to identify FHL patients with (i) initial presentation with isolated neurological symptoms; (ii) absence of cytopenia and splenomegaly at presentation; and (iii) systemic HLH features no earlier than 3 months after neurological presentation. Thirty-eight (20 unreported) patients were identified with initial diagnoses including acute demyelinating encephalopathy, leukoencephalopathy, CNS vasculitis, multiple sclerosis, and encephalitis. Median age at presentation was 6.5 years, most commonly with ataxia/gait disturbance (75%) and seizures (53%). Diffuse multifocal white matter changes (79%) and cerebellar involvement (61%) were common MRI findings. CSF cell count and protein were increased in 22/29 and 15/29 patients, respectively. Fourteen patients progressed to systemic inflammatory disease fulfilling HLH-2004 criteria at a mean of 36.9 months after initial neurological presentation. Mutations were detected in PRF1 in 23 patients (61%), RAB27A in 10 (26%), UNC13D in 3 (8%), LYST in 1 (3%), and STXBP2 in 1 (3%) with a mean interval to diagnosis of 28.3 months. Among 19 patients who underwent HSCT, 11 neurologically improved, 4 were stable, one relapsed, and 3 died. Among 14 non-transplanted patients, only 3 improved or had stable disease, one relapsed, and 10 died. Isolated CNS-HLH is a rare and often overlooked cause of inflammatory brain disease. HLH-directed therapy followed by HSCT seems to improve survival and outcome.

Published
2020

20. Thematic stream: inflammatory arthritis (PP01-PP31): PP01. Autoinflammatory Synovitis in Familial Mediterranean Fever is Characterized by Numerous Neutrophils Lacking Myeloperoxidase and Lysozyme, Macrophages, Mast Cells and B Cells, Up-Regulation of Galectin-1, P65 (REL A)/NF-KB and Inos, but not COX-2

Author

James Cheng-Chung Wei, Tugba Baysak, Aysegul Kucukali Turkyilmaz, Hisashi Yamanaka, Izzet Fresko, Metin Ozgen, Moots Robert, Bunyamin Kisacik, Won Park, Yoshiya Tanaka, Ibrahim Batmaz, Özer Arıcan, Aytul Cakci, Serdar Budak, Jisoo Lee, Kouichi Amano, Rezzan Günaydin, Hatice Bodur, Sema Yilmaz, Yasin Tuluce, Bonnie Vlahos, Derya Kaskari, Tastekin Ebru, Ömür Kayıkçı, Suleyman Yildirim, Chun-Huang Huang, Inita Bulina, Mehmet Tosun, Erten Surkan, Johan Rönnelid, Walter Conca, Ayşegül Koç, Akın Erdal, Vıldan Altunayoğlu Çakmak, Ayhan Dinc, Ajda Bal, Mohammed Mullazehi, Hasan Battal, Sibel Süzen, Sun-Won Park, Daina Andersone, Li-Jie Shiu, Murat Toprak, Gülsüm Emel Pamuk, Çiftdemir Mert, Hyo Jin Choi, Tuba Baykal, Hideto Kameda, Josef S. Smolen, Alif Adlan Mohd Thabit, Masao Nawata, Mehmet Karakoç, Ozcan Hiz, Gulen Hatemi, Chan Hee Lee, Murat Zinnuroglu, Halil Koyuncu, Pierre Tane, Peter Nash, Tsutomu Takeuchi, Mehmet I. Arman, Shunsuke Fukuyo, Filiz Alp, Heselynn Hussein, Zuhal Atılgan, Murat Uludağ, Aylin Rezvani, Huri Ozdogan, Halil Ozkol, Ekin Sen, Yuko Kaneko, Seung Jae Hong, Ji Young Hwang, Constance Hammond, Kılınç Serdar, Eri Satoh, Chang-Hee Suh, Altinay Goksel Karatepe, Umut Kalyoncu, Daisuke Hoshi, Albert Kamanyi, Engin Tezcan, Inta Jaunalksne, Blanche Laure Ndontsa, Hong-Shen Lee, Hui Jen Ding, Mahir Ugur, Marius Mbiantcha, Zeynep Erden, Feride Gogus, Sebahattin Yurdakul, Haner Direskeneli, Altunoglu Alpaslan, Omer Karadag, Sina Esmaeilzadeh, Ruey-Hong Wong, Andrew S Koenig, Pedro Miranda, Bruce Freundlich, Taciser Kaya, Sedat Kiraz, You-Hyun Lee, Şafak Karamehmetoğlu, Ahmet Kiziltunc, Hayato Nagasawa, Erhan Capkin, Takahiko Kurasawa, Nurettin İrem Örnek, Aysegul Jale Saraç, Karel Pavelka, De Silva Vijitha, Deniz Dulgeroglu, Necati Çakır, Emel Ozcan, Remzi Çevik, alternative medicines, Erten Serhat Fuat, Kazim Senel, Ihsan Ertenli, Macfarlane Gary, Omer Nuri Pamuk, Levent Ediz, Salim Dönmez, Tugba Yalcin, El-Metwally Ashraf, Sedat Yilmaz, Suhail Al-Salam, Kentaro Hanami, Heidi Kokkonen, Kerem Gün, Solbritt Rantapää-Dahlqvist, Usta Ufuk, Kemal Nas, Selma Yazıcı, Muammer Muslım Kose, Fatih Baygutalp, Elif Gulcu, Kazuyoshi Saito, Murat Karkucak, Tastekin Nurettin, Ronald Pedersen, Birtane Murat, and Telesphore Benoit Nguelefack

Subjects
medicine.medical_specialty, Ankylosing spondylitis, business.industry, Inflammatory arthritis, Arthritis, medicine.disease, Gastroenterology, Etanercept, Rheumatology, Synovitis, Internal medicine, Rheumatoid arthritis, Immunology, medicine, Pharmacology (medical), Methotrexate, Adverse effect, business, medicine.drug
Abstract

Background: Recent recommendations have established clinical remission ideally or low disease activity (LDA) as therapeutic targets in all patients with rheumatoid arthritis (RA). Controlled studies of biologic agents have primarily assessed treatment effects in patients with severe RA; patients with moderate disease activity, whoconstitute a larger group, have received far less attention. In Period 1 of the PRESERVE trial, the proportion of subjects with moderately active RA achieving LDA or remission were evaluated after treatment with etanercept 50mg once weekly (QW) plus methotrexate for 36 weeks.Methods: Subjects with DAS28 >3.2 and ≤5.1 despite stable doses of oral methotrexate received open-label etanercept 50mg QW plus methotrexate (screening dose permitted to be titrated up to 25 mg/wk through week 28) for 36 weeks.Results: 834 subjects received treatment and were analyzed. Subjects were mostly female (83%), Caucasian (74%), RFþ (73%), and aCCPþ (78%), with a mean age of 48 years and disease duration of 7 years. Mean baseline DAS28 was 4.4; SDAI, 19.1; ESR, 22.2 mm/hr; and CRP, 12.3 mg/L. Efficacy results from Period 1 are shown in the table. The most commonly reported adverse events (AEs) were headache (6.1%), nasopharyngitis (5.4%), and upper respiratory tract infection (4.4%). Twenty-two subjects (2.6%) discontinued due to AEs.Conclusions: Substantial proportions of subjects with moderately active RA who received etanercept 50mg QW plus methotrexate for 36 weeks achieved LDA (85%-86%), DAS28 remission (67%), or SDAI remission (25%). Therefore, these ambitious goals can be achieved realistically in a high percentage of patients with moderate disease activity.

Published
2011
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