Annals of the Rheumatic Diseases

Clinical heterogeneity, unpredictable course and flares are characteristics of systemic lupus erythematosus (SLE). Although SLE is—by and large—a systemic disease, occasionally it can be organ-dominant, posing diagnostic challenges. To date, diagnosis of SLE remains clinical with a few cases being negative for serologic tests. Diagnostic criteria are not available and classification criteria are often used for diagnosis, yet with significant caveats. Newer sets of criteria (European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) 2019) enable earlier and more accurate classification of SLE. Several disease endotypes have been recognised over the years. There is increased recognition of milder cases at presentation, but almost half of them progress overtime to more severe disease. Approximately 70% of patients follow a relapsing-remitting course, the remaining divided equally between a prolonged remission and a persistently active disease. Treatment goals include long-term patient survival, prevention of flares and organ damage, and optimisation of health-related quality of life. For organ-threatening or life-threatening SLE, treatment usually includes an initial period of high-intensity immunosuppressive therapy to control disease activity, followed by a longer period of less intensive therapy to consolidate response and prevent relapses. Management of disease-related and treatment-related comorbidities, especially infections and atherosclerosis, is of paramount importance. New disease-modifying conventional and biologic agents—used alone, in combination or sequentially—have improved rates of achieving both short-term and long-term treatment goals, including minimisation of glucocorticoid use.

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Trajectories of COVID-19 information in the Annals of the Rheumatic Diseases: the first months of the pandemic

Kim Lauper, Johannes W J Bijlsma, Gerd R Burmester

doi : 10.1136/annrheumdis-2020-219217

Annals of the Rheumatic Diseases 2021;80:26-30.

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EULAR definition of difficult-to-treat rheumatoid arthritis

Gy?rgy Nagy, Nadia MT Roodenrijs, Paco MJ Welsing, Melinda Kedves, Attila Hamar, Marlies C van der Goes, Alison Kent, Margot Bakkers, Etienne Blaas, Ladislav Senolt, Zoltan Szekanecz, Ernest Choy, Maxime Dougados, Johannes WG Jacobs, Rinie Geenen, Hans WJ Bijlsma, Angela Zink, Daniel Aletaha, Leonard Schoneveld, Piet van Riel, Loriane Gutermann, Yeliz Prior, Elena Nikiphorou, Gianfranco Ferraccioli, Georg Schett, Kimme L Hyrich, Ulf Mueller-Ladner, Maya H Buch, Iain B McInnes, Désirée van der Heijde, Jacob M van Laar

doi : 10.1136/annrheumdis-2020-217344

Annals of the Rheumatic Diseases 2021;80:31-35

Background Despite 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.

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EULAR points to consider for the diagnosis and management of rheumatic immune-related adverse events due to cancer immunotherapy with checkpoint inhibitors

Marie Kostine, Axel Finckh, Clifton O Bingham, Karen Visser, Jan Leipe, Hendrik Schulze-Koops, Ernest H Choy, Karolina Benesova, Timothy R D J Radstake, Andrew P Cope, Olivier Lambotte, Jacques-Eric Gottenberg, Yves Allenbach, Marianne Visser, Cindy Rusthoven, Lone Thomasen, Shahin Jamal, Aurélien Marabelle, James Larkin, John B A G Haanen, Leonard H Calabrese, Xavier Mariette, Thierry Schaeverbeke

doi : 10.1136/annrheumdis-2020-217139

Annals of the Rheumatic Diseases 2021;80:36-48.

Background Rheumatic and musculoskeletal immune-related adverse events (irAEs) are observed in about 10% of patients with cancer receiving checkpoint inhibitors (CPIs). Given the recent emergence of these events and the lack of guidance for rheumatologists addressing them, a European League Against Rheumatism task force was convened to harmonise expert opinion regarding their identification and management.

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EULAR recommendations for a core data set for pregnancy registries in rheumatology

Yvette Meissner, Rebecca Fischer-Betz, Laura Andreoli, Nathalie Costedoat-Chalumeau, Diederik De Cock, Radboud J E M Dolhain, Frauke Forger, Doreen Goll, Anna Molto, Catherine Nelson-Piercy, Rebecca ?zdemir, Luigi Raio, Sebastian Cruz Rodr?guez-Garc?a, Savino Sciascia, Marianne Wallenius, Astrid Zbinden, Angela Zink, Anja Strangfeld

doi : 10.1136/annrheumdis-2020-218356

Annals of the Rheumatic Diseases 2021;80:49-56.

Background and objective There is an urgent need for robust data on the trajectories and outcomes of pregnancies in women with inflammatory rheumatic diseases (IRD). In particular when rare outcomes or rare diseases are to be investigated, collaborative approaches are required. However, joint data analyses are often limited by the heterogeneity of the different data sources.

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2019 EULAR points to consider for non-physician health professionals to prevent and manage fragility fractures in adults 50 years or older

Jo Adams, Nicky Wilson, Emalie Hurkmans, Margot Bakkers, Petra Bal??ov?, Mark Baxter, Anne-Birgitte Blavnsfeldt, Karine Briot, Catharina Chiari, Cyrus Cooper, Razvan Gabriel Dragoi, Gabriele G?bler, Willem Lems, Erika Mosor, Sandra Pais, Cornelia Simon, Paul Studenic, Simon Tilley, Jenny de la Torre-Aboki, Tanja A Stamm

doi : 10.1136/annrheumdis-2020-216931

Annals of the Rheumatic Diseases 2021;80:57-64.

Objective To establish European League Against Rheumatism (EULAR) points to consider for non-physician health professionals to prevent and manage fragility fractures in adults 50 years or older.

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2019 EULAR points to consider for the assessment of competences in rheumatology specialty training

Francisca Sivera, Alessia Alunno, Aurélie Najm, Tadej Avcin, Xenofon Baraliakos, Johannes W Bijlsma, Sara Badreh, Gerd Burmester, Nada Cikes, Jose AP Da Silva, Nemanja Damjanov, Maxime Dougados, Jean Dudler, Christopher J Edwards, Annamaria Iagnocco, Frédéric Lioté, Elena Nikiphorou, Marloes van Onna, Simon R Stones, Dimitrios Vassilopoulos, Catherine Haines, Sofia Ramiro

doi : 10.1136/annrheumdis-2020-218015

Annals of the Rheumatic Diseases 2021;80:65-70.

Background and aim Striving for harmonisation of specialty training and excellence of care in rheumatology, the European League Against Rheumatism (EULAR) established a task force to develop points to consider (PtCs) for the assessment of competences during rheumatology specialty training.

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Points to consider for the treatment of immune-mediated inflammatory diseases with Janus kinase inhibitors: a consensus statement

Peter Nash, Andreas Kerschbaumer, Thomas D?rner, Maxime Dougados, Roy M Fleischmann, Klaus Geissler, Iain McInnes, Janet E Pope, Désirée van der Heijde, Michaela Stoffer-Marx, Tsutomu Takeuchi, Michael Trauner, Kevin L Winthrop, Maarten de Wit, Daniel Aletaha, Xenofon Baraliakos, Wolf-Henning Boehncke, Paul Emery, John D Isaacs, Joel Kremer, Eun Bong Lee, Walter P Maksymowych, Marieke Voshaar, Lai-Shan Tam, Yoshiya Tanaka, Filip van den Bosch, René Westhovens, Ricardo Xavier, Josef S Smolen

doi : 10.1136/annrheumdis-2020-218398

Annals of the Rheumatic Diseases 2021;80:71-87.

Objectives Janus kinase inhibitors (JAKi) have been approved for use in various immune-mediated inflammatory diseases. With five agents licensed, it was timely to summarise the current understanding of JAKi use based on a systematic literature review (SLR) on efficacy and safety.

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Preliminary predictive criteria for COVID-19 cytokine stormEditor's Choice

Roberto Caricchio, Marcello Gallucci, Chandra Dass, Xinyan Zhang, Stefania Gallucci, David Fleece, Michael Bromberg, Gerard J Criner

doi : 10.1136/annrheumdis-2020-218323

Annals of the Rheumatic Diseases 2021;80:88-95.

Objectives To develop predictive criteria for COVID-19-associated cytokine storm (CS), a severe hyperimmune response that results in organ damage in some patients infected with COVID-19. We hypothesised that criteria for inflammation and cell death would predict this type of CS.

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Comparative effectiveness of first-line tumour necrosis factor inhibitor versus non-tumour necrosis factor inhibitor biologics and targeted synthetic agents in patients with rheumatoid arthritis: results from a large US registry study

Dimitrios A Pappas, Gregory St John, Carol J Etzel, Stefano Fiore, Taylor Blachley, Toshio Kimura, Rajeshwari Punekar, Kelechi Emeanuru, Jeannie Choi, Susan Boklage, Joel M Kremer

doi : 10.1136/annrheumdis-2020-217209

Annals of the Rheumatic Diseases 2021;80:96-102.

Objectives This study evaluated the comparative effectiveness of a tumour necrosis factor inhibitor (TNFi) versus a non-TNFi (biological disease-modifying antirheumatic drugs (bDMARDs) and targeted synthetic DMARDs (tsDMARDs)) as the first-line treatment following conventional synthetic DMARDs, as well as potential modifiers of response, observed in US clinical practice.

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Axial involvement in patients with early peripheral spondyloarthritis: a prospective MRI study of sacroiliac joints and spine

Thomas Renson, Philippe Carron, Ann-Sophie De Craemer, Liselotte Deroo, Manouk de Hooge, Simon Krabbe, Lennart Jans, Min Chen, Mikkel ?stergaard, Filip E Van den Bosch, Dirk Elewaut

doi : 10.1136/annrheumdis-2020-218480

Annals of the Rheumatic Diseases 2021;80:103-108.

Objectives To assess axial involvement on MRI in early peripheral spondyloarthritis (pSpA) and to evaluate whether axial inflammation predicts relapse on treatment withdrawal.

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Molecular pathways in patients with systemic lupus erythematosus revealed by gene-centred DNA sequencing

Johanna K Sandling, Pascal Pucholt, Lina Hultin Rosenberg, Fabiana H G Farias, Sergey V Kozyrev, Maija-Leena Eloranta, Andrei Alexsson, Matteo Bianchi, Leonid Padyukov, Christine Bengtsson, Roland Jonsson, Roald Omdal, Benedicte A Lie, Laura Massarenti, Rudi Steffensen, Marianne A Jakobsen, S?ren T Lillevang, on behalf of the ImmunoArray Development Consortium and DISSECT consortium, Karoline Lerang, ?yvind Molberg, Anne Voss, Anne Troldborg, S?ren Jacobsen, Ann-Christine Syv?nen, Andreas J?nsen, Iva Gunnarsson, Elisabet Svenungsson, Solbritt Rantap??-Dahlqvist, Anders A Bengtsson, Christopher Sj?wall, Dag Leonard, Kerstin Lindblad-Toh, Lars R?nnblom

doi : 10.1136/annrheumdis-2020-218636

Annals of the Rheumatic Diseases 2021;80:109-117.

Objectives Systemic lupus erythematosus (SLE) is an autoimmune disease with extensive heterogeneity in disease presentation between patients, which is likely due to an underlying molecular diversity. Here, we aimed at elucidating the genetic aetiology of SLE from the immunity pathway level to the single variant level, and stratify patients with SLE into distinguishable molecular subgroups, which could inform treatment choices in SLE.

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Genomic Risk Score impact on susceptibility to systemic sclerosis

Lara Bossini-Castillo, Gonzalo Villanueva-Martin, Martin Kerick, Marialbert Acosta-Herrera, Elena L?pez-Isac, Carmen P Sime?n, Norberto Ortego-Centeno, Shervin Assassi, International SSc Group, Australian Scleroderma Interest Group (ASIG), PRECISESADS Clinical Consortium, PRECISESADS Flow Cytometry study group, Nicolas Hunzelmann, Armando Gabrielli, J K de Vries-Bouwstra, Yannick Allanore, Carmen Fonseca, Christopher P Denton, Timothy RDJ Radstake, Marta Eugenia Alarc?n-Riquelme, Lorenzo Beretta, Maureen D Mayes, Javier Martin

doi : 10.1136/annrheumdis-2020-218558

Annals of the Rheumatic Diseases 2021;80:118-127.

Objectives Genomic Risk Scores (GRS) successfully demonstrated the ability of genetics to identify those individuals at high risk for complex traits including immune-mediated inflammatory diseases (IMIDs). We aimed to test the performance of GRS in the prediction of risk for systemic sclerosis (SSc) for the first time.

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Fast diagnostic test for familial Mediterranean fever based on a kinase inhibitor

Flora Magnotti, Tiphaine Malsot, Sophie Georgin-lavialle, Fatima Abbas, Amandine Martin, Alexandre Belot, Maxime Fauter, Muriel Rabilloud, Mathieu Gerfaud-Valentin, Pascal Sève, Agnes Duquesne, Arnaud Hot, Stephane Durupt, Léa Savey, Irina Giurgea, Gilles Grateau, Thomas Henry, Yvan Jamilloux

doi : 10.1136/annrheumdis-2020-218366

Annals of the Rheumatic Diseases 2021;80:128-132.

Background and objective Familial Mediterranean fever (FMF) is the most frequent hereditary autoinflammatory disease. Its diagnosis relies on a set of clinical criteria and a genetic confirmation on identification of biallelic pathogenic MEFV variants. MEFV encodes pyrin, an inflammasome sensor. Using a kinase inhibitor, UCN-01, we recently identified that dephosphorylation of FMF-associated pyrin mutants leads to inflammasome activation. The aim of this study was to assess whether quantifying UCN-01-mediated inflammasome activation could discriminate FMF patients from healthy donors (HD) and from patients with other inflammatory disorders (OID).

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Age-based (<65 vs ?65 years) incidence of infections and serious infections with tofacitinib versus biological DMARDs in rheumatoid arthritis clinical trials and the US Corrona RA registry

Kevin L Winthrop, Gustavo Citera, David Gold, Dan Henrohn, Carol A Connell, Andrea B Shapiro, Harry Shi, Alina M Onofrei, Dimitrios A Pappas, Hendrik Schulze-Koops

doi : 10.1136/annrheumdis-2020-218992

Annals of the Rheumatic Diseases 2021;80:134-136.

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Management of rheumatic diseases in the time of covid-19 pandemic: perspectives of rheumatology practitioners from India

Latika Gupta, Durga Prasanna Misra, Vishwesh Agarwal, Suma Balan, Vikas Agarwal

doi : 10.1136/annrheumdis-2020-217509

Annals of the Rheumatic Diseases 2021;80:e1.

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Antirheumatic agents in covid-19: is IL-6 the right target?

Pier Leopoldo Capecchi, Pietro Enea Lazzerini, Luca Volterrani, Maria Antonietta Mazzei, Barbara Rossetti, Giacomo Zanelli, David Bennett, Elena Bargagli, Federico Franchi, Matteo Cameli, Serafina Valente, Luca Cantarini, Bruno Frediani

doi : 10.1136/annrheumdis-2020-217523

Annals of the Rheumatic Diseases 2021;80:e2.

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The conundrum of COVID-19 treatment targets: the close correlation with rheumatology. Response to: ‘Management of rheumatic diseases in the time of covid-19 pandemic: perspectives of rheumatology pracitioners from India’ by Gupta et al and ‘Antirheumatic agents in covid-19: is IL-6 the right target?’ by Capeechi et al

Sara Monti, Carlomaurizio Montecucco

doi : 10.1136/annrheumdis-2020-217545

Annals of the Rheumatic Diseases 2021;80:e3.

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Online management of rheumatoid arthritis during COVID-19 pandemic

Yang Zhang, Jian Wang, Liang Zhao, Jun Xiao, Zhanjun Shi

doi : 10.1136/annrheumdis-2020-217548

Annals of the Rheumatic Diseases 2021;80:e4.

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Challenges and opportunities in telerheumatology in the COVID-19 era. Response to: ‘Online management of rheumatoid arthritis during COVID-19 pandemic’ by Zhang et al

Gabriel Figueroa-Parra, Carmen Magdalena Gamboa-Alonso, Dionicio Angel Galarza-Delgado

doi : 10.1136/annrheumdis-2020-217631

Annals of the Rheumatic Diseases 2021;80:e5.

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Hydroxychloroquine reduces the risk of covid-19 in patients with rheumatic diseases: myth or reality?

Wenhui Xie, Yu Wang, Zhuoli Zhang

doi : 10.1136/annrheumdis-2020-217556

Annals of the Rheumatic Diseases 2021;80:e6.

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Still early to define a clear role of antimalarial drugs for COVID-19 in patients with rheumatic disease. Response to: ‘Hydroxychloroquine reduces the risk of covid-19 in patients with rheumatic diseases: myth or reality?’ by Xie et al

Carmen Magdalena Gamboa-Alonso, Gabriel Figueroa-Parra, Dionicio Angel Galarza-Delgado

doi : 10.1136/annrheumdis-2020-217633

Annals of the Rheumatic Diseases 2021;80:e7.

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To consider or not antimalarials as a prophylactic intervention in the SARS-CoV-2 (COVID-19) pandemic

Konstantinos Parperis

doi : 10.1136/annrheumdis-2020-217557

Annals of the Rheumatic Diseases 2021;80:e8.

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Response to ‘To consider or not antimalarials as a prophylactic intervention in the SARS-CoV-2 (Covid-19) pandemic’ by Parperis

Francesca Romana Spinelli, Fulvia Ceccarelli, Manuela Di Franco, Fabrizio Conti

doi : 10.1136/annrheumdis-2020-217634

Annals of the Rheumatic Diseases 2021;80:e9.

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Rituximab for granulomatosis with polyangiitis in the pandemic of covid-19: lessons from a case with severe pneumonia

Philippe Guilpain, Clément Le Bihan, Vincent Foulongne, Patrice Taourel, Nathalie Pansu, Alexandre Thibault Jacques MARIA, Boris Jung, Romaric Larcher, Kada Klouche, Vincent Le Moing

doi : 10.1136/annrheumdis-2020-217549

Annals of the Rheumatic Diseases 2021;80:e10.

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Diagnostic and therapeutic challenges for patients with ANCA-associated vasculitides at the time of COVID-19. Response to: ‘Rituximab for granulomatosis with polyangiitis in the pandemic of COVID-19: lessons from a case with severe pneumonia’ by Guilpain et al

Sara Monti, Carlomaurizio Montecucco

doi : 10.1136/annrheumdis-2020-217555

Annals of the Rheumatic Diseases 2021;80:e11.

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