Acta Reumatológica Portuguesa
The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register
Aims: To characterise the idiopathic inflammatory myopathies (IIM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/myositis) and the patients in its cohort. Methods: Reuma.pt is a web-based system with standardised patient files gathered in a registry. This was a multicentre open cohort study, including patients registered in Reuma.pt/myositis up to January 2022. Results: Reuma.pt/myositis was designed to record all relevant data in clinical practice and includes disease-specific diagnosis and classification criteria, clinical manifestations, immunological data, and disease activity scores. Two hundred eighty patients were included, 71.4% female, 89.4% Caucasian, with a median age at diagnosis and disease duration of 48.9 (33.6-59.3) and 5.3 (3.0-9.8) years. Patients were classified as having definite (N=57/118, 48.3%), likely (N=23/118, 19.5%), or possible (N=2/118, 1.7%) IIM by 2017 EULAR/ACR criteria. The most common disease subtypes were dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), and myositis in overlap syndromes (N=41/280, 14.6%). The most common symptoms were proximal muscle weakness (N=180/215, 83.7%) and arthralgia (N=127/249, 52.9%), and the most common clinical signs were Gottron’s sign (N=75/184, 40.8%) and heliotrope rash (N=101/252, 40.1%). Organ involvement included lung (N=78/230, 33.9%) and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent were anti-SSA/SSB (N=70/231, 30.3%), anti-Jo1 (N=56/236, 23.7%), and anti-Mi2 (N=31/212, 14.6%). Most patients had a myopathic pattern on electromyogram (N=101/138, 73.2%), muscle oedema in magnetic resonance (N=33/62, 53.2%), and high CK (N=154/200, 55.0%) and aldolase levels (N=74/135, 54.8%). Cancer was found in 11/127 patients (8.7%), most commonly breast cancer (N=3/11, 27.3%). Most patients with cancer-associated myositis had DM (N=8/11, 72.7%) and expressed MSA (N=6/11) and/or MAA (N=3/11). The most used drugs were glucocorticoids (N=201/280, 71.8%), methotrexate (N=117/280, 41.8%), hydroxychloroquine (N=87/280, 31.1%), azathioprine (N=85/280, 30.4%), and mycophenolate mofetil (N=56/280, 20.0%). At the last follow-up, there was a median MMT8 of 150 (142-150), modified DAS skin of 0 (0-1), global VAS of 10 (0-50) mm, and HAQ of 0.125 (0.000-1.125). Conclusions: Reuma.pt/myositis adequately captures the main features of inflammatory myopathies’ patients, depicting, in this first report, a heterogeneous population with frequent muscle, joint, skin, and lung involvements.
Eduardo Dourado, Ana Teresa Melo, Raquel Campanilho-Marques, Matilde Bandeira, Patrícia Martins, Vanessa Fraga, José Luís Ferraro, André Saraiva, Marlene Sousa, Hugo Parente, Catarina Soares, Ana Margarida Correia, Diogo Esperança Almeida, Sara Paiva Dinis, Ana Sofia Pinto, Filipe Oliveira Pinheiro, Maria Seabra Rato, Tiago Beirão, Beatriz Samões, Bernardo Santos, Carolina Mazeda, Ana Chícharo, Margarida Faria, Agna Neto, Maria Helena Lourenço, Luísa Brites, Marília Rodrigues, Joana Silva-Dinis, João Madruga Dias, Filipe C. Araújo, Nádia Martins, Maura Couto, Ana Valido, Maria José Santos, Sofia Barreira, João Eurico Fonseca. The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register. ARP Rheumatology, Vol 2, nº3 2023:188-199. PMID: 37728117