The idiopathic inflammatory myopathies module of the Rheumatic Diseases Portuguese Register
Authors
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 Teodósio 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;
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
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Ana Teresa Melo
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Raquel Campanilho-Marques
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Matilde Bandeira
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Patrícia Martins
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Vanessa Fraga
Hospital Garcia de Orta
José Luís Ferraro
Hospital Beatriz Ângelo
André Saraiva
Centro Hospitalar Universitário de Coimbra
Marlene Sousa
Centro Hospitalar Universitário de Coimbra
Hugo Parente
Unidade Local de Saúde do Alto Minho
Catarina Soares
Unidade Local de Saúde do Alto Minho
Ana Margarida Correia
Hospital de Braga
Diogo Esperança Almeida
Hospital de Braga
Sara Paiva Dinis
Unidade Local de Saúde da Guarda
Ana Sofia Pinto
Unidade Local de Saúde da Guarda
Filipe Oliveira Pinheiro
Centro Hospitalar Universitário de São João
Maria Seabra Rato
Centro Hospitalar Universitário de São João
Tiago Beirão
Centro Hospitalar de Vila Nova de Gaia / Espinho
Beatriz Samões
Centro Hospitalar de Vila Nova de Gaia / Espinho
Bernardo Santos
Centro Hospitalar do Baixo Vouga
Carolina Mazeda
Centro Hospitalar do Baixo Vouga
Ana Teodósio Chícharo
Centro Hospitalar Universitário do Algarve
Margarida Faria
Hospital Dr. Nélio Mendonça
Agna Neto
Hospital Dr. Nélio Mendonça
Maria Helena Lourenço
Centro Hospitalar de Lisboa Ocidental
Luísa Brites
Centro Hospitalar de Leiria
Marília Rodrigues
Centro Hospitalar de Leiria
Joana Silva-Dinis
Centro Hospitalar Universitário Lisboa Central
João Madruga Dias
Centro Hospitalar de Médio Tejo
Filipe C. Araújo
Hospital CUF Cascais
Nádia Martins
Centro Hospitalar Tondela-Viseu EPE
Maura Couto
Centro Hospitalar Tondela-Viseu EPE
Ana Valido
Unidade Local de Saúde do Litoral Alentejano
Maria José Santos
Hospital Garcia de Orta
Sofia Barreira
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
João Eurico Fonseca
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Ana Teresa Melo
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Raquel Campanilho-Marques
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Matilde Bandeira
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Patrícia Martins
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
Vanessa Fraga
Hospital Garcia de Orta
José Luís Ferraro
Hospital Beatriz Ângelo
André Saraiva
Centro Hospitalar Universitário de Coimbra
Marlene Sousa
Centro Hospitalar Universitário de Coimbra
Hugo Parente
Unidade Local de Saúde do Alto Minho
Catarina Soares
Unidade Local de Saúde do Alto Minho
Ana Margarida Correia
Hospital de Braga
Diogo Esperança Almeida
Hospital de Braga
Sara Paiva Dinis
Unidade Local de Saúde da Guarda
Ana Sofia Pinto
Unidade Local de Saúde da Guarda
Filipe Oliveira Pinheiro
Centro Hospitalar Universitário de São João
Maria Seabra Rato
Centro Hospitalar Universitário de São João
Tiago Beirão
Centro Hospitalar de Vila Nova de Gaia / Espinho
Beatriz Samões
Centro Hospitalar de Vila Nova de Gaia / Espinho
Bernardo Santos
Centro Hospitalar do Baixo Vouga
Carolina Mazeda
Centro Hospitalar do Baixo Vouga
Ana Teodósio Chícharo
Centro Hospitalar Universitário do Algarve
Margarida Faria
Hospital Dr. Nélio Mendonça
Agna Neto
Hospital Dr. Nélio Mendonça
Maria Helena Lourenço
Centro Hospitalar de Lisboa Ocidental
Luísa Brites
Centro Hospitalar de Leiria
Marília Rodrigues
Centro Hospitalar de Leiria
Joana Silva-Dinis
Centro Hospitalar Universitário Lisboa Central
João Madruga Dias
Centro Hospitalar de Médio Tejo
Filipe C. Araújo
Hospital CUF Cascais
Nádia Martins
Centro Hospitalar Tondela-Viseu EPE
Maura Couto
Centro Hospitalar Tondela-Viseu EPE
Ana Valido
Unidade Local de Saúde do Litoral Alentejano
Maria José Santos
Hospital Garcia de Orta
Sofia Barreira
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa
João Eurico Fonseca
Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa