In:
Annals of the Rheumatic Diseases, BMJ, Vol. 81, No. Suppl 1 ( 2022-06), p. 742-742
Abstract:
The idiopathic inflammatory myopathies (IMM) module of the Rheumatic Diseases Portuguese Register (Reuma.pt/Myositis) is a tool used to systematically evaluate IIM patients. Objectives To clinically characterise the Reuma.pt/Myositis cohort. Methods Multicentre open cohort study, including IIM patients registered in Reuma.pt up to January 2022. Data collected included demographic, clinical, and treatment data and patient-reported outcomes. Data were presented as frequencies and median (interquartile range) for categorical and continuous variables, respectively. Results 280 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, respectively. Patients were classified as having definite (N=57/118, 48.3%; N=35/224, 15.6%), likely (N=23/118, 19.5%; N=50/224, 22.3%), or possible (N=2/118, 1.7%; N=46/224, 20.5%) IIM by 2017 EULAR/ACR and Bohan-Peter criteria, respectively. Disease subtypes included dermatomyositis (DM, N=122/280, 43.6%), polymyositis (N=59/280, 21.1%), myositis in overlap syndromes (N=41/280, 14.6%), clinically amyopathic DM (N=17/280, 6.1%), nonspecific myositis (N=13/280, 4.6%), mixed connective tissue disease (N=12/280, 4.3%), immune-mediated necrotizing myositis (N=9/280, 3.2%), and inclusion bodies myopathy (N=7/280, 2.5%). Over the course of the disease, the most common symptoms were proximal muscle weakness (N=180/215, 83.7%), arthralgia (N=127/249, 52.9%), erythema (N=63/166, 38.0%), fatigue (N=47/127, 37.0%), Raynaud’s phenomenon (N=76/234, 32.5%), and dysphagia (N=33/121, 27.3%), and the most common clinical signs were Gottron’s sign (N=75/184, 40.8%), heliotrope rash (N=101/252, 40.1%), Gottron’s papules (N=93/237, 39.2%), and arthritis (N=38/98, 38.8%). Organ involvement included lung (N=78/230, 33.9%), oesophageal (N=40/221, 18.1%), and heart (N=11/229, 4.8%) involvements. Most patients expressed myositis-specific (MSA, N=158/242, 65.3%) and/or myositis-associated (MAA, 112/242, 46.3%) antibodies. The most frequent antibodies 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%) at diagnosis, with median highest CK levels of 1308 (518-3172) and aldolase of 42 (12-121) mg/dL. Neoplasia was found in 11/127 patients (8.7%), most commonly breast (N=3/11, 27.3%), non-melanoma skin (N=2/11, 18.2%), and colorectal (N=2/11, 18.2%) cancer (Table 1). 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 over the course of disease 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%), mycophenolate mofetil (N=56/280, 20.0%), intravenous immunoglobulin (N=55/280, 19.6%), and rituximab (N=45/280, 16.1%). 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). Table 1. Autoantibodies in cancer-associated myositis Cancer IIM Autoantibodies Breast DM (3) Mi2, SRP (+ SSA/SSB), Pm/Scl Skin (non-melanoma) Clinically amyopathic DM, PM Jo1, SAE1 (+SSA/SSB) Colorectal DM (2) Mi2 (2) Kidney DM - Lung DM - Lymphoma Inclusion bodies myopathy - Unknown DM - Conclusion 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. Of interest, most patients reached low disease activity at the last follow-up appointment. Disclosure of Interests None declared
Type of Medium:
Online Resource
ISSN:
0003-4967
,
1468-2060
DOI:
10.1136/annrheumdis-2022-eular.3259
Language:
English
Publisher:
BMJ
Publication Date:
2022
detail.hit.zdb_id:
1481557-6
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