Abstract

Background Idiopathic inflammatory myopathies (IIM) is a heterogeneous group of autoimmune diseases with a broad spectrum of clinical presentations including musculoskeletal, respiratory, neurological, dermatological, gastrointestinal and cardiovascular symptoms. It is known that presence of autoantibodies is a predictor of disease course. However, there is very limited data summarizing long-term observations assessing, when the particular symptoms are expected to occur. Objectives To assess the development of the symptoms in IIM patients during the disease course with respect to their serology patterns. Methods Medical history of IIM patients treated in our clinic between 2008 and 2019 were reviewed. We excluded patients with: insufficient data for the first period of disease course, history of IIM Results Numbers below are presenting MTSO in months for each symptom. Anti-Jo1 antibodies positive patients (n:30) have presented: rash (0), mechanic hands (1.2), myocarditis (5), muscle weakness (10.3), Raynaud’s phenomenon (RP) (10.4), arthritis (20.7), interstitial lung disease (ILD) (23.5), fever (29.1), Gottron’s sign (31.3), muscle pain (46.2), dysphagia (53.4). Anti-Mi-2 antibodies positive patients (n:12) have presented: RP (0), mechanic hands (0), rash (0.2), Gottron’s sign (0.3), fever (2.5), muscle weakness (4.5), dysphagia (4.5), muscle pain (9), ILD (15.5). Anti-PM-Scl antibodies positive patients (n:7) have presented: rash (1), fever (1), Gottron’s sign (1.25), mechanic hands (4), ILD (4), RP (5), arthritis (10.2), muscle weakness (46.3), muscle pain (108.5). Anti-SRP antibodies positive patients (n:5) have presented: muscle weakness, fever, arthritis and ILD symptoms simultaneously (0), then dysphagia (4) and muscle pain (10). Anti-NXP2 antibodies positive patients (n:4) have presented: muscle weakness and pain simultaneously with dysphagia (0), ILD developed 4 months later. Anti-MDA5 antibodies positive patients (n:2) have presented: arthritis (0), fever (0.5), muscle pain (2), rash (3). Anti-Ro52 antibodies positive patients (n:12) have presented: RP (3), Gottron’s sign (3.3), rash (3.5), arthritis (5), muscle weakness (6.2), muscle pain (11), myocarditis (11), ILD (12), mechanic hands (13), dysphagia (18.3). MSAs and MAAs negative patients (n:21) have presented: fever (0), RP (0), dysphagia (0,3), muscle pain (0,4), muscle weakness (1.9), rash (20), arthritis (66). Conclusion Our study shows differences in disease course in IIM patients with each serology pattern adding new data on chronology of appearances of each symptom, which is important in predicting the future course of the disease and planning long-term management.

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