Background Idiopathic inflammatory myopathies (IIMs) include a group of muscular diseases characterized by the presence of muscle inflammation. The mortality of the IIMs has been estimated between 13 and 50%.
Objectives To evaluate mortality rate and associated factors in patients with IIMs.
Methods Retrospective, observational study, where patients with IIMs (Bohan & Peter 1975) were included. Data were obtained from medical records from patients with myopathy (Increase CK, muscle weakness, cutaneous involvement, interstitial lung involvement) evaluated in a reference rheumatology center of Argentina (1992–2016). Descriptive statistics were performed. Chi2 test, Student's test or Mann Whitney as appropriate multivariate logistic regression analysis.
Results From 102 patients evaluated 89 enter the study, 73% were female. Mean age at diagnosis 48±14 years. Clinical Manifestations: Skin involvement 77% (erythema Heliotrope 51%, rash on the neck and V-sign 60%, back and shoulders 50%, photosensitivity 60%, Gottron's papules 50%, pruritus 33%, erythema peri nail 21%), pulmonary involvement 19% Raynaud 28%, muscle weakness 86%, muscle weakness of the neck 33%, respiratory muscles 13%, myalgias 60% and dysphagia 53%.
Muscle biopsy: performed in 36/89 with pathological findings in 83%, electromyogram performed in 35%. Intensive care unit admission 14/89 (16%). Laboratory: raised CPK 68% with an mean value 3527 IU/ml, raised Transaminase 60%, ANA positive 65%, SSA/RO 25%, Jo1 4.4%, RNP 7%, increased CRP 28% and ERA 59%.
Clinical Subtypes IIMs: Dermatomyositis (DM): 61%, Antisynthetic syndrome (AS): 6%, Myopathy associated with connective tissue disease: 19%, Associated with statins: 4, 4%, Polimyositis: 10%. Association with neoplasia was observed in 15%. Treatments: Corticoids pulses 21%, corticoids 97% (mean starting dose 45 mg meprednisone), methotrexate 77%, hydroxychloroquine 36%, azathioprine 30%, cyclophosphamide 16%, intravenous immunoglobulin 15%, biological 10% and cyclosporine 3%.
Mortality was 22.5% (20/89 patients), CI95 (14.3–32.5). Mean time from diagnosis to the event was 18 months. The primary cause of death was sepsis 14/20 (70%).
Conclusions Mortality of patients with inflammatory myopathies was 22%, and the primary cause was infectious. In the analysis of multiple variables, male sex, presence of neoplasms and serious infectious complications were significantly factors associated with mortality.
J Clin Rheumatol 2016; 22: 51–56.
Med Clin 1999; 112:521–6.
Disclosure of Interest None declared