Navegando por Palavras-chave "Antisynthetase Syndrome"

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    Características clínicas, resposta ao tratamento e sobrevida em uma coorte de doenças pulmonares intersticiais com achados de miosites
    (Universidade Federal de São Paulo (UNIFESP), 2021) Miranda, Gustavo Frazatto Medeiros De [UNIFESP]; Pereira, Carlos Alberto De Castro [UNIFESP]; Universidade Federal de São Paulo
    Introduction: Interstitial lung diseases (ILD) include a large number of conditions, which can sometimes be associated with myositis findings. Objectives: The aims of present study were to compare the clinical, functional, and tomographic characteristics, treatment response and survival in a Brazilian cohort of patients with ILD and myositis, classified into two groups: (1) With myositis-specific antibodies (MSA); and (2) myositis-associated antibodies (MAA) or clinical features of myositis. Methods: The diagnosis of myositis was characterized by: (1) presence of a mechanic's hands; or (2) elevation of muscle enzymes (creatine phosphokinase or aldolase) 2.5 times above the upper limit of normal associated with subjective muscle weakness or myalgia. All patients included in the study had pulmonary involvement on high-resolution chest tomography (HRCT). Data between the two groups were compared by t-test, median test, chi-square test, Fisher's exact test and survival curves were compared by Kaplan-Meier test. Significant functional decline was characterized by decrease in forced vital capacity (FVC) ≥ 5% of predicted value. Results: Mean age was 51 years and median follow-up was 50 months [IQ=25 - 98]. The mean FVC was 61.9 ± 16.6%. Among 77 patients analyzed, 43 (55.9%) were included in group 1, and 34 (44.1%) in group 2. The groups were similar, except that group 2 had more male subjects (61% versus 39%), more commonly audible pulmonary crackles (91% versus 72) and no SD pattern in capillaroscopy. There was no difference in types and duration of treatment between groups, neither in survival. Overall survival was greater than 80% at five years. Predictive factors for shorter survival were the presence honeycombing on HRCT (log-rank=34.65; p<0.001), and a decrease in FVC from 5% of predicted in longitudinal assessment (log-rank=16.50; p<0.001). Conclusion: Clinical, functional, and tomographic characteristics are similar between patients with ILD and presence of specific antisynthetase antibodies, and those with clinical findings of myositis, with or without associated antibodies. Presence of honeycombing on HRCT and a decrease in predicted FVC ≥ 5% are predictors of lower survival.