Clinical analysis of polymyositis and dermatomyositis with respiratory failure as the initial presentation / 中华风湿病学杂志

ABSTRACT

Objective Analyzing polymyositis (PM) and dermatomyositis (DM) cases that had respiratory failure at the initial presentation in order to get a better understanding of their clinical features. Methods Eight PM or DM patients with respiratory failure as their first symptom were included in this study. Their clinical manifestation, laboratory and radiology changes and prognosis were analyzed retrospectively. Results In 8 patients, respiratory failure occurred before skin and muscle symptom for about two weeks to 3 month. Pulmonary interstitial disease was revealed by chest X ray and CT. Pulse methylprednisonlone was used combined with anti-infection and ameliorating respiratory function treatment. Five of the patients became deteriorated and 3 died of respiratory failure despite of the treatment, while the other 3 improved but one died of respiratory failure 6 months later. Conclusion About 5%~30% patients with PM and DM have lung damage. Pulmonary changes may occur before skin and muscle symptoms in 4% patients. Such patients have abrupt onsets and their disease progresses quickly with high mortality despite of corticosteroids therapy.