[Pulmonary damage during polymyositis and dermatomyositis: interstitial lung disease]

Abstract

Interstitial lung disease is a serious complication of polymyositis/dermatomyositis and leads to death from chronic respiratory insufficiency in 30 to 66% of cases. It is a criterion of poor prognosis in these disorders. Its onset occurs at variable points in the course of polymyositis/dermatomyositis, and precedes them in half of all cases. Presentation may also vary: acute (25%), insidious (60%), or infraclinical, discovered fortuitously (15%). The examinations of choice for early screening are high-resolution computed tomography (CT) and pulmonary function tests, which should be performed during the initial work-up and during ongoing surveillance. Moreover, high-resolution CT also makes it possible to determine the type of histologic lesions in the interstitial lung disease. Today, diagnosis of this disease does not generally require histological confirmation; nonspecific interstitial lung disease seems to be the most common histologic form of lung damage in polymyositis/dermatomyositis (40 to 80%). Anti-Jo1 antibodies are a sensitive marker of interstitial lung disease during polymyositis/dermatomyositis, and close surveillance of lung function is recommended in patients with these autoantibodies. Systematic testing for them is also justified in patients with apparently idiopathic interstitial lung disease, to rule out underlying polymyositis/dermatomyositis. No clear treatment protocols have been established for interstitial lung disease during polymyositis/dermatomyositis. Corticosteroid treatment is the first choice. Its combination with cyclophosphamide may be most effective in corticosteroid-resistant forms of polymyositis/dermatomyositis, especially when begun early; it may also be appropriate to begin corticosteroids as soon as factors predicting poor prognosis are detected.