Pulmonary involvement in systemic sclerosis. Part I: Chronic pulmonary interstitial fibrosis.

Introduction. - Chronic pulmonary interstitial fibrosis is the most frequen t respiratory manifestation in systemic sclerosis, occurring in 80% of case s. It remains a severe complication of the disease and is the primary cause of mortality related to respiratory insufficiency in 20 to 60% of cases. Current knowledge and key points. - The date of onset of interstitial lung disease remains undetermined, and only in rare cases does it reveal the pre sence of systemic sclerosis. The clinical signs are only observable at a la ter stage, when at least 50% of the lung parenchyma is affected. The method s of choice adopted for early diagnosis of this disease are high resolution computed tomography and pulmonary functional investigations; they should b e carried out during the preliminary investigation and at follow-up once a year. Moreover, high resolution computed tomography also provides prognosti c data, for there is a correlation between the type of lesion and its sever ity as determined by high resolution computed tomography and by histologica l findings. The value of other methods of investigation, in particular bron choalveolar lavage, has not yet been clearly established. The association o f cyclophosphamide and corticoids is currently being evaluated (indications , administration modalities, duration), and this combination may be the mos t effective treatment. Future prospects and projects. - Interstitial lung disease is one of the ma jor causes of morbidity and mortality in systemic sclerosis. Early diagnosi s and management of this disease in therefore of utmost importance. (C) 199 9 Editions scientifiques et medicales Elsevier SAS.