engleski

Pulmonary manifestations of connective tissue disease(CTD) ; lung invlvement as the initial sign of CTD

Numerous connective tissue diseases (CTD) involve the lungs, but most frequently systemic sclerosis (SSc), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), polymyositis/dermatomyositis (PM/DM), mixed connective tissue disease (MCTD) and Sjögren syndrome (SS). Many other connective tissue diseases (i.e. overlap syndromes, ankylosing spondylitis) also show number of different pulmonary manifestations. Interstitial lung disease, bronchiolitis, bronchiectasis, pleural effusion, respiratory muscle weakness, diffuse alveolar hemorrhage and pulmonary hypertension are seen. The patients with established diagnosis of connective tissue disease and lung affection are in most cases a therapeutic problem. The early recognition of these complications is important because timely and adequate, often aggressive immunosuppressive therapy is needed in order to preserve organ integrity and function. On the other hand, there is a group of patients who present as interstitial lung disease, pleural effusion, diffuse alveolar hemorrhage or bronchiolitis, as the commencing, initial sign of connective tissue disease. Here we present a group of 26 patients with interstitial lung disease in which during time, months or even years the connective tissue disease was diagnosed. Finally, there were 6 pts with SSc, five with RA, five with MCTD, four SLE, two with PM/DM, and the rest were unclassified CTD. The characteristics of clinical presentation, diagnostic procedures and prognosis are shown.

connective tissue disease, pulmonary manifestations

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