Broncheoalveolar Fluid Granulocytosis Predicts Progression of Fibrosing Alveolitis in Patients with Systemic Sclerosis

Journal/Book: Z Rheumatol 1998; 57 Suppl. 1: 37 (F 47). 1998;

Abstract: Department of Medicine Charité University Hospital Berlin Objective. About 70 % of patients with systemic sclerosis (SSc) develop pulmonary involvement. The disease course may vary considerably from stable condition for years to fatal progressive interstitial fibrosis. Aim of the study was to determine parameters that allow to predict the prognosis of fibrosing alveolitis (FA) associated with SSc. Methods. Seventy-three consecutive patients with SSc according to the American College of Rheumatology preliminary criteria for the diagnosis of SSc were enrolled in this study. All patients had clinical lung functional and/or CT sings of interstitial lung involvement. Sixty-six patients (90 %) were positive for antinuclear antibodies (ANA) and 32 (44 %) had antibodies against topoisomerase I (anti-Scl 70). During the study 36 patients (49 %) were on a low-dose prednisolone therapy (< 12.5 mg/day) 6 patients received low-dose prednisolone plus azathioprine and 2 patients each were on a azathioprine or D-penicillamine monotherapy. Every patient underwent clinical examination lung function tests computed tomography (CT) gallium scan and bronchoalveolar lavage (BAL). Fourty-nine patients 26 with pathologic and 23 with normal BAL fluid (BALF) findings were prospectively followed for two years and revaluated annually. Results. At baseline 51 subjects (70 %) showed radiologic signs of lung fibrosis and/or alveolitis by CT scanning. Pathologic BALF findings were demonstrated in 35 patients (48 %). BALF differential counts included BALF granulocytosis in 18 BALF lymphocytosis in 12 and a mixed increase of both granulocytes and lymphocytes in 5 patients. On follow-up only patients with BALF granulocytosis showed progression of FA with a significant decrease of diffusion capacity for carbon monoxide. ... le

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