SARCOIDOSIS IN SYSTEMIC-SCLEROSIS - REPORT OF 7 CASES

Objective. To describe the clinical, radiologic, and pathologic featur es of coexistent systemic sclerosis (SSc) and sarcoidosis, 2 condition s of unknown cause associated with altered cellular immune response. M ethods. We reviewed clinical information, results from laboratory and radiologic studies, and lung or lymph node biopsy samples of 7 patient s with concurrent SSc and sarcoidosis evaluated at 2 academic referral centers between 1989 and 1993. Results, Each patient fulfilled Americ an College of Rheumatology criteria for the classification of SSc. SSc and sarcoidosis developed simultaneously in 4 patients, whereas in 3 others sarcoidosis was diagnosed more than 6 years after the onset of SSc. The onset of sarcoidosis was characterized by fever, weight loss, or increasing respiratory symptoms. Each patient had radiographic evi dence of intrathoracic lymphadenopathy and/or interstitial lung diseas e. Examination of lung or lymph node biopsies demonstrated noncaseatin g granulomas. Treatment with corticosteroids was associated with impro ved lung function. Conclusion, Since sarcoidosis coexists with SSc mor e frequently than previously suggested, it should be considered in pat ients with SSc presenting with new pulmonary symptoms, Recognizing sar coidosis in patients with SSc is important, since these patients may b enefit from corticosteroid therapy.