Sarcoidosis in systemic sclerosis: report of 7 cases

To describe the clinical, radiologic, and pathologic features of coexistent systemic sclerosis (SSc) and sarcoidosis, 2 conditions of unknown cause associated with altered cellular immune response.

We reviewed clinical information, results from laboratory and radiologic studies, and lung or lymph node biopsy samples of 7 patients with concurrent SSc and sarcoidosis evaluated at 2 academic referral centers between 1989 and 1993.

Each patient fulfilled American 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 evidence of intrathoracic lymphadenopathy and/or interstitial lung disease. Examination of lung or lymph node biopsies demonstrated noncaseating granulomas. Treatment with corticosteroids was associated with improved lung function.

Since sarcoidosis coexists with SSc more frequently than previously suggested, it should be considered in patients with SSc presenting with new pulmonary symptoms. Recognizing sarcoidosis in patients with SSc is important, since these patients may benefit from corticosteroid therapy.