Pure extra-thoracic sarcoidosis: about 24 cases

Abstract

Introduction. Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia. Methods. Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January 1996 to December 2016. Results. Twenty-four patients presented pure extra-thoracic sarcoidosis (30% of the cases of systemic sarcoidosis). There was a female predominance (79%). The mean age was 50 years. The main features were polyadenopathies (10 patients: 41.7%), cutaneous involvement (10 patients: 41.6%), inflammatory polyarthralgia (8 cases: 33.3%), general symptoms (6 patients: 25%), uveitis (6 cases: 25%), cholestasis (3 cases) and kidney involvement (2 cases). Lymphopenia, hypercalcemia, and hypercalciuria were observed each one in 12.5% of the cases, and high angiotensin converting enzyme (ACE) level in 38.5% of the cases. The histological proof was required in all the patients. Statistically significant associations identified were mainly hepatic involvement with general symptoms (p=0.035), peripheral lymphadenopathies (p=0.035) and kidney damage (p=0.022), and cutaneous involvement with articular manifestations (p=0.032). Systemic corticosteroids were used in half of the cases, with a good outcome in 21 cases, and recurrence in two cases. One patient was lost to follow up. The comparison to the form with mediastino-pulmonary involvement showed less lymphadenopathies (p=0.001), less lymphopenia (p=0.006), and less frequent use of corticosteroids (p=0.044). Conclusion. Our series was characterized by the frequency of the pure extra-thoracic form and the diversity of the systemic manifestations.

Keywords: Tunisia; glucocorticoids; granuloma; lymphadenopathy; sarcoidosis; uveitis.