Peritoneal and pulmonary sarcoidosis: an extremely rare entity

H. L. Chan, F. Pugin, M. Menth, B. Egger

Objective

Sarcoidosis is a chronic, multisystemic, granulomatous disease of unknown etiology which may affect every organ system. However, >95% of patients with sarcoidosis have lung involvement and peritoneal lesions are quite rare. Most of these patients present with ascites and abdominal pain. We present here one of this rare cases with peritoneal and pulmonary manifestations of sarcoidosis.

Methods

A 29 year-old Ethiopian female patient, asylum seeker, was admitted for investigation of dyspnea, dry cough and epigastric pain. She reported fatigue, night sweats and a weight loss of 6 kg during the last 6 months. CT-scan revealed pleural effusion and also the presence of ascites together with nodular lesions of peritoneal surface. Thoracic and abdominal liquids punctured were serous with a lymphocytic predominance. Ziehl-Neelsen stain and cultures for tuberculosis were negative, no malignant cells were found. Laboratory values demonstrated a moderate systemic inflammation but normal angiotensin-converting enzyme levels. Last but not least a diagnostic laparoscopy with biopsies of some peritoneal nodules was performed.

Results

Histology revealed noncaseating granulomas with multinucleated giant cells. Ziehl-Neelsen and Gomori methenamine silver stains were negative and there were no signs for malignancy. Based on all these findings the diagnosis of sarcoidosis was made. The patient underwent a corticosteroid administration and was discharged home 15 days later. The 3-months follow-up was completely uneventful.

Conclusion

Sarcoidosis is a granulomatous disorder of unknown cause with a worldwide annual incidence of 0.56 per 100000. Multiple organs may be affected, predominantly the lung, the eyes and the skin. Peritoneal involvement is an infrequent site of its manifestation, especially in the absence of splenic, hepatic, adnexal, or small intestinal involvement. Patients are usually female and the disease manifests between the second and fourth decade of life. Most cases have a benign course, resolving either spontaneously or with the help of corticosteroid administration. Young patients presenting pleural effusions and ascites should always be investigated for sarcoidosis. However, diagnosis may be difficult to establish without a laparoscopic exploration and biopsies for histological examination.