Pleural effusion with splenic rupture as manifestations of recurrence of sarcoidosis following prolonged remission

A 55-year-old man presented with a 3-week history of dry cough and left pleuritic chest pain with a new exudative pleural effusion. Sixteen years earlier, he was diagnosed with sarcoidosis presenting with hilar lymphadenopathy, erythema nodosum, mildly disturbed liver function tests and noncaseating granulomata on liver biopsy, with no evidence of pulmonary parenchymal disease. He was treated with prednisone and in recent years maintained at a low daily dose, until it was eventually discontinued two years prior to his present illness. There was no evidence of infection or malignancy, and the fluid resolved following treatment with naproxen. Three weeks later the patient presented with sudden onset of dyspnea and left chest pain. After starting intravenous heparin for suspected pulmonary emboli, the patient developed hemodynamic instability which was accompanied by abdominal tenderness and decreasing hematocrit. Splenic rupture was diagnosed, and the patient underwent splenectomy. Pathology specimens revealed a hemorrhagic infarct with subcapsular hematoma, and numerous noncaseating granulomata within the splenic tissue. This patient had recurrent sarcoidosis with two rare manifestations of the disease, 2 years after withdrawing low dose prednisone, given for a prolonged time. The possibility of reemergence of the disease in organs other than the organs involved in the initial presentation should always be considered in sarcoidosis.