Meigs' syndrome: A rare cause of recurrent pleural effusion in scleroderma

Meigs' syndrome represents a triad of pleural effusion, ascites, and an ovarian tumor, usually benign, occurring together. We describe here a case of Meigs' syndrome in a patient with systemic sclerosis, the first such report to our knowledge, in systemic sclerosis. A 53-year-old woman with systemic sclerosis presented with recurrent right-sided pleural effusion, which led to symptoms of shortness of breath, chest tightness, and a non-productive cough. Physical examination revealed a palpable, mobile mass in the right lower quadrant, in addition to typical physical features of scleroderma. Thoracentesis yielded exudative pleural fluid with cytology negative for malignancy. Pleural biopsy was consistent with inflammatory changes, but negative for malignancy. CT scan of the chest, abdomen, and pelvis revealed a soft tissue mass in the pelvis, which appeared to arise from the left ovary. The patient's cancer antigen 125 (CA-125) level was elevated at 222 U/mL (normal range, 0-30 U/mL). The patient underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Histology of the left ovarian mass was consistent with an ovarian fibrothecoma, a benign tumor of the ovary. At her 1-month follow-up appointment, the patient had complete resolution of the right-sided pleural effusion. To date, at 10 months past the initial presentation, she has not had recurrence of pleural effusion. Although rare, Meigs' syndrome should be considered as a possible cause of recurrent serositis in women with rheumatologic diseases. Removal of the ovarian tumor leads to prompt resolution of the serositis.