Massive Ascites in the Absence of Liver Disease: A Rare Case of Pseudo-pseudo Meigsʼ Syndrome

Abstract

Pseudo-pseudo Meigs' syndrome (PPMS) is an extremely rare phenomenon seen in patients with Systemic Lupus Erythematosus (SLE), described by the presence of large volume exudative ascites, pleural effusions, and an elevated CA-125. In the below case, we depict a young patient who presented with massive abdominal ascites, pleural effusions, significant lymphadenopathy, and hypoalbuminemia. Although rare and of uncertain etiology, PPMS is becoming more recognized in the literature, and earlier detection is crucial in avoiding an extensive and costly workup. Patient is a 23-year-old female, who presented to the emergency department for 8 months of progressively worsening abdominal distention and pain. Patient underwent extensive workup including CT abdomen/pelvis, which showed massive abdominal/pelvic ascites, and enlarged inguinal lymph nodes without signs of cirrhosis or splenomegaly. Subsequent paracentesis removed 10 liters of clear fluid with serum ascites albumin gradient of 0.2. No malignant cells were identified. Hepatology was consulted and concluded no evidence of liver abnormality. Additional lab work ruled out cardiac or renal causes, but did show elevated CA-125. Bone marrow biopsy showed no evidence of myelodysplasia. PET scan showed marked lymphadenopathy of the head, neck, thorax, and pelvis, but ultrasound guided excisional lymph node biopsies revealed benign lymphoid hyperplasia. Quantiferon gold testing for tuberculosis was negative. On the brink of exploratory laparotomy, the patient was tested for SLE. She had multiple positive rheumatologic tests consistent with lupus and was formally diagnosed with SLE by rheumatology. Her condition has been stable on prednisone and hydroxychloroquine. PPMS is an extremely rare manifestation of SLE that should be on the differential for patients who present with ascites of unknown etiology. Other SLE related gastrointestinal manifestations are clinically important as well and include intestinal pseudo-obstruction, mesenteric vasculitis, protein losing enteropathy, and acute pancreatitis. Unfortunately, SLE is often overlooked in these patients. If diagnosed in a timely manner, further extensive and costly workup, including surgery may be avoided. The general consensus among experts is to treat the underlying SLE. Prognosis based on the few cases that have been published is good, as patients usually respond to corticosteroids, and in more refractory cases, immunomodulators or immune-suppressors.Figure: Massive Abdominal Ascites.