Introduction: Pseudo-pseudo Meigs syndrome (PPMS) is a rare presentation of systemic lupus erythematous (SLE) characterized by ascites, pleural effusion, and elevated CA-125 level.
Case Description/Methods: A 30-year-old woman with SLE and history of small bowel obstruction presented with diarrhea, weight loss, anasarca, and alopecia. She was initially tachycardic and hypertensive. Her exam revealed profound anasarca with ascites and bilateral pleural effusions. Labs were notable for Cr=5.65 mg/dL (baseline 0.7 mg/dL), spot protein/creatinine=3.9, ferritin=554.6 ng/ml, ESR=145 mm/Hr, CRP=6 mm/Hr, C3=49 mg/dL, C4=19 mg/dL, unremarkable FSH and LH, +ANA, dsDNA 356 U/ml, and +pANCA/MPO. Outpatient workup showed elevated CA-125 to 260.8 U/mL but ovarian malignancy was ruled out by MRI and CT abdomen/pelvis. Transvaginal ultrasound was normal. Inflammatory bowel disease was ruled out by EGD and colonoscopy during a previous hospitalization. Renal ultrasound showed bilateral hydronephrosis. The patient underwent a paracentesis (SAAG 1.3), and thoracentesis that showed total serum protein 4.2 g/dl, pleural fluid LDH 67 U/L, no serum LDH or pleural protein available. Kidney biopsy demonstrated class IV / V lupus nephritis. She was treated with a three-day course of pulse dose methylprednisolone followed by prednisone, hydroxychloroquine, and furosemide infusion. Her symptoms mostly resolved and CA-125 trended down to 27 U/mL.
Discussion: PPMS is a rare condition with few cases reported in the literature. This case illustrates the challenges of evaluating abdominal symptoms and ascites in patients with SLE, and the importance of a kidney biopsy in the evaluation of nephrotic range proteinuria. Due to infrequency and concern for malignancy due to elevated CA-125 level, patients with this syndrome undergo extensive workup. CA-125 has been shown to be elevated due to inflammatory markers enhancing its expression in peritoneal mesothelial cells. Prior publications have shown the correlation of CA-125 with extent of serositis in PPMS. Additionally, our patient has many other clinical features reported in PPMS patients including elevated ferritin, alopecia, and positive pANCA. CT scan of abdomen and pelvis is essential in the workup of this syndrome to rule out ovarian malignancy. Moreover, imaging may exclude mesenteric vasculitis, an uncommon feature of SLE which can be seen as targetoid lesions of the bowel on CT scan. Steroids are the treatment for PPMS.
