A case of mixed connective tissue disease with pseudo-pseudo Meigs’ syndrome (PPMS)-like features

Abstract

Pseudo-pseudo Meigs' syndrome (PPMS) has been reported to be a rare presentation of patients with systemic lupus erythematosus (SLE). However, such a presentation is not common in other forms of connective tissue disease. We presented a case of gross ascites, pleural effusion, and marked elevation of CA-125 level (PPMS-like features) that led to a diagnosis of MCTD. The patient responded to systemic steroid therapy.

Systemic lupus erythematosus presenting with progressive massive ascites and CA-125 elevation indicating Tjalma syndrome? A case report

“…Therefore, lupus peritonitis, nephrotic syndrome, constrictive pericarditis and Budd–Chiari syndrome were ruled out. We postulated that hypoproteinemia was due to protein-losing enteropathy, resulting in intestinal damage caused by SLE (diarrhea, bowel wall edema and mesenteric vasculitis), consistent with previous studies[ 6 , 7 ]. However, 99m-labeled human serum albumin is required for definite diagnosis[ 8 ], which is not available at our hospital.…”

Pseudo-pseudo Meigs’ syndrome in Rheumatology

“…Peritoneal and pleural fluids of the patients originally with MS were transudates [1]. In pseudo-MS similar features are due to other benign or malignant tumors; Pseudo Pseudo-MS (PPMS) occurs in Systemic Lupus Erythematosus (SLE) alone or SLE plus scleroderma [2][3][4][5][6]. The term PPMS was first utilized in 2005 by Schmitt et al, in the case report of a 33-year-old woman with the classical characteristics of the syndrome and enlarged cystic ovaries [2].…”

“…The differential diagnosis of PPMS constitutes a challenging task mainly for non-specialists. A major concern is about the interpretation of elevated levels of CA 125 described in patients with PPMS [2][3][4][5][6], because this tumor marker is considered indicative of ovarian malignancy. High levels of CA125 may be also due to pelvic tuberculosis and nephrotic syndrome [3,4].…”

“…PPMS is an exceeding uncommon condition diagnosed in rheumatologic patients, and the scarcely reported cases were associated with SLE, or SLE and scleroderma (MCTD) [2][3][4][5][6].…”

“…As benign and malignant ovarian tumors are more frequent than PPMS, one must rule out the typical MS and the PMS caused by ovarian malignancy that are more prevalent entities [1][2][3][4][5][6].…”