Deciphering the puzzle: a case report of Tjalma syndrome (pseudo-pseudo Meigs' syndrome) with profoundly elevated CA-125 and pleural effusion

Abstract

Elevated CA-125 levels, polyserous effusions (such as pleural effusion, ascites, etc.) in young women with systemic lupus erythematosus (SLE) may signal pseudo-pseudo Meigs' syndrome (PPMS), after excluding other causes. We describe a 32-year-old SLE patient with recurrent bilateral pleural effusions and unexplained hypercalcemia for 10 months. Extensive evaluations revealed no infections or tumors. Cytokine analysis showed elevated interleukin (IL) levels, especially IL-6 in pleural effusion. Treatment with immunosuppressive therapy resulted in reduced cancer antigen (CA) 125 levels and decreased effusion volume, demonstrating a positive response to intervention in this case of PPMS.

Keywords: CA-125; Tjalma syndrome; pleural effusion; pseudo-pseudo Meigs’ syndrome; systemic lupus erythematosus.

Figure 1

Clinical and Diagnostic Progression in Patient with PPMS and SLE. (A) Computed Tomography (CT) image depicting extensive pleural effusion before treatment (indicated by red arrows) (2022-10-17). (B-C) Subsequent to the histopathological examination of the parietal pleura, the pathology report indicates the presence of lymphocytes, histiocytes, and normal mesothelial cells, with no discernible tumor cells identified. The red arrow signifies values exceeding the normal range. (D) Graph showing the progression of serum calcium concentration, with the left vertical axis indicating pleural effusion volume and the horizontal axis representing time. Ultrasonography assessed the pleural effusion volume, with the first week of patient admission as the starting point. (E) Subsequent CT image illustrating a significant reduction in pleural effusion post-treatment (2022-11-16). (F) Cytokine concentration in both serum and pleural effusion before the initiation of immunosuppressive therapy.