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Tjalma Syndrome A Rare Manifestation of Systemic Lupus Erythematosus

Updated: Apr 19, 2023

Systemic Lupus Erythematosus

Tjalma syndrome, also known as pseudo-pseudo Meigs' syndrome, is a rare manifestation of conditions in patients with systemic lupus erythematosus (SLE). The syndrome is characterized by the presence of ascites, pleural effusion, and an elevated cancer antigen-125 (CA-125) level. The triad of an ovarian tumor, ascites, and pleural effusion is known as Meigs' syndrome. When the ascites and pleural effusion are associated with tumors other than ovarian, it is referred to as pseudo-Meigs' syndrome. Tjalma described Tjalma syndrome, or pseudo-pseudo Meigs' syndrome (PPMS), as a rare clinical condition in systemic lupus erythematosus (SLE) patients, characterized by ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) levels in individuals without any associated malignant or benign tumors. Physicians need to be aware that the presence of elevated cancer markers such as CA-125, with ascites and pleural effusion, does not necessarily equate to a cancer diagnosis, and to thoroughly investigate alternate causes such as Tjalma syndrome. This may prevent unnecessary anxiety and surgical interventions, as Tjalma syndrome is treatable with immunosuppressant medications. The pathogenesis of Tjalma syndrome is not clear, but it is hypothesized that uncontrolled severe inflammation may be one of the causes through lympho-aggregation, and an immune complex deposition on the peritoneal membrane generating a local inflammatory reaction or a vasculitic phenomenon involving the peritoneal vessels. Massive ascites are an uncommon initial presentation of SLE and have rarely been reported in the literature. Usually, ascites in such patients are a resulting consequence of active disease, nephrotic syndrome, protein-losing enteropathy, and constrictive pericarditis. CA-125 is a glycoprotein that reacts with epithelial tumor cells of ovarian cancer; however, it has been detected in other mesothelial cells, such as the peritoneum, pleura, pericardium, epithelium of fallopian tubes, lungs, breast, prostate, and conjunctiva, in addition to various clinical conditions such as early pregnancy, ascites, menstruation, nephrotic syndrome, endometriosis, leiomyoma, congestive heart failure, cirrhosis, rheumatoid arthritis, tuberculosis, and SLE. Elevation of CA-125 in SLE may be related to activation of cytokines such as interleukin 1 and interferon γ, which increases the expression of CA-125 in human peritoneal mesothelial cells. Reference Indian Journal of Rheumatology (2022) Tjalma Syndrome: A Rare Manifestation of Systemic Lupus Eryt... : Indian Journal of Rheumatology (

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