INTRODUCTION: Pseudo-Pseudo-Meigs syndrome (PPMS), also known as Tjalma syndrome, is a rare manifestation of systemic lupus erythematosus (SLE). Pseudo-Pseudo-Meigs syndrome presents with a classic triad of pleural effusion, ascites, and elevated levels of protein cancer antigen 125 (CA-125). Unlike Meigs and Pseudo-Meigs syndromes, PPMS is not associated with an ovarian or abdominal/pelvic tumor, respectively. This systematic review analyzes existing cases of PPMS, highlighting trends and unique presentations of the syndrome. METHODS: PubMed, Google Scholar, Web of Science, Scopus, and Ovid Embase were searched using “Tjalma Syndrome” or “pseudo-pseudo Meigs.” Articles in languages other than English were included. Articles were excluded if they were irrelevant to PPMS or were duplicates. RESULTS: From 334 initial results, 285 were excluded, leaving 49 articles and 52 patients for final analysis. Newly diagnosed SLE was present in 34 patients (65.38%). Out of 41 patients tested for anti-dsDNA antibodies, 32 (78.05%) had positive results. Hypocomplementemia was present in 42 out of 43 tested patients (97.67%). Lupus nephritis was seen concurrently in 16 patients, or 55.17% of patients tested. With treatment, the median CA-125 level was reduced from 307 U/mL (interquartile range, 612.5 U/mL) to 22.4 U/mL (interquartile range, 23.05 U/mL). CONCLUSIONS/IMPLICATIONS: Although a rare entity, PPMS has recently seen a growing number of reported cases. Given the similarities in presentation between PPMS and Meigs or Pseudo-Meigs syndromes, it is crucial for gynecologists to consider PPMS as a differential once malignancy has been ruled out.
Acosta et al. (2026) studied this question.