Clinical Images: Pseudo‐pseudo Meigs syndrome
Pseudo-pseudo Meigs syndrome (PPMS), also called Tjalma syndrome is a relative rare presentation of systemic lupus erythematosus (SLE). The PPMS consists of triad with ascites, pleural effusion, and elevated cancer antigen-125 (CA-125) but without any presence of either benign or malignant ovarian tumor in the pelvis.1, 2 Only about sporadic 30 cases of PPMS have ever been reported in the past 20 years (from 2005 to 2025).3-5 PPMS always poses a challenge in differential diagnosis because its clinical presentation seems very similar to that of a malignancy, yet it is a consequence of SLE itself. The patient, a 61-year-old woman, presented with a three-month history of dyspnea and abdominal distension. (A) A chest radiograph revealed massive right-sided pleural effusion, whereas abdominal computed tomography showed ascites without identifiable lesions. Pleural effusion results were exudative and negative for malignant cell or infectious pathogens. The serum CA-125 level was 363 U/mL (reference: <35.0). (B and C) Positron emission tomography (PET) revealed significant pleural effusion (asterisk) and a lot of ascites (arrows) but did not identify any other fluorodeoxyglucose-avid lesions. Diagnostic laparoscopy results revealed omental nodules consistent with chronic inflammation, and thoracoscopy results confirmed chronic fibrous pleuritis. Laboratory testing results showed anti−nuclear antibodies (1:160), anti-double-stranded DNA antibody (105 U/mL), hypocomplementemia (C3: 28.8 mg/dL; C4: 5.1 mg/dL), proteinuria (0.391 g/day), and leukopenia (WBC: 2810/μL). A diagnosis of PPMS as initial presentation of SLE was made.1, 2 Treatments with systemic steroid and immunosuppressant significantly alleviated refractory massive ascites and pleural effusion. This case is distinguished by the comprehensive exclusion of malignancy through PET, laparoscopy, and thoracoscopy, confirming the absence of tumors. Histopathology revealed chronic inflammation consistent with autoimmune serositis. PPMS presented as the initial manifestation of SLE, with markedly elevated CA-125 and prompt improvement after immunosuppressive therapy. Disclosure form. Please note: The publisher is not responsible for the content or functionality of any supporting information supplied by the authors. Any queries (other than missing content) should be directed to the corresponding author for the article.
Authors
- Hsien‐Tzung Liao (ORCID: https://orcid.org/0000-0001-5930-4840)
Institutions
- Taipei Veterans General Hospital (TW)
Publication Details
- Journal
- ACR Open Rheumatology
- Published
- 2025-12-01
- DOI
- https://doi.org/10.1002/acr2.70151
- Primary Topic
- Ovarian cancer diagnosis and treatment
- Type
- article
- Field-Weighted Citation Impact
- 0.00