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

Institutions

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
Controls
|||
ALL TIME
JAN
FEB
MAR
APR
MAY
JUN
JUL
AUG
SEP
OCT
article

Clinical Images: Pseudo‐pseudo Meigs syndrome

Hsien‐Tzung Liao
ACR Open Rheumatology
Ovarian cancer diagnosis and treatment
article

Clinical Images: Pseudo‐pseudo Meigs syndrome

Hsien‐Tzung Liao
article en

Abstract

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.

ACR Open RheumatologyVol. 7(12)
Taipei Veterans General Hospital (TW)
Openalex Percentile: Top 48%
Ovarian cancer diagnosis and treatment
AI Navigator

Ask Laika to Summarize, Analyze, and Connect papers live on the map.

Summarize Papers & Methodologies

Extract key findings, datasets, and comparative methods across publications.

Benchmark Rankings & Visual Analytics

Rank top research institutions, authors, funders, topics, and journals by Field-Weighted Citation Impact (FWCI) and paper volume with instant charts.

Connect Distant Disciplines

Bridge topological clusters on the map to find hidden collaborative intersections.