A Case Report of Pseudo–Pseudo Meigs’ Syndrome

Introduction
Pseudo–pseudo Meigs’ syndrome (PPMS) is a rare clinical entity characterized by the triad of ascites, pleural effusion, and elevated serum CA125 levels in the absence of ovarian or pelvic malignancies. This syndrome is most commonly associated with systemic lupus erythematosus (SLE), though its pathophysiology remains poorly understood. The present case report describes a 24-year-old female with SLE who developed PPMS, highlighting the diagnostic challenges, clinical course, and therapeutic interventions.

Case Presentation
A 24-year-old female with a 4-year history of SLE presented to the rheumatology department with fatigue, progressive abdominal distension, and bilateral lower extremity edema. Her SLE diagnosis, established at another institution, met clinical criteria, though specific diagnostic details were unavailable. Initial treatment included prednisolone, hydroxychloroquine, and cyclophosphamide, which stabilized her condition for two years. She experienced intermittent lower extremity pain relieved by prednisolone.

Eight months prior to admission, she developed worsening abdominal distension and leg edema. Abdominal ultrasonography at a local hospital revealed ascites, managed with diuretics. Five months later, she developed shortness of breath; thoracic computed tomography (CT) confirmed bilateral pleural effusion. Diuretics provided minimal relief.

Clinical Findings and Diagnostic Workup
On admission to our hospital in March 2017, physical examination showed diminished breath sounds, ascites, and pitting edema of the legs and ankles. Vital signs were normal. Laboratory findings included leukopenia (WBC 1,340/mm³), elevated C-reactive protein (14 mg/dL), hypocomplementemia (C3: 45.1 mg/dL, C4: 4.84 mg/dL), and a positive Coombs test. Antinuclear antibodies were positive (1:320, granular pattern), but anti-dsDNA, anti-Sm, anticardiolipin antibodies, and lupus anticoagulant were negative. Proteinuria was mild (0.36 g/24h), with normal renal function. Serum albumin was 3.35 g/dL, and ferritin was 42.49 ng/mL. Notably, CA125 was markedly elevated (949 IU/mL), while other tumor markers (AFP, CEA, CA199) were normal.

Imaging studies revealed pleuropericardial effusions, massive ascites, and splenomegaly. Vaginal ultrasound showed heterogeneous endometrial echoes without masses. Endoscopy, colonoscopy, and bone marrow biopsy were unremarkable. Pleural fluid analysis revealed exudative effusion without malignant cells, acid-fast bacilli, or bacterial growth. Tuberculosis testing (TB-Igra) was negative. Endometrial curettage showed no atypia. Positron emission tomography/CT (PET/CT) demonstrated pulmonary inflammation, polyserositis, and bilateral femoral head necrosis (maximum standardized uptake value: 2.6), but no malignancy.

Diagnosis and Management
A diagnosis of PPMS associated with SLE was established after excluding malignancies, nephrotic syndrome, protein-losing enteropathy, and constrictive pericarditis. The patient received intravenous methylprednisolone (80 mg/day) and mycophenolate mofetil. Symptoms improved significantly within three months, with reduced pleural effusion and ascites on follow-up imaging.

Pathophysiology of PPMS
PPMS is a diagnostic dilemma, often mimicking malignant or infectious etiologies. In SLE, serositis is common, but massive ascites is rare. The syndrome is attributed to chronic inflammation of serosal membranes, leading to increased vascular permeability and fluid accumulation. Elevated CA125, a glycoprotein expressed by mesothelial cells, reflects peritoneal irritation rather than malignancy. Cytokine-mediated activation of mesothelial cells (e.g., IL-6, TNF-α) may drive CA125 overproduction.

Role of CA125 in PPMS
CA125 is a nonspecific biomarker elevated in various inflammatory and nonmalignant conditions. In PPMS, its rise correlates with serosal inflammation rather than ovarian pathology. Studies suggest that mechanical distension from ascites and cytokine interactions stimulate CA125 release. Among SLE patients with serositis, CA125 elevation is common, though underrecognized.

Therapeutic Considerations
PPMS responds well to immunosuppression. High-dose corticosteroids (e.g., methylprednisolone 1 g/day or prednisolone 40–80 mg/day) are first-line, often combined with agents like mycophenolate mofetil, azathioprine, or cyclophosphamide. Early intervention avoids unnecessary invasive procedures (e.g., laparoscopy) prompted by elevated CA125.

Literature Review and Comparative Analysis
A literature review identified nine prior PPMS cases, with this report being the tenth. Common features include female predominance, SLE association, exudative effusions, and CA125 elevation (range: 200–1,200 IU/mL). Most patients required moderate-to-high corticosteroids, with immunosuppressants for relapse prevention. Chronic serositis, rather than acute inflammation, characterizes PPMS, as evidenced by indolent symptom progression and absence of fever or abdominal pain.

Clinical Implications
PPMS underscores the importance of recognizing SLE-related serositis in young women with ascites and pleural effusion. Elevated CA125 should not reflexively prompt malignancy evaluations if SLE activity is evident. Timely immunosuppression reduces morbidity and avoids invasive diagnostics.

Conclusion
PPMS is a rare but treatable manifestation of SLE. Clinicians must maintain a high index of suspicion to differentiate it from malignant or infectious causes. Corticosteroids and immunosuppressants remain cornerstone therapies, with favorable outcomes observed in reported cases. Further research is needed to elucidate the molecular mechanisms linking SLE, CA125, and serosal inflammation.

doi.org/10.1097/CM9.0000000000000231

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