Abstract
RATIONALE: Pseudo-pseudo Meigs syndrome (PPMS), characterized by pleural effusion, ascites, and elevated cancer antigen-125 (CA-125) levels, is a rare complication of autoimmune diseases, particularly of systemic lupus erythematosus-systemic sclerosis (SLE-SSc) overlap syndrome. PATIENT CONCERNS: A 39-year-old female presented with progressive symptoms, including finger swelling (since 13 years), skin hardening (since 10 years), bilateral second toe ulceration (since 3 months), and abdominal distention (since 1 month). During treatment for pulmonary arterial hypertension (PAH), she developed significant ascites with elevated CA-125 levels. DIAGNOSES: Comprehensive clinical evaluation and laboratory tests confirmed SLE-SSc overlap syndrome. Subsequently, contrast-enhanced abdominal magnetic resonance imaging and multidisciplinary consultation established the diagnosis of PPMS, attributed to both active disease and possible medication effects. INTERVENTIONS: Management included optimization of immunosuppressive therapy and discontinuation of PAH medications (Sildenafil and Ambrisentan). OUTCOMES: Complete resolution of ascites was achieved, and the patient maintains stable condition under regular outpatient follow-up. LESSONS: This case emphasizes the importance of considering PPMS in autoimmune disease patients with unexplained ascites and elevated CA-125 levels, while suggesting a potential association between PAH medications and PPMS development.