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.
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.
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.
Management included optimization of immunosuppressive therapy and discontinuation of PAH medications (Sildenafil and Ambrisentan).
Complete resolution of ascites was achieved, and the patient maintains stable condition under regular outpatient follow-up.
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.