Tjalma syndrome is a rare manifestation of systemic lupus erythematosus (SLE) characterized by pleural effusion, ascites, and elevated cancer antigen 125 (CA-125) levels in the absence of ovarian malignancy. We report the case of a woman in her 50s who presented with recurrent pleuritic chest pain, dyspnea, peripheral edema, ascites, and constitutional symptoms. Initial investigations were inconclusive, resulting in repeated admissions and multidisciplinary referrals. Subsequent immunological testing confirmed SLE. Given the constellation of serositis and elevated CA-125, a diagnosis of Tjalma syndrome was established. Treatment with immunosuppressants such as corticosteroids, hydroxychloroquine, and azathioprine resulted in symptomatic improvement. However, the disease course was complicated by constrictive pericarditis requiring pericardiectomy and later inflammatory arthritis requiring escalation of immunosuppression. This case highlights the importance of considering autoimmune etiologies in patients with unexplained multisystem effusions and elevated tumor markers, thereby avoiding misdiagnosis and unnecessary oncological interventions.
Kodamanchile et al. (2026) studied this question.