Description Tjalma Syndrome (Pseudo-Pseudo Meigs Syndrome) is a rare clinical condition that can occur in patients with systemic lupus erythematosus (SLE). This syndrome is characterized by massive ascites, pleural effusions and increased CA-125 levels without related to benign or malignant tumors. Tjalma Syndrome is a rare condition reported in the literature and currently there are only 14 reports of this condition.

A 31-year-old woman had a major complaint of massive ascites for 3 months. She had fatigue, hair loss, joints pain, shortness of breath and lower legs edema. Her laboratory revealed positive ANA 1: 10.000, speckled patterns, lymphopenia, proteinuria with urine protein creatinine ratio of 1.773 mg/gr, and she fulfilled the SLE classification criteria based on ACR/EULAR 2019 with mucocutaneous, musculoskeletal, renal involvement and serositis. Analysis of the ascites fluid and pleural effusion showed the exudate and negative results for tuberculosis nor malignant cells. Her CA-125 was markly increased, with no benign or malignant tumors found on either imaging or anatomic pathology examination. Based on these findings, the patient was diagnosed as SLE and Tjalma Syndrome. She was given methylprednisolone pulse dose 500 mg for 3 consecutive days, followed by oral methylprednisolone 0.8 mg/kg daily, mycophenolic acid 720 mg twice daily, hydroxychloroquine 200 mg/d, ramipril 5 mg/d, furosemide 40 mg/d, calcium carbonate 500 mg twice/d and therapeutic ascites punctuation. Patient was discharge in better condition.

Conclusions Tjalma Syndrome is a rare clinical condition, but can be revealed as main presentation of SLE. This case report shows that Tjalma Syndrome could be one of the differential diagnosis of patients with ascites, pleural effusions, and increased CA-125 levels in SLE patients. Possible malignancy should be ruled out, and Tjalma syndrome should be considered so that the patient with this condition can get the proper management and avoid unnecessary surgical examination.