Jixiang Xun, Xiaohan Liu, Bohan Chang, Xue Wang, Cheng Peng, Pingting Yang, Xudong Liu
Primary Sjögren's syndrome may be complicated by PPMS, with PPMS as the initial clinical manifestation. Early multidisciplinary collaboration is crucial to avoid misdiagnosis, and the corticosteroid combined with immunosuppressant treatment has been proved that it was associated with clinical improvement.
OBJECTIVE: To explore the clinical diagnostic methods and therapeutic outcomes for rheumatologic and immunologic disorders characterized by "polypleural effusion with elevated CA12-5", aiming to avoid misdiagnosis and mistreatment due to similar symptoms.
METHODS: A retrospective analysis was conducted on the clinical data of a 56-year-old female patient. Multidisciplinary consultation (gynecology, thoracic medicine, rheumatology and immunology), imaging studies, laboratory tests, and pathological biopsy results were combined to establish a definitive diagnosis. After diagnosis, the patient received combined treatment with corticosteroids and immunosuppressants, with dynamic evaluation of clinical efficacy.
RESULTS: Systematic examinations ruled out gynecological tumors, tuberculosis, and other diseases. Based on abnormal rheumatologic and immunologic markers, organ involvement, and treatment response, the patient was diagnosed with primary Sjögren's syndrome, pseudo-pseudo Meigs' syndrome (PPMS,Tjalma syndrome), interstitial pneumonia and so on. After treatment with methylprednisolone combined with hydroxychloroquine and mycophenolate mofetil, CA12-5 levels significantly decreased, polypleural effusion gradually resolved, and the condition remained stable over the long term.
CONCLUSION: Primary Sjögren's syndrome may be complicated by PPMS, with PPMS as the initial clinical manifestation. Early multidisciplinary collaboration is crucial to avoid misdiagnosis, and the corticosteroid combined with immunosuppressant treatment has been proved that it was associated with clinical improvement.