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Journal of Shanghai Jiaotong University(Medical Science) ; (12): 1710-1714, 2017.
Article in Chinese | WPRIM | ID: wpr-665311

ABSTRACT

A 24-year-old male suffered from acute nephritic syndrome, liver dysfunction, and mesenteric mass. Laboratory examination showed a variety of autoantibodies (ANA, SM, and A-β2-GP1) were positive. The biopsies of the kidney and the mesenteric mass were performed. The diagnosis was typeⅤ + Ⅲ lupus nephritis accompanied with Castleman's disease. Then the patient was given induction therapy of glucocorticoids and cyclophosphamide for the first 3 months, followed by rituximab as maintenance therapy. The patient was followed up after 0, 3, and 9 months. After 3-month treatment, lupus nephritis was partially remitted, and systemic lupus erythematosus disease activity index (SLEDAI) decreased to 4 scores in an inactivity phase from 20 scores in a serious activity phase at baseline. Nine months later, lupus nephritis was completely remitted and 50% mesenteric mass was regressed through CT scanning. Lupus nephritis can accompany with multicentric Castleman's disease. Due to lack of clinical specificity and effective therapy, patients may have a high misdiagnosis rate and poor prognosis. The most reliable way to establish a definitive diagnosis relays on histopathologic confirmation. The management of induction therapy of glucocorticoids and cyclophosphamide, followed maintenance therapy of rituximab may become a beneficial treatment.

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