Lupus nephritis accompanied with Castleman's disease: a case report and literature review / 上海交通大学学报（医学版）

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.