Abstract: [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 type
Ⅴ + 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.

Key words: Castleman’s disease, lupus nephritis, autoimmune disease, rituximab