Recurrent Nephrotic Syndrome Associated with Kimura’s Disease in a Young Non-Oriental Male

Abstract

Kimura’s disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Renal disease is rarely associated with it. We describe a young non-Oriental male with KD and relapsing steroid-responsive minimal-change nephrotic syndrome. This case of KD associated nephrotic syndrome is unique in the relapsing nature of the nephropathy and the non-Oriental origin of the patient. We comment on KD and nephrotic syndrome and on treatment strategy.

Kimura’s Disease: A Diagnostic Challenge

“…Proteinuria may occur in 12% to 16% of cases. 13 Nephrotic syndrome is the most common presentation 13 ; a wide spectrum of histologic lesions such as minimal change disease or mesangioproliferative glomerulonephritis, focal segmental glomerulosclerosis, membranous nephropathy, IgM nephropathy, and IgA nephropathy have been described. 5 Our patient has normal renal function and no evidence of proteinuria.…”

Recurrent Kimura's disease successfully treated with steroids and cetirizine

“…Nephrotic syndrome is the most common presentation. 4 The etiology and pathogenesis are yet unknown. It is believed to be an allergic or autoimmune response to an unknown stimulus which results in the release of cytokines.…”

Kimura’s Disease: Report of a Case & Review of Literature

“…It is a benign condition with self-limiting course. Renal involvement in the disease characterized by proteinuria (12-16 %) may be seen [2].…”