kidney involvement in Crow–Fukase syndrome

Abstract EN

Crow–Fukase syndrome, also known as POEMS syndrome, is a rare plasma dyscrasia characterized by monoclonal gammopathy and various combinations of polyneuropathy, organomegaly, endocrinopathy and dermatological changes, and their initials stand for the acronym POEMS.

Substantial kidney involvement is rarely related to this disease.

Our report is about five patients suffering from the POEMS syndrome with kidney involvement that rapidly progressed to end-stage renal disease.

Our report is about three females and two males with a mean age of 60.6 years.

Neuropathy was noted in all the cases.

Endocrinopathy included hypothyroidism and/or diabetes.

Skin changes were noted in one case, and included peri-orbital hyperpigmentation.

Monoclonal gammopathy was present in all the cases and was related to multiple myeloma in three cases.

Kidney involvement presented in all the five cases.

Treatment included Melphalan, Thalidomid, steroids and hemodialysis.

Survival was short for three patients, from five to 34 months.