A Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient

Abstract

Multicentric Castleman disease (MCD) represents a group of poorly understood lymphoproliferative disorders related to proinflammatory hypercytokinaemia. In immunocompetent patients its etiology is still unknown, hence the designation of idiopathic multicentric Castleman disease (iMCD). To successfully diagnose iMCD, diagnostic criteria must be fulfilled and a large array of alternative diagnosis excluded. Peripheral neuropathy and nephropathy are relatively common findings in cases associated with POEMS syndrome, but very rarely reported in iMCD. We present the case of a 64-year-old man with iMCD (HIV and HHV-8 negative) with nephrotic syndrome and severe motor polyneuropathy. Alternative diagnoses were excluded. The patient was treated with intravenous glucocorticoid followed by rituximab. Complete clinical and laboratorial remission was achieved and maintained at 2-year follow-up.

Keywords

• multicentric castleman
• nephrotic syndrome
• polyneuropathy