Open AccessA Rare Association between Idiopathic Multicentric Castleman Disease, Nephrotic Syndrome and Polyneuropathy in an immunocompetent patient
Author(s) -
Luís Landeiro,
Aldevina Campos de Freitas,
Margarida Proença,
José Cabeçadas,
Albertiunes,
Alexandra Bayão Horta
Publication year - 2021
Publication title -
european journal of case reports in internal medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.125
H-Index - 1
ISSN - 2284-2594
DOI - 10.12890/2021_002831
Subject(s) - medicine , nephrotic syndrome , polyneuropathy , rituximab , etiology , castleman disease , medical diagnosis , disease , nephropathy , gastroenterology , pediatrics , lymphoma , dermatology , pathology , endocrinology , diabetes mellitus
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.