title: Monoclonal gammopathy-associated pauci-immune extracapillary-proliferative glomerulonephritis successfully treated with bortezomib.
abstract: Extracapillary-proliferative glomerulonephritis is a rare complication of multiple myeloma. Partial remission of kidney involvement with cyclophosphamide therapy has previously been described. We report the case of a 60-year-old male patient diagnosed with rapidly progressive glomerulonephritis associated with IgG kappa monoclonal gammopathy. His kidney biopsy revealed pauci-immune extracapillary-proliferative glomerulonephritis without cryoglobulinaemia. Treatment with the proteasome inhibitor bortezomib induced rapid clinical and histological remission of his kidney disease. The patient's renal function remained stable on bortezomib maintenance therapy. Our findings suggest that bortezomib is a promising therapeutic approach to ameliorate severe kidney damage in monoclonal gammopathy- and myeloma-associated pauci-immune extracapillary-proliferative glomerulonephritis.
authors.affiliation: Department II of Internal Medicine , University of Cologne , Cologne , Germany.
authors.forename: Franziska
authors.initials: F
authors.lastname: Grundmann
authors.forename: Marco
authors.initials: M
authors.lastname: Witthus
authors.forename: Heike
authors.initials: H
authors.lastname: Göbel
authors.forename: Tuelay
authors.initials: T
authors.lastname: Kisner
authors.forename: Rainer
authors.initials: R
authors.lastname: Siewert
authors.forename: Thomas
authors.initials: T
authors.lastname: Benzing
authors.forename: Christine E
authors.initials: CE
authors.lastname: Kurschat
keywords: MGRS; MGUS; bortezomib; glomerulonephritis; multiple myeloma