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.

Keywords: MGRS; MGUS; bortezomib; glomerulonephritis; multiple myeloma.

Fig. 1.

Renal biopsy findings in a 60-year-old patient with pauci-immune extracapillary-proliferative glomerulonephritis due to IgG kappa multiple myeloma. (A) Biopsy before bortezomib treatment (200×, PAS) showing a proliferating glomerular crescent. The tubulo-interstitial space appears to be normal. Polymorphonuclear leucocytes within the glomerular tuft. No infiltrates. (B) Biopsy after bortezomib treatment (200×, PAS) showing sclerosing glomeruli and sclerosing crescents, interstitial fibrosis and tubular atrophy with only scarce inflammatory infiltrates. The majority of glomeruli showed normal appearance.