Successful treatment with bortezomib and dexamethasone for proliferative
glomerulonephritis with monoclonal IgG deposits in multiple myeloma: a case
report
#MMPMID28385149
Noto R
; Kamiura N
; Ono Y
; Tabata S
; Hara S
; Yokoi H
; Yoshimoto A
; Yanagita M
BMC Nephrol
2017[Apr]; 18
(1
): 127
PMID28385149
show ga
BACKGROUND: Proliferative glomerulonephritis with monoclonal IgG deposits
(PGNMID) is a form of renal involvement by monoclonal IgG deposits that was found
in mesangial, subendothelial or subepithelial regions. The distribution of
glomerular deposits was completely different from that in monoclonal
immunoglobulin deposition disease. PGNMID is reported to be rarely associated
with a hematological malignancy. Previously, only five cases of PGNMID with
multiple myeloma have been reported. However, the pathogenic relationship between
PGNMID and multiple myeloma was unclear because a detailed description was not
provided. We report that a patient with PGNMID associated with multiple myeloma
was treated with bortezomib and dexamethasone and underwent the second renal
biopsy after treatment, showing that chemotherapy was effective for PGNMID
clinically and pathologically. CASE PRESENTATION: A 75-year-old man presented
with progressive leg edema, had nephrotic range proteinuria, hypoalbuminemia,
moderate renal failure, and occult blood in his urine. Electrophoresis results
showed serum and urinary monoclonal spikes of IgG? type immunoglobulin. A renal
biopsy specimen showed lobular mesangial proliferation with mesangiolysis,
glomerular micro-aneurysm, and endocapillary hypercellularity. Immunofluorescence
results revealed strong granular capillary and mesangial staining for IgG1, C3
and ? light chain in glomeruli without tubular deposits of any immunoglobulin.
Electron microscopy also showed dense granular deposits in subendothelial and
mesangial areas. PGNMID associated with multiple myeloma (IgG? type) was
diagnosed on the basis of a subsequent bone marrow examination. Bortezomib and
dexamethasone therapy significantly reduced proteinuria and elevated serum
albumin level. Eight months later, the second renal biopsy showed no active
lesions and that the IgG1 and ? light chain deposits had drastically disappeared.
CONCLUSIONS: This is the first case of PGNMID with multiple myeloma successfully
treated with bortezomib and dexamethasone in which comparative renal biopsies
were performed before and after treatment. Our findings suggest the pathogenesis
of PGNMID and therapeutic options for PGNMID.