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Diagnosis: Proliferative Glomerulonephritis with Monoclonal IgG deposists (IgG3λ)
Glomerular diseases in patients with monoclonal Ig can be classified in two categories by electron microscopy (EM). The first category includes diseases with fibril formation, mainly amyloidosis, and diseases with microtubule formation, including cryoglobulinemic and immunotactoid GN. The second category is characterized by nonorganized electron-dense granular deposits. They are localized along basement membranes in most tissues, especially in the kidney, and define a disease now termed “monoclonal Ig deposition disease”. In addition, Nasr et al. have described a novel form of glomerular disease, termed “proliferative glomerulonephritis with monoclonal IgG deposits,” in which monoclonal Ig deposits are associated with glomerular proliferative lesions, mimicking different patterns of immune-complex GN, such as membranoproliferative or endocapillary proliferative GN (Guiard E, et al. Patterns of noncryoglobulinemic glomerulonephritis with monoclonal Ig deposits: correlation with IgG subclass and response to rituximab. Clin J Am Soc Nephrol. 2011;6(7):1609-16. [PubMed link] [Free full text]).
Several reports have underlined the fact that monoclonal entire IgG and IgM can cause nephrotic or nephritic syndromes and lead to renal failure in patients presenting with plasma cell dyscrasia or lymphroproliferative disease or without overt malignancy. Monoclonal proliferative GN can also be observed in patients with no detectable cryoglobulinemia and no hypocomplementemia (Guiard E, et al. Patterns of noncryoglobulinemic glomerulonephritis with monoclonal Ig deposits: correlation with IgG subclass and response to rituximab. Clin J Am Soc Nephrol. 2011;6(7):1609-16. [PubMed link] [Free full text]).
A week after renal biopsy, hematologic studies demonstrated multiple myeloma in the patient; then, the GN was related to monoclonal complete IgG produced by the neoplastic cells.
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References
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Sumida K, Ubara Y, Marui Y, Nakamura M, Takaichi K, Tomikawa S, Fujii T, Ohashi K.Recurrent Proliferative Glomerulonephritis With Monoclonal IgG Deposits of IgG2λ Subtype in a Transplanted Kidney: A Case Report. Am J Kidney Dis. 2013;62(3):587-90. [PubMed link]
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Guiard E, Karras A, Plaisier E, Duong Van Huyen JP, Fakhouri F, Rougier JP, Noel LH, Callard P, Delahousse M, Ronco P. Patterns of noncryoglobulinemic glomerulonephritis with monoclonal Ig deposits: correlation with IgG subclass and response to rituximab. Clin J Am Soc Nephrol. 2011;6(7):1609-16. [PubMed link] [Free full text]
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Nasr SH, Sethi S, Cornell LD, Fidler ME, Boelkins M, Fervenza FC, Cosio FG, D'Agati VD. Proliferative glomerulonephritis with monoclonal IgG deposits recurs in the allograft. Clin J Am Soc Nephrol. 2011;6(1):122-32. [PubMed link] [Free full text]
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Nasr SH, Satoskar A, Markowitz GS, Valeri AM, Appel GB, Stokes MB, Nadasdy T, D'Agati VD. Proliferative glomerulonephritis with monoclonal IgG deposits. J Am Soc Nephrol. 2009;20(9):2055-64. [PubMed link] [Free full text]
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Nasr SH, Markowitz GS, Stokes MB, Seshan SV, Valderrama E, Appel GB, Aucouturier P, D’Agati VD. Proliferative glomerulonephritis with monoclonal IgG deposits: A distinct entity mimicking immune-complex glomerulonephritis. Kidney Int. 2004;65:85-96, 2004 [PubMed link]