Paraprotein-Related Kidney Disease: Diagnosing and Treating Monoclonal Gammopathy of Renal Significance

Abstract

Paraprotein-related kidney disease represents a complex group of diseases caused by an abnormal paraprotein secreted by a clone of B cells. The disease manifestations range from tubulopathies, such as the Fanconi syndrome, to a spectrum of glomerular diseases that can present with varying degrees of proteinuria and renal dysfunction. Diagnosis of these diseases can be challenging because of the wide range of manifestations as well as the relatively common finding of a serum paraprotein, especially in elderly patients. Thus, renal biopsy along with detailed hematologic workup is essential to link the presence of the paraprotein to the associated renal disease. Recent advances in treatment with more effective and targeted chemotherapies, as well as stem cell transplantation, have improved the renal and overall prognosis for many of these disorders.

Keywords: Aged; B-Lymphocytes; Biopsy; Fanconi Syndrome; Humans; Kidney Diseases; Paraproteins; Prognosis; Stem Cell Transplantation; kidney; kidney disease; monoclonal gammopathy; multiple myeloma; paraproteinemia; proteinuria.

Copyright © 2016 by the American Society of Nephrology.

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Figure 1.

Diagnostic evaluation of paraprotein–related kidney disease. *Lymph node biopsy may be indicated if bone marrow aspirate/biopsy is negative and suspicion for lymphoma is high, especially if a monoclonal IgM is present.