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Diagnosis and treatment of monoclonal gammopathy of renal significance

Authors
Nelson Leung, MD
S Vincent Rajkumar, MD
Section Editor
Robert A Kyle, MD
Deputy Editor
Albert Q Lam, MD

INTRODUCTION

Monoclonal gammopathy of renal significance (MGRS) represents a group of kidney disorders caused by a monoclonal immunoglobulin that is secreted by a nonmalignant or premalignant B cell or plasma cell clone [1,2]. By definition, these disorders do not meet diagnostic criteria for overt, symptomatic multiple myeloma or a lymphoproliferative disorder. The term MGRS was proposed in 2012 by the International Kidney and Monoclonal Gammopathy Research Group to collectively describe patients who would otherwise meet the criteria for monoclonal gammopathy of undetermined significance (MGUS) but demonstrate renal insufficiency and monoclonal immunoglobulin deposition in the kidney [1]. The goal was to differentiate patients with MGUS, who have no evidence of end-organ damage, from those with MGRS, who are at risk of developing progressive renal disease. MGRS can also be associated with other hematologic disorders, including smoldering multiple myeloma [3,4], smoldering Waldenström macroglobulinemia [5], and monoclonal B cell lymphocytosis (MBL) [6].

MGRS-associated kidney diseases encompass a wide spectrum of renal pathology and include such lesions as immunoglobulin-associated amyloidosis, monoclonal immunoglobulin deposition disease (MIDD), proliferative glomerulonephritis with monoclonal immunoglobulin deposits (PGNMID), C3 glomerulopathy with monoclonal gammopathy, light chain proximal tubulopathy (Fanconi syndrome), and several others.

This topic will review the diagnosis and treatment of MGRS. The diagnosis and management of patients with MGUS and the etiology, pathogenesis, clinical manifestations, diagnosis, and treatment of kidney diseases associated with multiple myeloma and other malignant monoclonal gammopathies are presented separately. (See "Diagnosis of monoclonal gammopathy of undetermined significance" and "Clinical course and management of monoclonal gammopathy of undetermined significance" and "Epidemiology, pathogenesis, and etiology of kidney disease in multiple myeloma and other monoclonal gammopathies" and "Clinical features, evaluation, and diagnosis of kidney disease in multiple myeloma and other monoclonal gammopathies" and "Treatment and prognosis of kidney disease in multiple myeloma and other monoclonal gammopathies".)

EPIDEMIOLOGY AND PROGNOSIS

Kidney disease is a common complication of multiple myeloma and other malignant conditions with monoclonal gammopathy. However, most of the kidney diseases that are associated with multiple myeloma, with the exception of light chain cast nephropathy and acute kidney injury caused by hypercalcemia, can occur in the absence of multiple myeloma or other hematologic malignancy. These renal disorders that are caused by the deposition of monoclonal immunoglobulins or their components meet criteria for the diagnosis of MGRS. The following studies illustrate the range of findings [7-14]:

In a study of 474 patients with immunoglobulin light chain (AL) amyloidosis, only 8 percent had evidence of multiple myeloma [8].

                   

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Literature review current through: Jul 2017. | This topic last updated: Apr 18, 2017.
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