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The IKMG met in April 2017 to refine the definition of MGRS and to update the diagnostic criteria for MGRS-related diseases. Accordingly, in this Expert Consensus Document, the IKMG redefines MGRS as a clonal proliferative disorder that produces a nephrotoxic monoclonal immunoglobulin and does not meet previously defined haematological criteria for treatment of a specific malignancy. The diagnosis of MGRS-related disease is established by kidney biopsy and immunofluorescence studies to identify the monotypic immunoglobulin deposits (although these deposits are minimal in patients with either C3 glomerulopathy or thrombotic microangiopathy). Accordingly, the IKMG recommends a kidney biopsy in patients suspected of having MGRS to maximize the chance of correct diagnosis. Serum and urine protein electrophoresis and immunofixation, as well as analyses of serum free light chains, should also be performed to identify the monoclonal immunoglobulin, which helps to establish the diagnosis of MGRS and might also be useful for assessing responses to treatment. Finally, bone marrow aspiration and biopsy should be conducted to identify the lymphoproliferative clone. Flow cytometry can be helpful in identifying small clones. Additional genetic tests and fluorescent in situ hybridization studies are helpful for clonal identification and for generating treatment recommendations. Treatment of MGRS was not addressed at the 2017 IKMG meeting; consequently, this Expert Consensus Document does not include any recommendations for the treatment of patients with MGRS.<\/jats:p>","DOI":"10.1038\/s41581-018-0077-4","type":"journal-article","created":{"date-parts":[[2018,12,3]],"date-time":"2018-12-03T18:31:23Z","timestamp":1543861883000},"page":"45-59","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":522,"title":["The evaluation of monoclonal gammopathy of renal significance: a consensus report of the International Kidney and Monoclonal Gammopathy Research 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F.B. declares that he has received honoraria from Celgene and Janssen. P.C. declares that he is a member of the advisory board of The Binding Site, receives research support from The Binding Site and has received honoraria from Janssen. A.D. declares that she has received research grants from Alnylam, Celgene, GlaxoSmithKline, Janssen, Pfizer, Prothena and Takeda. F.C.F. declares that he has received an unrestricted grant from Genentech and Janssen and is a member of the advisory board for Alnylam. J.D.G. declares that he is a member of the advisory boards for Alnylam, Eidos and GlaxoSmithKline. V.K. declares that he has received honoraria from Amgen, Celgene and Takeda. H.J.L. declares that she has received research funding from Amgen and Takeda and is a member of the speaker\u2019s bureau for Amgen, Bristol-Myers Squibb, Celgene, Janssen and Takeda. H.L. declares that he has received research support from Amgen and Takeda and is a member of the speaker\u2019s bureau for Amgen, Bristol-Myers Squibb, Celgene, Janssen and Takeda and consults for PharmaMar. G.M. declares that he has received honoraria from Janssen, Pfizer and Prothena. P.M. declares that he is a member of the advisory boards of Amgen, Celgene and Janssen and has received clinical trial support from Janssen. S.H.N declares that he has received honoraria from Alnylam. C.P.V. declares that he has received honoraria from Amgen, Celgene, Janssen, Prothena and Takeda. P.M.V. declares that he is a member of the advisory boards of Bristol-Myers Squibb, Celgene, Janssen, Oncopeptides and TeneoBio and is a member of the speaker\u2019s bureau for Amgen and Janssen and consults for Celgene, Novartis, Oncopeptides and TeneoBio. B.M.W. declares that in October 2017 he became an employee of Janssen. 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