Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management

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Abstract

Randall-type monoclonal immunoglobulin deposition disease (MIDD) is a rare disease that belongs to the spectrum of monoclonal gammopathy of renal significance (MGRS). Renal involvement is prominent in MIDD, but extra-renal manifestations can be present and may affect global prognosis. Recent data highlighted the central role of molecular characteristics of nephrotoxic monoclonal immunoglobulins in the pathophysiology of MIDD, and the importance of serum free light chain monitoring in the diagnosis and follow-up disease. Clone-targeted therapy is required to improve the overall and renal survival, and the achievement of a rapid and deep hematological response is the goal of therapy. This review will focus on the recent progress in the pathogenesis and management of this rare disease.

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Paul E. Stevens (2013)

The Kidney Disease: Improving Global Outcomes (KDIGO) organization developed clinical practice guidelines in 2012 to provide guidance on the evaluation, management, and treatment of chronic kidney disease (CKD) in adults and children who are not receiving renal replacement therapy. The KDIGO CKD Guideline Development Work Group defined the scope of the guideline, gathered evidence, determined topics for systematic review, and graded the quality of evidence that had been summarized by an evidence review team. Searches of the English-language literature were conducted through November 2012. Final modification of the guidelines was informed by the KDIGO Board of Directors and a public review process involving registered stakeholders. The full guideline included 110 recommendations. This synopsis focuses on 10 key recommendations pertinent to definition, classification, monitoring, and management of CKD in adults.

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Henk Lokhorst, Torben Plesner, Jacob Laubach … (2015)

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New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes.

M A Gertz, Shaji Kumar, Ute Hegenbart … (2012)

To identify the criteria for hematologic and cardiac response to treatment in immunoglobulin light chain (AL) amyloidosis based on survival analysis of a large patient population. We gathered for analysis 816 patients with AL amyloidosis from seven referral centers in the European Union and the United States. A different cohort of 374 patients prospectively evaluated at the Pavia Amyloidosis Research and Treatment Center was used for validation. Data was available for all patients before and 3 and/or 6 months after initiation of first-line therapy. The prognostic relevance of different criteria for hematologic and cardiac response was assessed. There was a strong correlation between the extent of reduction of amyloidogenic free light chains (FLCs) and improvement in survival. This allowed the identification of four levels of response: amyloid complete response (normal FLC ratio and negative serum and urine immunofixation), very good partial response (difference between involved and uninvolved FLCs [dFLC] 50%), and no response. Cardiac involvement is the major determinant of survival, and changes in cardiac function after therapy can be reliably assessed using the cardiac biomarker N-terminal natriuretic peptide type B (NT-proBNP). Changes in FLC and NT-proBNP predicted survival as early as 3 months after treatment initiation. This study identifies and validates new criteria for response to first-line treatment in AL amyloidosis, based on their association with survival in large patient populations, and offers surrogate end points for clinical trials.

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Author and article information

Contributors

Dil Sahal: Role: Academic Editor

Hassan Izzedine: Role: Academic Editor

Vincent Audard: Role: Academic Editor

Carole Henique: Role: Academic Editor

Mario Ollero: Role: Academic Editor

Journal

Journal ID (nlm-ta): Diagnostics (Basel)

Journal ID (iso-abbrev): Diagnostics (Basel)

Journal ID (publisher-id): diagnostics

Title: Diagnostics

Publisher: MDPI

ISSN (Electronic): 2075-4418

Publication date (Electronic): 02 March 2021

Publication date Collection: March 2021

Volume: 11

Issue: 3

Electronic Location Identifier: 420

Affiliations

[1 ]Department of Nephrology Hôpital Necker, and INSERM U830 “Stress and Cancer” Laboratory, Institut Curie, 75015 Paris, France

[2 ]Department of Nephrology, CHU Poitiers, 86000 Poitiers, France; joly.floren@ 123456gmail.com (F.J.); audrey.sibille@ 123456chu-poitiers.fr (A.S.); vincent.javaugue@ 123456chu-poitiers.fr (V.J.); estelle.desport@ 123456chu-poitiers.fr (E.D.); Guy.TOUCHARD@ 123456chu-poitiers.fr (G.T.); Frank.BRIDOUX@ 123456chu-poitiers.fr (F.B.)

[3 ]Centre National de Référence Maladies Rares: Amylose AL et Autres Maladies à Dépôts d’Immunoglobulines Monoclonales, 86000 Poitiers, France

[4 ]INSERM CIC 1402, 86000 Poitiers, France

[5 ]CNRS UMR 7276-CRIBL, University of Limoges, 87000 Limoges, France; christophe.sirac@ 123456unilim.fr

[6 ]Department of Pathology, CHU Poitiers, 86000 Poitiers, France; Jean-Michel.GOUJON@ 123456chu-poitiers.fr

[7 ]Department of Immunology and Hematology, Hôpital Saint Louis, 75010 Paris, France; jpfermand@ 123456yahoo.fr

Author notes

[* ]Correspondence: camille.cohen@ 123456inserm.fr

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Christophe Sirac https://orcid.org/0000-0002-1183-4989

Article

Publisher ID: diagnostics-11-00420

DOI: 10.3390/diagnostics11030420

PMC ID: 7999117

PubMed ID: 33801393

SO-VID: 4fb4ae83-bb7e-46a0-9fd3-eb2cc6218442

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Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license ( http://creativecommons.org/licenses/by/4.0/).

Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management

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Most cited references 55

Evaluation and management of chronic kidney disease: synopsis of the kidney disease: improving global outcomes 2012 clinical practice guideline.

Targeting CD38 with Daratumumab Monotherapy in Multiple Myeloma.

New criteria for response to treatment in immunoglobulin light chain amyloidosis based on free light chain measurement and cardiac biomarkers: impact on survival outcomes.

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