Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management
| Title: | Randall-Type Monoclonal Immunoglobulin Deposition Disease: New Insights into the Pathogenesis, Diagnosis and Management |
|---|---|
| Authors: | Camille Cohen; Florent Joly; Audrey Sibille; Vincent Javaugue; Estelle Desport; Jean-Michel Goujon; Guy Touchard; Jean-Paul Fermand; Christophe Sirac; Frank Bridoux |
| Source: | Diagnostics, Vol 11, Iss 3, p 420 (2021) |
| Publisher Information: | MDPI AG |
| Publication Year: | 2021 |
| Collection: | Directory of Open Access Journals: DOAJ Articles |
| Subject Terms: | MGRS; plasma cell dyscrasia; glomerular disease; monoclonal gammopathy; Medicine (General); R5-920 |
| Description: | 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. |
| Document Type: | article in journal/newspaper |
| Language: | English |
| Relation: | https://www.mdpi.com/2075-4418/11/3/420; https://doaj.org/toc/2075-4418; https://doaj.org/article/48843799de3b4089aa06b0c245abb268 |
| DOI: | 10.3390/diagnostics11030420 |
| Availability: | https://doi.org/10.3390/diagnostics11030420; https://doaj.org/article/48843799de3b4089aa06b0c245abb268 |
| Accession Number: | edsbas.65E79480 |
| Database: | BASE |