Epidemiological and clinical characteristics and outcome of monoclonal gammopathy of renal significance‐related lesions in Latin America

Autor: Gonzalo P. Méndez, Dorotea Fantl, Vivianne Lois, Natalia Schutz, Paola Mur, Camila Peña, Daniela Zamora, Christine Rojas, Verónica Verri, Hernán López-Vidal, Grupo de estudio latinoamericano de mieloma múltiple, Gonzalo Correa, Guillermo Andrés Duque Silva, Claudia Shanley, Jhoanna Ramirez, Gustavo Glavic, Paola Ochoa, Eloisa Riva, José Tomás Gonzalez, Ricardo Valjalo, Alejandro Majlis, Karen Jiset Saavedra Escobar
Rok vydání: 2019
Předmět:
Zdroj: Nephrology. 25:442-449
ISSN: 1440-1797
1320-5358
DOI: 10.1111/nep.13685
Popis: Background Monoclonal gammopathy of renal significance (MGRS)-related lesions are infrequent entities. There are no publications on these disorders in Latin America (LA). The aim of this study was to describe epidemiological and clinical characteristics of these patients in LA. Methods We performed a multicentre retrospective study. Patients with diagnosis of MGRS between 2012 and 2018 were included. Epidemiological and clinical data were collected from clinical records. Results Twenty-seven patients from Chile, Argentina, Ecuador and Uruguay were included. Half debuted with a nephrotic syndrome, and 32% required dialysis. Proliferative glomerulonephritis with monoclonal immunoglobulin deposits was found in 33%, amyloidosis in 26% and monoclonal immunoglobulin deposition disease also in 26%. The immunoglobulin most frequently found in renal biopsies was IgG kappa. In 67% a paraprotein was found. Twenty patients received an anti-plasma cell regimen, and 3 a rituximab-based regimen (IgM-MGRS). Renal response (RR) was achieved in 56%. Early treatment (≤3 months) was associated with higher RR (75% vs 43%). Three patients relapsed within 21.5 months, and 3 progressed: 1 to multiple myeloma, 1 to systemic amyloidosis and another to systemic light-chain deposition disease. Two patients died, both due to infection during induction treatment. Conclusion There was a higher than expected frequency of patients requiring dialysis. The most common MGRS-related lesion was PGNMD. Early treatment was associated with better response. As a rare disease, increasing awareness and promoting early diagnosis are necessary in LA to improve outcomes. SUMMARY AT A GLANCE A collection of 27 cases of MGRS from Latin America with information on epidemiology, clinical characteristics, treatment and outcome of patients diagnosed of MGRS-related renal lesions.
Databáze: OpenAIRE
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