Cargando…

Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus

Childhood-onset systemic lupus erythematosus (cSLE) only represents 20% of all SLE patients, and males with SLE only represent 10%. To study this rare SLE subset, males diagnosed with cSLE over a 30-year period were identified. Organ involvement, autoantibody production, hypocomplementemia, and kidn...

Descripción completa

Detalles Bibliográficos
Autores principales: Wenderfer, Scott E., Orjuela, Alvaro, Bekheirnia, Mir Reza, Pereira, Maria, Muscal, Eyal, Braun, Michael C., De Guzman, Marietta
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9149811/
https://www.ncbi.nlm.nih.gov/pubmed/35645367
http://dx.doi.org/10.3390/pediatric14020030
_version_ 1784717282866364416
author Wenderfer, Scott E.
Orjuela, Alvaro
Bekheirnia, Mir Reza
Pereira, Maria
Muscal, Eyal
Braun, Michael C.
De Guzman, Marietta
author_facet Wenderfer, Scott E.
Orjuela, Alvaro
Bekheirnia, Mir Reza
Pereira, Maria
Muscal, Eyal
Braun, Michael C.
De Guzman, Marietta
author_sort Wenderfer, Scott E.
collection PubMed
description Childhood-onset systemic lupus erythematosus (cSLE) only represents 20% of all SLE patients, and males with SLE only represent 10%. To study this rare SLE subset, males diagnosed with cSLE over a 30-year period were identified. Organ involvement, autoantibody production, hypocomplementemia, and kidney biopsy findings were compared to cSLE females. Outcomes were assessed using SLE Disease Activity Index scores, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and Childhood Arthritis and Rheumatology Research Alliance definitions for nephritis responsiveness. Of 95 males and 545 females with cSLE, 62% and 57% developed nephritis, respectively. Median age of cSLE onset was 14 years in both genders. Among males, 80% of non-Hispanic whites, 64% of blacks, 59% of Hispanics, and 50% of Asians developed nephritis. The prevalence of pure and mixed class V membranous nephritis was 33%. Median follow-up was 3.2 years (range 0.1–18). Complete kidney responses were seen in 70% after a median 24 months; however, relapse rates were 46%. Kidney disease flares were 56% nephritic and 44% proteinuric. Males and females with cSLE present with comparable rates and nephritis class. While overall and kidney response rates are favorable, kidney disease relapses are common among males.
format Online
Article
Text
id pubmed-9149811
institution National Center for Biotechnology Information
language English
publishDate 2022
publisher MDPI
record_format MEDLINE/PubMed
spelling pubmed-91498112022-05-31 Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus Wenderfer, Scott E. Orjuela, Alvaro Bekheirnia, Mir Reza Pereira, Maria Muscal, Eyal Braun, Michael C. De Guzman, Marietta Pediatr Rep Article Childhood-onset systemic lupus erythematosus (cSLE) only represents 20% of all SLE patients, and males with SLE only represent 10%. To study this rare SLE subset, males diagnosed with cSLE over a 30-year period were identified. Organ involvement, autoantibody production, hypocomplementemia, and kidney biopsy findings were compared to cSLE females. Outcomes were assessed using SLE Disease Activity Index scores, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and Childhood Arthritis and Rheumatology Research Alliance definitions for nephritis responsiveness. Of 95 males and 545 females with cSLE, 62% and 57% developed nephritis, respectively. Median age of cSLE onset was 14 years in both genders. Among males, 80% of non-Hispanic whites, 64% of blacks, 59% of Hispanics, and 50% of Asians developed nephritis. The prevalence of pure and mixed class V membranous nephritis was 33%. Median follow-up was 3.2 years (range 0.1–18). Complete kidney responses were seen in 70% after a median 24 months; however, relapse rates were 46%. Kidney disease flares were 56% nephritic and 44% proteinuric. Males and females with cSLE present with comparable rates and nephritis class. While overall and kidney response rates are favorable, kidney disease relapses are common among males. MDPI 2022-05-06 /pmc/articles/PMC9149811/ /pubmed/35645367 http://dx.doi.org/10.3390/pediatric14020030 Text en © 2022 by the authors. https://creativecommons.org/licenses/by/4.0/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 (https://creativecommons.org/licenses/by/4.0/).
spellingShingle Article
Wenderfer, Scott E.
Orjuela, Alvaro
Bekheirnia, Mir Reza
Pereira, Maria
Muscal, Eyal
Braun, Michael C.
De Guzman, Marietta
Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title_full Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title_fullStr Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title_full_unstemmed Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title_short Lupus Nephritis, Autoantibody Production and Kidney Outcomes in Males with Childhood-Onset Systemic Lupus Erythematosus
title_sort lupus nephritis, autoantibody production and kidney outcomes in males with childhood-onset systemic lupus erythematosus
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9149811/
https://www.ncbi.nlm.nih.gov/pubmed/35645367
http://dx.doi.org/10.3390/pediatric14020030
work_keys_str_mv AT wenderferscotte lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT orjuelaalvaro lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT bekheirniamirreza lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT pereiramaria lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT muscaleyal lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT braunmichaelc lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus
AT deguzmanmarietta lupusnephritisautoantibodyproductionandkidneyoutcomesinmaleswithchildhoodonsetsystemiclupuserythematosus