Cargando…

IgG4-Related Disease With Renal and Pulmonary Involvement

Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids...

Descripción completa

Detalles Bibliográficos
Autores principales: Osuorji, Chinenye, Master, Kiron, Osuorji, Ikenna
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Cureus 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428951/
https://www.ncbi.nlm.nih.gov/pubmed/34522549
http://dx.doi.org/10.7759/cureus.17071
_version_ 1783750470565625856
author Osuorji, Chinenye
Master, Kiron
Osuorji, Ikenna
author_facet Osuorji, Chinenye
Master, Kiron
Osuorji, Ikenna
author_sort Osuorji, Chinenye
collection PubMed
description Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids therapy. We report the case of a 66-year-old man presenting with acute on chronic renal failure and pulmonary nodules seen on PET-CT scan. He also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. He failed steroid therapy but responded subsequently to rituximab with complete resolution of his symptoms.
format Online
Article
Text
id pubmed-8428951
institution National Center for Biotechnology Information
language English
publishDate 2021
publisher Cureus
record_format MEDLINE/PubMed
spelling pubmed-84289512021-09-13 IgG4-Related Disease With Renal and Pulmonary Involvement Osuorji, Chinenye Master, Kiron Osuorji, Ikenna Cureus Nephrology Immunoglobulin G4-related disease (IgG4-RD) is a rare immune-mediated disease affecting multiple organs and tissues. There is often the presence of elevated serum Ig4 subtype with histological evidence of lymphoplasmacytic infiltration, fibrosis, and phlebitis. The mainstay of treatment is steroids therapy. We report the case of a 66-year-old man presenting with acute on chronic renal failure and pulmonary nodules seen on PET-CT scan. He also had elevated serum IgG4 subclass and histological features in keeping with IgG4-RD. He failed steroid therapy but responded subsequently to rituximab with complete resolution of his symptoms. Cureus 2021-08-10 /pmc/articles/PMC8428951/ /pubmed/34522549 http://dx.doi.org/10.7759/cureus.17071 Text en Copyright © 2021, Osuorji et al. https://creativecommons.org/licenses/by/3.0/This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
spellingShingle Nephrology
Osuorji, Chinenye
Master, Kiron
Osuorji, Ikenna
IgG4-Related Disease With Renal and Pulmonary Involvement
title IgG4-Related Disease With Renal and Pulmonary Involvement
title_full IgG4-Related Disease With Renal and Pulmonary Involvement
title_fullStr IgG4-Related Disease With Renal and Pulmonary Involvement
title_full_unstemmed IgG4-Related Disease With Renal and Pulmonary Involvement
title_short IgG4-Related Disease With Renal and Pulmonary Involvement
title_sort igg4-related disease with renal and pulmonary involvement
topic Nephrology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8428951/
https://www.ncbi.nlm.nih.gov/pubmed/34522549
http://dx.doi.org/10.7759/cureus.17071
work_keys_str_mv AT osuorjichinenye igg4relateddiseasewithrenalandpulmonaryinvolvement
AT masterkiron igg4relateddiseasewithrenalandpulmonaryinvolvement
AT osuorjiikenna igg4relateddiseasewithrenalandpulmonaryinvolvement