A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge

Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycopheno...

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Autores principales: Gormley, Sinead, Bravo, Paola Tacuri, Kos, Xavier, Solanki, Kamal, Khashram, Manar
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2022
Materias:
Case report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9108322/
https://www.ncbi.nlm.nih.gov/pubmed/35586678
http://dx.doi.org/10.1016/j.jvscit.2022.02.003
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author Gormley, Sinead
Bravo, Paola Tacuri
Kos, Xavier
Solanki, Kamal
Khashram, Manar
author_facet Gormley, Sinead
Bravo, Paola Tacuri
Kos, Xavier
Solanki, Kamal
Khashram, Manar
author_sort Gormley, Sinead
collection PubMed
description Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions.
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spelling pubmed-91083222022-05-17 A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge Gormley, Sinead Bravo, Paola Tacuri Kos, Xavier Solanki, Kamal Khashram, Manar J Vasc Surg Cases Innov Tech Case report Immunoglobulin (Ig)G4-related disease (IgG4-RD) with retroperitoneal fibrosis (RPF) is a rare, fibroinflammatory disease involving the soft tissues of the retroperitoneum. A 73-year-old man with IgG4-related RPF affecting the abdominal aorta and iliac arteries was treated with steroids and mycophenolate mofetil. The prevalence of the disease remains unknown because it is often misdiagnosed and can mimic many malignant, infectious, and inflammatory conditions. Autoimmune pancreatitis is a common presenting condition of IgG4-RD. Because As IgG4-RD is responsive to steroids, diagnosing IgG4-related RPF early can prevent the exposure of patients with RPF to unnecessary diagnostic and therapeutic interventions. Elsevier 2022-03-08 /pmc/articles/PMC9108322/ /pubmed/35586678 http://dx.doi.org/10.1016/j.jvscit.2022.02.003 Text en © 2022 The Authors https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Case report
Gormley, Sinead
Bravo, Paola Tacuri
Kos, Xavier
Solanki, Kamal
Khashram, Manar
A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title_full A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title_fullStr A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title_full_unstemmed A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title_short A case of IgG4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
title_sort case of igg4-related retroperitoneal fibrosis with significant involvement of the abdominal aorta—a clinical and diagnostic challenge
topic Case report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9108322/
https://www.ncbi.nlm.nih.gov/pubmed/35586678
http://dx.doi.org/10.1016/j.jvscit.2022.02.003
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