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Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis

A man in his 50s with resistant hypertension and history of Langerhans cell histiocytosis (LCH) was referred to rheumatology after suspicion of inflammatory arteritis was raised. This followed detection of bilateral renal artery stenosis during investigation for severe hypertension refractory to med...

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Autores principales: Lakhani, Priyanka Mayur, Borysiewicz, Catherine, Mason, Justin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8928256/
https://www.ncbi.nlm.nih.gov/pubmed/35292540
http://dx.doi.org/10.1136/bcr-2021-242393
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author Lakhani, Priyanka Mayur
Borysiewicz, Catherine
Mason, Justin
author_facet Lakhani, Priyanka Mayur
Borysiewicz, Catherine
Mason, Justin
author_sort Lakhani, Priyanka Mayur
collection PubMed
description A man in his 50s with resistant hypertension and history of Langerhans cell histiocytosis (LCH) was referred to rheumatology after suspicion of inflammatory arteritis was raised. This followed detection of bilateral renal artery stenosis during investigation for severe hypertension refractory to medical therapy. CT angiography revealed diffuse wall thickening of the abdominal aorta, in keeping with an aortitis. However, there was no serological or clinical evidence suggestive of a vasculitic process. Medical history included cranial diabetes insipidus, subclavian artery stenosis and spinal stenosis requiring surgery, over the course of 8 years. These findings led to consideration of Erdheim-Chester disease (ECD), a form of non-Langerhans cell histiocytosis, where there is abnormal proliferation of histiocytes which causes tissue fibrosis and sclerosis of the long bones. Subsequent plain radiographs of the long bones revealed appearances consistent with a diagnosis of ECD. Thus, a diagnosis of an LCH/ECD overlap syndrome was made.
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spelling pubmed-89282562022-04-01 Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis Lakhani, Priyanka Mayur Borysiewicz, Catherine Mason, Justin BMJ Case Rep Case Reports: Unusual presentation of more common disease/injury A man in his 50s with resistant hypertension and history of Langerhans cell histiocytosis (LCH) was referred to rheumatology after suspicion of inflammatory arteritis was raised. This followed detection of bilateral renal artery stenosis during investigation for severe hypertension refractory to medical therapy. CT angiography revealed diffuse wall thickening of the abdominal aorta, in keeping with an aortitis. However, there was no serological or clinical evidence suggestive of a vasculitic process. Medical history included cranial diabetes insipidus, subclavian artery stenosis and spinal stenosis requiring surgery, over the course of 8 years. These findings led to consideration of Erdheim-Chester disease (ECD), a form of non-Langerhans cell histiocytosis, where there is abnormal proliferation of histiocytes which causes tissue fibrosis and sclerosis of the long bones. Subsequent plain radiographs of the long bones revealed appearances consistent with a diagnosis of ECD. Thus, a diagnosis of an LCH/ECD overlap syndrome was made. BMJ Publishing Group 2022-03-15 /pmc/articles/PMC8928256/ /pubmed/35292540 http://dx.doi.org/10.1136/bcr-2021-242393 Text en © BMJ Publishing Group Limited 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Case Reports: Unusual presentation of more common disease/injury
Lakhani, Priyanka Mayur
Borysiewicz, Catherine
Mason, Justin
Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title_full Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title_fullStr Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title_full_unstemmed Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title_short Erdheim-Chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
title_sort erdheim-chester disease: a rare cause of bilateral renal artery stenosis, mimicking large vessel vasculitis
topic Case Reports: Unusual presentation of more common disease/injury
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8928256/
https://www.ncbi.nlm.nih.gov/pubmed/35292540
http://dx.doi.org/10.1136/bcr-2021-242393
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