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Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis

BACKGROUND AND OBJECTIVES: Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) have overlapping clinical considerations, which frequently involve peripheral neuropathy. The current study aimed to discriminate between the clinicopathological features of HES and E...

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Autores principales: Takeuchi, Hiroki, Kawamura, Kazuyuki, Kawasaki, Teruaki, Oka, Nobuyuki
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9705778/
https://www.ncbi.nlm.nih.gov/pubmed/36457867
http://dx.doi.org/10.3389/fneur.2022.1057767
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author Takeuchi, Hiroki
Kawamura, Kazuyuki
Kawasaki, Teruaki
Oka, Nobuyuki
author_facet Takeuchi, Hiroki
Kawamura, Kazuyuki
Kawasaki, Teruaki
Oka, Nobuyuki
author_sort Takeuchi, Hiroki
collection PubMed
description BACKGROUND AND OBJECTIVES: Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) have overlapping clinical considerations, which frequently involve peripheral neuropathy. The current study aimed to discriminate between the clinicopathological features of HES and EGPA, focusing on the mechanism of peripheral nerve damage. METHODS: A total of 53 patients who underwent nerve biopsies at our laboratory were examined: nine patients with idiopathic HES (iHES), three patients with reactive HES, 14 patients with myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-positive EGPA, and 27 patients with negative EGPA. Nerve biopsies were performed using light and electron microscopy. RESULTS: Polyneuropathy was more common than mononeuritis multiplex in iHES, which differed from that in ANCA-negative EGPA groups (p = 0.012). Nerve biopsies showed that iHES was associated with neuropathy features such as rare vasculitis and non-vasculitic eosinophilic infiltrates, which differed from those of ANCA-negative EGPA. Fibrinoid necrosis was found only in the reactive HES and ANCA-positive groups. The percentage of endoneurial vessels occluded with eosinophils tended to be higher in iHES (1.8%) than in ANCA-positive EGPA (0%) and negative EGPA (0.7%). In a patient with ANCA-negative EGPA, the endoneurial vessels were occluded with platelets, fibrinoid materials, and eosinophils, demonstrating the morphology of eosinophil extracellular traps. CONCLUSION: iHES with neuropathy showed a pattern more similar to polyneuropathy than mononeuritis multiplex, which is dominant in ANCA-negative EGPA, and tended to show vasculitis in the peripheral nerves less frequently compared with EGPA. Eosinophilic infiltration and endoneurial vascular occlusion by eosinophils may cause nerve damage.
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spelling pubmed-97057782022-11-30 Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis Takeuchi, Hiroki Kawamura, Kazuyuki Kawasaki, Teruaki Oka, Nobuyuki Front Neurol Neurology BACKGROUND AND OBJECTIVES: Hypereosinophilic syndrome (HES) and eosinophilic granulomatosis with polyangiitis (EGPA) have overlapping clinical considerations, which frequently involve peripheral neuropathy. The current study aimed to discriminate between the clinicopathological features of HES and EGPA, focusing on the mechanism of peripheral nerve damage. METHODS: A total of 53 patients who underwent nerve biopsies at our laboratory were examined: nine patients with idiopathic HES (iHES), three patients with reactive HES, 14 patients with myeloperoxidase-anti-neutrophil cytoplasmic antibody (ANCA)-positive EGPA, and 27 patients with negative EGPA. Nerve biopsies were performed using light and electron microscopy. RESULTS: Polyneuropathy was more common than mononeuritis multiplex in iHES, which differed from that in ANCA-negative EGPA groups (p = 0.012). Nerve biopsies showed that iHES was associated with neuropathy features such as rare vasculitis and non-vasculitic eosinophilic infiltrates, which differed from those of ANCA-negative EGPA. Fibrinoid necrosis was found only in the reactive HES and ANCA-positive groups. The percentage of endoneurial vessels occluded with eosinophils tended to be higher in iHES (1.8%) than in ANCA-positive EGPA (0%) and negative EGPA (0.7%). In a patient with ANCA-negative EGPA, the endoneurial vessels were occluded with platelets, fibrinoid materials, and eosinophils, demonstrating the morphology of eosinophil extracellular traps. CONCLUSION: iHES with neuropathy showed a pattern more similar to polyneuropathy than mononeuritis multiplex, which is dominant in ANCA-negative EGPA, and tended to show vasculitis in the peripheral nerves less frequently compared with EGPA. Eosinophilic infiltration and endoneurial vascular occlusion by eosinophils may cause nerve damage. Frontiers Media S.A. 2022-11-15 /pmc/articles/PMC9705778/ /pubmed/36457867 http://dx.doi.org/10.3389/fneur.2022.1057767 Text en Copyright © 2022 Takeuchi, Kawamura, Kawasaki and Oka. https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Takeuchi, Hiroki
Kawamura, Kazuyuki
Kawasaki, Teruaki
Oka, Nobuyuki
Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title_full Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title_fullStr Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title_full_unstemmed Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title_short Distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
title_sort distinct features of hypereosinophilic syndrome with neuropathy from eosinophilic granulomatosis with polyangiitis
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9705778/
https://www.ncbi.nlm.nih.gov/pubmed/36457867
http://dx.doi.org/10.3389/fneur.2022.1057767
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