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Schnitzler syndrome and Schnitzler-like syndromes

Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with...

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Autor principal: Chu, Cong-Qiu
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337259/
https://www.ncbi.nlm.nih.gov/pubmed/35089885
http://dx.doi.org/10.1097/CM9.0000000000002015
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author Chu, Cong-Qiu
author_facet Chu, Cong-Qiu
author_sort Chu, Cong-Qiu
collection PubMed
description Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation of NLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although no NLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases.
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spelling pubmed-93372592022-08-01 Schnitzler syndrome and Schnitzler-like syndromes Chu, Cong-Qiu Chin Med J (Engl) Review Articles Schnitzler syndrome is a rare disease of adult-onset with main features including chronic urticarial rash, recurrent fever, arthralgia or arthritis, monoclonal gammopathy of undetermined significance (MGUS), and marked systemic inflammation. Schnitzler syndrome is often underdiagnosed. Patients with Schnitzler syndrome may present to dermatologists and allergists for urticaria, hematologists for MGUS, or rheumatologists for arthritis. It is important to recognize Schnitzler syndrome for its remarkable response to interleukin (IL)-1 blockade. Besides, many cases of Schnitzler-like syndromes do not meet the diagnostic criteria of classical Schnitzler syndrome but display excellent response to IL-1 inhibitors. The overly produced IL-1 is the result of a somatic mosaic gain of function mutation of NLRP3 (nucleotide-binding oligomerization domain [NOD]-like receptor [NLR] family pyrin domain containing 3) gene in some patients with Schnitzler-like syndromes. Inflammasome activation is evident in patients with classical Schnitzler syndrome although no NLRP3 gene mutation is identified. Collectively, Schnitzler syndrome and Schnitzler-like syndromes represent a spectrum of IL-1 mediated adult-onset autoinflammatory diseases. Lippincott Williams & Wilkins 2022-05-20 2022-01-27 /pmc/articles/PMC9337259/ /pubmed/35089885 http://dx.doi.org/10.1097/CM9.0000000000002015 Text en Copyright © 2022 The Chinese Medical Association, produced by Wolters Kluwer, Inc. under the CC-BY-NC-ND license. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-Non Commercial-No Derivatives License 4.0 (CCBY-NC-ND), where it is permissible to download and share the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal. http://creativecommons.org/licenses/by-nc-nd/4.0 (https://creativecommons.org/licenses/by-nc-nd/4.0/)
spellingShingle Review Articles
Chu, Cong-Qiu
Schnitzler syndrome and Schnitzler-like syndromes
title Schnitzler syndrome and Schnitzler-like syndromes
title_full Schnitzler syndrome and Schnitzler-like syndromes
title_fullStr Schnitzler syndrome and Schnitzler-like syndromes
title_full_unstemmed Schnitzler syndrome and Schnitzler-like syndromes
title_short Schnitzler syndrome and Schnitzler-like syndromes
title_sort schnitzler syndrome and schnitzler-like syndromes
topic Review Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9337259/
https://www.ncbi.nlm.nih.gov/pubmed/35089885
http://dx.doi.org/10.1097/CM9.0000000000002015
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