Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease

Abstract Schnitzler’s syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed li...

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Main Authors:	FS van Leersum, J Potjewijd, M van Geel, PM Steijlen, M Vreeburg
Format:	Article
Language:	English
Published:	BMC 2019-06-01
Series:	Orphanet Journal of Rare Diseases
Subjects:	Schnitzler’s syndrome Waldenströms macroglobulinemia Autoinflammatory disease Hypothesis Interleukin-1 MYD88
Online Access:	http://link.springer.com/article/10.1186/s13023-019-1117-2

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spelling	doaj-64fe91dade284d698c84c1c568ad3f032020-11-25T03:10:43ZengBMCOrphanet Journal of Rare Diseases1750-11722019-06-011411410.1186/s13023-019-1117-2Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s diseaseFS van Leersum0J Potjewijd1M van Geel2PM Steijlen3M Vreeburg4Department of Dermatology, Maastricht University Medical CentreDepartment of Internal Medicine, Division of Clinical and Experimental Immunology, Maastricht University Medical CentreDepartment of Dermatology, Maastricht University Medical CentreDepartment of Dermatology, Maastricht University Medical CentreClinical Genetics, Maastricht University Medical CentreAbstract Schnitzler’s syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed link between interleukin-1 and the monoclonal gammopathy is not yet elucidated, a mutual factor in pathophysiology however seems likely. Here we present a novel hypothesis of a shared pathophysiologic mechanism between Schitzler’s syndrome and monoclonal gammopathy.http://link.springer.com/article/10.1186/s13023-019-1117-2Schnitzler’s syndromeWaldenströms macroglobulinemiaAutoinflammatory diseaseHypothesisInterleukin-1MYD88
collection	DOAJ
language	English
format	Article
sources	DOAJ
author	FS van Leersum J Potjewijd M van Geel PM Steijlen M Vreeburg
spellingShingle	FS van Leersum J Potjewijd M van Geel PM Steijlen M Vreeburg Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease Orphanet Journal of Rare Diseases Schnitzler’s syndrome Waldenströms macroglobulinemia Autoinflammatory disease Hypothesis Interleukin-1 MYD88
author_facet	FS van Leersum J Potjewijd M van Geel PM Steijlen M Vreeburg
author_sort	FS van Leersum
title	Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease
title_short	Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease
title_full	Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease
title_fullStr	Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease
title_full_unstemmed	Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease
title_sort	schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with waldenström’s disease
publisher	BMC
series	Orphanet Journal of Rare Diseases
issn	1750-1172
publishDate	2019-06-01
description	Abstract Schnitzler’s syndrome is an auto-inflammatory disorder which is characterized by two mandatory features: an urticarial rash and a monoclonal gammopathy. Although the pathophysiology of this syndrome is not yet fully understood, a role for interleukin-1 seems apparent. While this presumed link between interleukin-1 and the monoclonal gammopathy is not yet elucidated, a mutual factor in pathophysiology however seems likely. Here we present a novel hypothesis of a shared pathophysiologic mechanism between Schitzler’s syndrome and monoclonal gammopathy.
topic	Schnitzler’s syndrome Waldenströms macroglobulinemia Autoinflammatory disease Hypothesis Interleukin-1 MYD88
url	http://link.springer.com/article/10.1186/s13023-019-1117-2
work_keys_str_mv	AT fsvanleersum schnitzlerssyndromeanovelhypothesisofasharedpathophysiologicmechanismwithwaldenstromsdisease AT jpotjewijd schnitzlerssyndromeanovelhypothesisofasharedpathophysiologicmechanismwithwaldenstromsdisease AT mvangeel schnitzlerssyndromeanovelhypothesisofasharedpathophysiologicmechanismwithwaldenstromsdisease AT pmsteijlen schnitzlerssyndromeanovelhypothesisofasharedpathophysiologicmechanismwithwaldenstromsdisease AT mvreeburg schnitzlerssyndromeanovelhypothesisofasharedpathophysiologicmechanismwithwaldenstromsdisease
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Schnitzler’s syndrome - a novel hypothesis of a shared pathophysiologic mechanism with Waldenström’s disease

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