Refractory serum immunoglobulin M elevation during anti-interleukin (IL)-1- or IL-6-targeted treatment in four patients with Schnitzler syndrome.
Schnitzler syndrome
anakinra
canakinumab
immunoglobulin M
tocilizumab
Journal
The Journal of dermatology
ISSN: 1346-8138
Titre abrégé: J Dermatol
Pays: England
ID NLM: 7600545
Informations de publication
Date de publication:
Nov 2021
Nov 2021
Historique:
revised:
26
07
2021
received:
01
07
2021
accepted:
10
08
2021
pubmed:
27
8
2021
medline:
4
11
2021
entrez:
26
8
2021
Statut:
ppublish
Résumé
Schnitzler syndrome is characterized by chronic urticarial rash, neutrophilic dermal infiltrate, recurrent fever, bone pain, elevated C-reactive protein, and neutrophilic leukocytosis. The pathophysiology of Schnitzler syndrome is unknown, but it is considered to be an acquired form of an autoinflammatory disease because of the resemblance to clinical phenotypes of cryopyrin-associated periodic syndrome, in which a gain-of-function mutation in NLRP3 causes overexpression of interleukin (IL)-1β. Schnitzler syndrome is generally accompanied by a monoclonal immunoglobulin (Ig)M gammopathy with a long-term risk of lymphoproliferation that is possibly associated with an MYD88 mutation. Herein, we present the following four patients with Schnitzler syndrome: a 63-year-old woman; a 65-year-old man; a 43-year-old woman; and a 63-year-old woman. Each patient fulfilled the Strasbourg diagnostic criteria, but none of the patients had any mutation in NLRP3 or MYD88 detected in their peripheral blood. Although approved treatment options for Schnitzler syndrome are lacking, our patients were treated with IL-1-targeted therapy (anakinra or canakinumab) or anti-IL-6 (tocilizumab). The acute inflammatory clinical manifestations improved completely with canakinumab and partially with anakinra and tocilizumab, but the serum IgM levels were gradually increased in all patients, even during treatment. To determine whether treatment with anti-IL-1β or IL-6 prevents conversion to a hematopoietic disorder, further collection of cases and long-term follow-up will be needed.
Identifiants
pubmed: 34435697
doi: 10.1111/1346-8138.16124
doi:
Substances chimiques
Antibodies, Monoclonal
0
Immunoglobulin M
0
Interleukin 1 Receptor Antagonist Protein
0
Interleukin-6
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
1789-1792Subventions
Organisme : Ministry of Health, Labour and Welfare
Informations de copyright
© 2021 Japanese Dermatological Association.
Références
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