Summary of the current status of clinically diagnosed cases of Schnitzler syndrome in Japan.
Autoinflammatory disorders
Chronic urticaria
Japan
Monoclonal gammopathy
Schnitzler syndrome
Journal
Allergology international : official journal of the Japanese Society of Allergology
ISSN: 1440-1592
Titre abrégé: Allergol Int
Pays: England
ID NLM: 9616296
Informations de publication
Date de publication:
Apr 2023
Apr 2023
Historique:
received:
22
08
2022
revised:
16
10
2022
accepted:
31
10
2022
medline:
4
4
2023
pubmed:
6
12
2022
entrez:
5
12
2022
Statut:
ppublish
Résumé
Schnitzler syndrome is a rare disorder with chronic urticaria, and there is no report summarizing the current status in Japan. A nationwide survey of major dermatology departments in Japan was conducted in 2019. We further performed a systematic search of PubMed and Ichushi-Web, using the keywords "Schnitzler syndrome" and "Japan" then contacted the corresponding authors or physicians for further information. Excluding duplicates, a total of 36 clinically diagnosed cases were identified from 1994 through the spring of 2022, with a male to female ratio of 1:1. The median age of onset was 56.5 years. It took 3.3 years from the first symptom, mostly urticaria, to reach the final diagnosis. The current status of 30 cases was ascertained; two patients developed B-cell lymphoma. SchS treatment was generally effective with high doses of corticosteroids, but symptoms sometimes recurred after tapering. Colchicine was administered in 17 cases and was effective in 8, but showed no effect in the others. Tocilizumab, used in six cases, improved laboratory abnormalities and symptoms, but lost its efficacy after several years. Rituximab, used in five cases, was effective in reducing serum IgM levels or lymphoma mass, but not in inflammatory symptoms. Four cases were treated with IL-1 targeting therapy, either anakinra or canakinumab, and achieved complete remission, except one case with diffuse large B-cell lymphoma. Since Schnitzler syndrome is a rare disease, the continuous collection and long-term follow-up of clinical information is essential for its appropriate treatment and further understanding of its pathophysiology.
Sections du résumé
BACKGROUND
BACKGROUND
Schnitzler syndrome is a rare disorder with chronic urticaria, and there is no report summarizing the current status in Japan.
METHODS
METHODS
A nationwide survey of major dermatology departments in Japan was conducted in 2019. We further performed a systematic search of PubMed and Ichushi-Web, using the keywords "Schnitzler syndrome" and "Japan" then contacted the corresponding authors or physicians for further information.
RESULTS
RESULTS
Excluding duplicates, a total of 36 clinically diagnosed cases were identified from 1994 through the spring of 2022, with a male to female ratio of 1:1. The median age of onset was 56.5 years. It took 3.3 years from the first symptom, mostly urticaria, to reach the final diagnosis. The current status of 30 cases was ascertained; two patients developed B-cell lymphoma. SchS treatment was generally effective with high doses of corticosteroids, but symptoms sometimes recurred after tapering. Colchicine was administered in 17 cases and was effective in 8, but showed no effect in the others. Tocilizumab, used in six cases, improved laboratory abnormalities and symptoms, but lost its efficacy after several years. Rituximab, used in five cases, was effective in reducing serum IgM levels or lymphoma mass, but not in inflammatory symptoms. Four cases were treated with IL-1 targeting therapy, either anakinra or canakinumab, and achieved complete remission, except one case with diffuse large B-cell lymphoma.
CONCLUSIONS
CONCLUSIONS
Since Schnitzler syndrome is a rare disease, the continuous collection and long-term follow-up of clinical information is essential for its appropriate treatment and further understanding of its pathophysiology.
Identifiants
pubmed: 36470790
pii: S1323-8930(22)00129-0
doi: 10.1016/j.alit.2022.11.004
pii:
doi:
Substances chimiques
Interleukin 1 Receptor Antagonist Protein
0
Types de publication
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
297-305Informations de copyright
Copyright © 2022 Japanese Society of Allergology. Published by Elsevier B.V. All rights reserved.