An unusual presentation of immunoglobulin A gammopathy in a patient with Schnitzler's syndrome.
IL-1 receptor antagonist
IgA gammopathy
Schnitzler's syndrome
Schnitzler-like syndromes
anakinra
Journal
International journal of rheumatic diseases
ISSN: 1756-185X
Titre abrégé: Int J Rheum Dis
Pays: England
ID NLM: 101474930
Informations de publication
Date de publication:
Oct 2023
Oct 2023
Historique:
revised:
09
03
2023
received:
09
11
2022
accepted:
19
04
2023
medline:
10
10
2023
pubmed:
16
5
2023
entrez:
16
5
2023
Statut:
ppublish
Résumé
This case summarizes a 69-year-old woman who presented with a history of recurrent fevers, widespread urticarial rash and generalized myalgias for several years with an eventual diagnosis of Schnitzler's syndrome. This is a rare autoinflammatory condition which typically involves a chronic urticarial rash, monoclonal immunoglobulin M (IgM) or IgG gammopathy. Rapid improvement in above symptoms were noted with anakinra, an interleukin-1 receptor antagonist. We report an unusual case of a 69-year-old woman who presented with an isolated IgA monoclonal gammopathy.
Identifiants
pubmed: 37191194
doi: 10.1111/1756-185X.14718
doi:
Substances chimiques
Immunoglobulin A
0
Interleukin 1 Receptor Antagonist Protein
0
Immunoglobulin M
0
Types de publication
Case Reports
Langues
eng
Sous-ensembles de citation
IM
Pagination
2085-2088Informations de copyright
© 2023 The Authors. International Journal of Rheumatic Diseases published by Asia Pacific League of Associations for Rheumatology and John Wiley & Sons Australia, Ltd.
Références
Simon A, Asli B, Braun-Falco M, et al. Schitzler's syndrome: diagnosis, treatment, and follow-up. Allergy. 2013;68(5):562-568.
De Koning HD. Schnitzler's syndrome: lessons from 281 cases. Clin Transl Allergy. 2014;4:41.
Lipsker D. The Schnitzler syndrome. Orphanet J Rare Dis. 2010;5:38.
Gusdorf L, Lipsker D. Schnitzler syndrome: a review. Curr Rheumatol Rep. 2017;19(8):46.
Strasbourg diagnostic criteria of Schnitzler syndrome. Allergy. 2013;68:562-568.
Szturz P, Sedivá A, Zurek M, et al. Anakinra treatment in Schnitzler syndrome-results of the first retrospective multicenter study in six patients from The Czech Republic. Klin Onkol. 2014;27(2):111-126.