Léčba anakinrou u Schnitzler syndromu - výsledky první retrospektivní multicentrické studie šesti pacientů z České republiky [Anakinra treatment in Schnitzler syndrome - results of the first retrospective multicenter study in six patients from the Czech Republic]

Détails

ID Serval
serval:BIB_C13DAC1C032D
Type
Article: article d'un périodique ou d'un magazine.
Collection
Publications
Titre
Léčba anakinrou u Schnitzler syndromu - výsledky první retrospektivní multicentrické studie šesti pacientů z České republiky [Anakinra treatment in Schnitzler syndrome - results of the first retrospective multicenter study in six patients from the Czech Republic]
Périodique
Klinicka onkologie
Auteur⸱e⸱s
Szturz Petr, Sedivá A., Zurek M., Adam Z., Stork J., Cermáková Z., Steyerová P., Vokáčová A., Hrbek J., Sýkora M., Spička I., Mechl Z., Mayer J.
ISSN
0862-495X (Print)
ISSN-L
0862-495X
Statut éditorial
Publié
Date de publication
2014
Peer-reviewed
Oui
Volume
27
Numéro
2
Pages
111-126
Langue
tchèque
Notes
Publication types: English Abstract ; Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Résumé
Schnitzler syndrome is a very rare, acquired, autoinflammatory disease of mostly adult onset with characteristic combination of chronic recurrent urticaria and monoclonal immunoglobulin M or G gammopathy predisposing the patients to malignant lymphoproliferation. In this work, we analyzed the results of bio-logical therapy with anakinra on a national level aiming to supply data for effective pharmaco-economic estimates, lay the grounds of nationwide patient registry, raise awareness among professional public and optimize provided health care.
The retrospective study (10/ 2006- 9/ 2013) included six males with definite Schnitzler syndrome verified by the new Strasbourg criteria. All patients were pretreated with antihistamines, nonsteroidal antiinflammatory drugs and glucocorticoids. Four patients underwent two or more treatment lines including intravenous bisphosphonates, 2- chlorodeoxyadenosine (cladribine), interferonα, PUVA photochemotherapy, cyclosporine A, thalidomide, bortezomib, chlorambucil, cyclophosphamide, colchicine and methotrexate. Anakinra monotherapy was initiated in standard dosing (100 mg subcutaneously daily).
Complete and partial remissions were achieved in five (83%) and one patients (17%), respectively. Complete remission was characterized by urticaria and pain regression (within hours), normalization of inflammatory markers (with--in days) and bone metabolism improvement assessed by the markers of osteoblastic osteoformation and osteoclastic osteoresorption in one case (within weeks). With normalized inflammatory markers (including interleukin6 and interleukin18), arthralgia and sporadic exacerbations of urticaria and fevers persist in the patient in partial remission with proven Q703K polymorphism in NLRP3 gene. The median treatment followup was 30.5 months (37.2 ± 31.2 (n = 6)). The dosing interval was prolonged in one case of complete remission to 48 hours. No serious adverse reactions occurred during anakinra application.
In Schnitzler syndrome, anakinra represents an effective, verified and safe medication with potentionally longterm administration not compromising its original efficacy and subjective tolerance. Anakinra, blocking autonomous inflammatory reaction of the organism via interleukin1 pathway, is a generally accepted first line treatment that should be made available in standard dosing for all Schnitzler patients.
Mots-clé
Antirheumatic Agents/therapeutic use, Czech Republic, Humans, Interleukin 1 Receptor Antagonist Protein/therapeutic use, Remission Induction, Retrospective Studies, Schnitzler Syndrome/diagnosis, Schnitzler Syndrome/drug therapy, Schnitzler Syndrome/immunology
Pubmed
Open Access
Oui
Création de la notice
07/01/2025 11:45
Dernière modification de la notice
08/01/2025 7:04
Données d'usage