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Retention rate of IL-1 inhibitors in Schnitzler’s syndrome


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21

 

  1. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Italy. crisafulli.francesca10@gmail.com
  2. Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.
  3. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Italy.
  4. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Italy.
  5. Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.
  6. Vita-Salute San Raffaele University, Milan, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UniRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
  7. Vita-Salute San Raffaele University, Milan, and Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UniRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy.
  8. Department of Clinical Sciences and Community Health, Research Center for Adult and Paediatric Rheumatic Diseases, Università degli Studi di Milano, and Division of Paediatric Rheumatology, ASST Gaetano Pini-CTO Institute, Milan, Italy.
  9. Rheumatology Unit, University of Verona, Policlinico G. B. Rossi, Verona, Italy.
  10. Rheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari Aldo Moro, Bari, Italy.
  11. Rheumatology Unit, Department of Emergency and Organ Transplantations, University of Bari Aldo Moro, Bari, Italy.
  12. Department of Rheumatology, Santa Chiara Hospital, Trento, Italy.
  13. Department of Rheumatology, Santa Chiara Hospital, Trento, Italy.
  14. Department of Rheumatology, IRCCS Fondazione Policlinico S. Matteo, Pavia, and University of Pavia, Italy.
  15. Department of Rheumatology, IRCCS Fondazione Policlinico S. Matteo, Pavia, and University of Pavia, Italy.
  16. Department of Rheumatology, IRCCS Fondazione Policlinico S. Matteo, Pavia, and University of Pavia, Italy.
  17. Department of Experimental and Clinical Medicine, University of Florence, Italy.
  18. Department of Experimental and Clinical Medicine, University of Florence, Italy.
  19. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Italy.
  20. Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Italy.
  21. Rheumatology and Clinical Immunology Unit, ASST Spedali Civili and University of Brescia, Italy.

CER14961
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PMID: 35084307 [PubMed]

Received: 02/07/2021
Accepted : 08/11/2021
In Press: 12/01/2022

Abstract

OBJECTIVES:
Schnitzler’s syndrome is a rare autoinflammatory disease. Clinical response to IL-1 inhibitor drugs has been described, but limited information is available on the long-term efficacy and safety of these agents in Schnitzler’s syndrome.
METHODS:
A retrospective study was conducted of patients with Schnitzler’s syndrome fulfilling Strasbourg diagnostic criteria followed in 9 Italian centres. The retention rate of IL-1 inhibitors was evaluated using Kaplan-Meier analysis.
RESULTS:
Fifteen of 20 patients with Schnitzler’s syndrome were treated with IL-1 inhibitors: in total, they received 16 courses of anakinra (median duration 20.0 months [6.0–58.3]), and 8 courses of canakinumab (median duration 19.0 months [13.5–31.0]). The retention rate of IL-1 inhibitors was 73.4% [SE 9.4] at 1 year and 63.6% [SE 10.4] at 2 years. There was no significant difference between the retention rate of anakinra and canakinumab. The retention rate was higher in patients with a definite diagnosis according to the Strasbourg criteria as compared with those with a probable diagnosis (p=0.03). At the last follow-up visit, all patients who started therapy with IL-1 inhibitors were still on treatment, although in some cases with an increased dosage compared to the start of therapy. A sparing effect on the use of conventional synthetic disease-modifying anti-rheumatic drugs and a significant reduction of prednisone dosage (p=0.02) and of serum amyloid A (SAA) levels (p=0.03) were observed.
CONCLUSIONS:
The retention rate of IL-1 inhibitors in patients with Schnitzler’s syndrome was high, particularly in patients with a definite diagnosis according to the Strasbourg criteria, reflecting their effectiveness in the treatment of this syndrome.

DOI: https://doi.org/10.55563/clinexprheumatol/14hu2k

Rheumatology Article