Paediatric Rheumatology
On demand corticosteroid use in the syndrome of undifferentiated recurrent fever: a literature review and results from JIR-CLiPS survey study
E.D. Batu1, S. Sener2, M. Rodrigues3, C. Vinit4, F. Hofer5, K. Laskari6, R. Craveiro Costa7, M. Santos Faria8, G. Ozomay Baykal9, O. Boyarchuk10, O. Gilliaux11, K. Pateras12, H.E. Sonmez13, N. Toplak14, M. Gattorno15, M. Hofer16
- Division of Paediatric Rheumatology, Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey. ezgidenizbatu@yahoo.com
- Division of Paediatric Rheumatology, Department of Paediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.
- Paediatric and Young Adult Rheumatology Unit, Centro Hospitalar Universitário São João, Porto Medical School, Porto, Portugal.
- Department of General Paediatrics, Jean Verdier Hospital, Bondy; and Department of Paediatric Rheumatology, Robert Debre Hospital, Paris, France.
- Fondation RES, Lausanne, Switzerland.
- First Department of Propaedeutic and Internal Medicine, National and Kapodistrian University of Athens, Greece.
- Hospital Pediátrico, Unidade Local de Saúde de Coimbra, Coimbra, Portugal.
- Department of Rheumatology, Hospital Central do Funchal, SESARAM, Funchal, Portugal.
- Division of Paediatric Rheumatology, Department of Paediatrics, Umraniye Training and Research Hospital, Istanbul, Turkey.
- Department of Children's Diseases and Paediatric Surgery, I. Horbachevsky Ternopil National Medical University, Ternopil, Ukraine.
- Department of Paediatrics, Clinique Notre Dame de Grâce, Gosselies, Belgium.
- Faculty of Public and One Health, University of Thessaly, Karditsa; and Department of Statistics, Athens University of Economics and Business, Athens, Greece.
- Division of Paediatric Rheumatology, Department of Paediatrics, Kocaeli University Faculty of Medicine, Kocaeli, Turkey.
- Department of Allergology, Rheumatology and Clinical Immunology, UCH, UMC, Faculty of Medicine, University of Ljubljana, Slovenia.
- Department of Rheumatology and Autoinflammatory Diseases, IRCCS Istituto Giannina Gaslini, Genoa, Italy.
- Department of Paediatrics, Hôpital Riviera-Chablais, Rennaz, Switzerland.
CER18307
Paediatric Rheumatology
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PMID: 40371538 [PubMed]
Received: 05/11/2024
Accepted : 05/03/2025
In Press: 07/05/2025
Abstract
OBJECTIVES:
We aimed to analyse the strategies of physicians regarding corticosteroid use in syndrome of undifferentiated recurrent fever (SURF) and examine the published data on this topic.
METHODS:
The JIR-CliPS questionnaire which addresses physicians’ practices about on demand corticosteroid use in SURF was distributed via e-mail to potential respondents. We systematically reviewed the MEDLINE and Scopus databases and extracted the data about on demand corticosteroid use in SURF.
RESULTS:
One hundred thirty-seven physicians (F/M=2.5; 66.4% paediatric rheumatologists) from 45 countries responded to the survey. Around 70% of physicians prescribe corticosteroids for SURF flares. Most physicians (81.9%) do not use corticosteroids in SURF patients routinely, and this practice is more common among less experienced physicians (p<0.001). Prednisolone at a dose of 1 mg/kg (54.4%) was the most commonly preferred corticosteroid. The most common definition of response to corticosteroids was “response within 12 hours” (51.6%). Most respondents (59.5%) consider changing treatment if corticosteroids cause a decrease in quality of life.
We found 10 articles in the literature describing 239 SURF patients treated with on demand corticosteroids. The most frequently preferred corticosteroid was prednisolone (63.8%). The response to corticosteroids was 70.8% and an increase in attack frequency was observed in almost 40% of patients.
CONCLUSIONS:
On demand corticosteroid use is not uncommon in the acute management of SURF attacks. However, most physicians do not use corticosteroids routinely and there is no consensus regarding the definition of response to treatment and when to change treatment neither in our survey results nor in the literature.