On demand corticosteroid use in the syndrome of undifferentiated recurrent fever: a literature review and results from JIR-CLiPS survey study.

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.