Corticosteroid use in PFAPA syndrome: clinical practice data from the Juvenile Inflammatory Rheumatism Clinical Practice Strategies (JIR-CliPS) Survey Study and a comprehensive literature review
Authors' conclusion
Physicians frequently use CS for PFAPA in their routine clinical practice. Regarding treatment modification, the quality of life was a prominent consideration for physicians.
Study context
- Population
- Physicians from 47 countries managing PFAPA syndrome (67.4% paediatric rheumatologists); published PFAPA patients treated with CS
- Participants
- 144 physicians47 countries
- Outcome
- Physicians' CS usage strategies in PFAPA syndrome
Methodological detailsinternational survey, systematic literature search (MEDLINE/PubMed, Scopus)
- International survey
- Systematic literature search (MEDLINE/PubMed, Scopus)
Main finding
As reported in the abstract
CS use in PFAPA flares (survey)
- Prescribe CS in PFAPA flares
- 133 (92.4%)
- Most frequently prescribed CS: prednisolone
- 63.2%
- Response defined as 'response within 12 h'
- 61 (42.4%)
- Consider another treatment if CS-related increase in attack frequency lowers quality of life
- 57.9%
- Routinely prescribe CS; more frequent among more experienced physicians (P < 0.001)
- 44 (30.6%)
Observational data: associations, not effects.
Further findings
Literature review: 46 articles, 4564 PFAPA patients treated with CS
- Prednisone most frequently preferred CS
- 48.2%
- Response to CS
- around 95%
- Increase in attack frequency
- almost 35%
Original publication
Corticosteroid use in PFAPA syndrome: clinical practice data from the JIR-CliPS Survey Study and a comprehensive literature review. Rheumatology (Oxford). Published 10 February 2025. doi:10.
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