Periodic fever and autoinflammatory syndromes
Recurrent fevers with high CRP and no infection. How attack length, triggers, ancestry and inheritance separate FMF, TRAPS, MKD, CAPS and PFAPA; colchicine for life in FMF and the 2024 EULAR/PReS update; IL-1 blockade; AA amyloidosis; and the adult-onset mimics (Schnitzler, VEXAS).
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Autoinflammatory diseases are disorders of innate immunity, mostly IL-1 driven through the inflammasome. There are no autoantibodies or autoreactive T cells.
- Suspect one in stereotyped attacks of fever with raised CRP, a symptom-free interval with normal CRP, negative cultures, and a pattern that repeats for at least 6 months.
- Most start in childhood. Adult physicians see FMF in people of Eastern Mediterranean ancestry (Armenian, Turkish, Arab, Sephardic Jewish, Greek, Lebanese), adult-onset mimics and AA amyloidosis as the late complication.
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Sources
- TGA prescription medicines registrations: Ilaris (canakinumab), extension of indication to TRAPS, HIDS/MKD and FMF, decision 15 January 2025AU/NZTherapeutic Goods Administration, 2025
- Ozen S, Sağ E, Oton T et al. EULAR/PReS endorsed recommendations for the management of familial Mediterranean fever (FMF): 2024 update. Ann Rheum Dis 2025;84:899-909EULAR and PReS, 2024 update (published 2025)
- Gattorno M et al. Classification criteria for autoinflammatory recurrent fevers (Eurofever/PRINTO). Ann Rheum Dis 2019;78:1025-32Eurofever and PRINTO, 2019
- De Benedetti F et al. Canakinumab for the treatment of autoinflammatory recurrent fever syndromes (CLUSTER). N Engl J Med 2018;378:1908-19New England Journal of Medicine, 2018
- Fautrel B et al. EULAR/PReS recommendations for the diagnosis and management of Still's disease, comprising systemic juvenile idiopathic arthritis and adult-onset Still's disease. Ann Rheum Dis 2024;83:1614-27EULAR and PReS, 2023 (published 2024)
- Beck DB et al. Somatic mutations in UBA1 and severe adult-onset autoinflammatory disease (VEXAS). N Engl J Med 2020;383:2628-38New England Journal of Medicine, 2020
- Therapeutic Guidelines: RheumatologyAU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Last updated
- 14 November 2026
What changed
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