Version July 2025
| Disease | Inheritance | Chromosome | Gene¶ | Number of variants¶ | Age of onset | Duration of attacks | Phenotypes | Percent AA amyloidΔ | Treatment◊ |
| FMF | R§ | 16 | MEFV | 390 | <10 years | 1 to 3 days | F; S; A; E | Up to 60% | Colchicine; IL-1/6 antagonists |
| TRAPS¥ | D | 12 | TNFRSF1A | 180 | <10 years | >7 days | F; R; S; A; M; P | 11 to 25% | Glucocorticoids; IL-1 antagonists |
| CAPS‡ | D | NLRP3† | 256 | ||||||
| MWS | D | NLRP3 | <20 years | 1 to 2 days** | U; D | Approximately 25% | IL-1 antagonists | ||
| FCAS | D | NLRP3 | <6 months | 1 to 2 days** | CU | 2 to 4% | |||
| CINCA/NOMID | D | NLRP3 | <6 months | A; U; D; Sk; Me | Approximately 20% | ||||
| HIDS/MKD | R | 12 | MVK | 276 | <6 months | 4 to 7 days | F; S; A; R; AS | Approximately 6% | IL-1/6 antagonists |
A: arthropathy; AS: aphthous stomatitis; CAPS: cryopyrin-associated periodic syndromes; CINCA/NOMID: chronic infantile neurologic cutaneous and articular syndrome/neonatal-onset multisystem inflammatory disease; CU: cold urticaria (starts approximately 10 hours after cold exposure); D: sensorineural deafness; E: erysipelas-like erythema; F: fever; FCAS: familial cold autoinflammatory syndrome; FMF: familial Mediterranean fever; HIDS/MKD: hyperimmunoglobulin D with periodic fever syndrome/mevalonate kinase deficiency; IL: interleukin; M: myalgias; Me: chronic meningitis; MWS: Muckle-Wells syndrome; P: periorbital edema; R: rash; S: serositis; Sk: skeletal abnormalities; TRAPS: tumor necrosis factor receptor-1 associated periodic syndrome; U: urticaria.
* Isolated cases of AA amyloid complicating adenosine deaminase 2 deficiency (DADA2; 140 variants) and A20 haploinsufficiency (HA20; 66 variants) have been reported[1].
¶ Number of variants listed in https://infevers.umai-montpellier.fr/web/ as of June 2022; not all are pathogenic; some are associated with increased frequency of AA amyloidosis.
Δ Percent developing AA amyloid if untreated.
◊ Treatment modalities: IL-1 antagonists: anakinra, canakinumab, rilonacept; IL-6 antagonists: tocilizumab; sarilumab.
§ May be dominant or heterozygous.
¥ TRAPS is also described as "Hibernian Fever."
‡ The cryopyrinopathies (CAPS) encompass a spectrum of disorders, all associated with mutations in NLRP3, of which 256 variants have been described to date. They include MWS, FCAS, CINCA, and NOMID. A few patients with an FCAS-like phenotype have been described with NLRP12 mutations (80 variants).
† Mosaicism has been identified in patients with late-onset, mild, or otherwise atypical autoinflammatory diseases in the spectrum of CAPS and TRAPS.
** May be episodic or daily.