| Disease | Gene locus | Protein | Inheritance | Clinical features |
|
FMF | MEFV 16p13.3 | Pyrin | AR | Fever, serositis, arthralgias or arthritides, erysipelas-like eruption on the legs, responsiveness to colchicine prophylaxis, and amyloidosis in untreated or noncompliant patients |
| TRAPS | TNFRSF1A 12p13 | p55 tumor necrosis factor receptor type-1 | AD | Fever, severe migrating muscle and joint involvement, conjunctivitis, periorbital edema, arthralgias or arthritis, sacroiliitis, serosal involvement, steroid responsiveness of febrile attacks, and risk of amyloidosis |
| MKD | MVK 12q24 | Mevalonate kinase | AR | Fever, widespread polymorphous rash, arthralgias, abdominal pain, diarrhea, lymph node enlargement, and oral aphthosis |
| CAPS | | | | | FCAS | NLRP3 1q44 |
Cryopyrin |
AD | Fever, cold-induced urticaria-like rash, conjunctivitis, arthralgias, and fatigue | MWS | Fever, urticaria-like rash, conjunctivitis, arthralgias, neurosensory deafness, and risk of amyloidosis | CINCAs | Fever, urticaria-like rash, uveitis, papilledema, deforming arthritis involving large joints, neurosensory deafness, aseptic chronic meningopathy and hydrocephalus, and risk of amyloidosis |
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AD: autosomal dominant, AR: autosomal recessive, CAPS: cryopyrin-associated periodic syndromes, CINCAs: chronic inflammatory neurological cutaneous articular syndrome, FCAS: familial cold autoinflammatory syndrome, FMF: familial Mediterranean fever, MKD: mevalonate kinase deficiency syndrome, MWS: Muckle-Wells syndrome, and TRAPS: tumor necrosis factor receptor-associated periodic syndrome.
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