| Disease | Gene locus | Protein | Inheritance | Clinical features | Treatment |
| FMF [22, 23] | MEFV 16p13.3 | Pyrin | AR | Fever, serositis, arthralgias or arthritides, erysipelas-like eruption on the legs, responsiveness to colchicine prophylaxis, amyloidosis in untreated or noncompliant patients | Colchicine, anakinra, canakinumab |
| TRAPS [24–30] | 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, risk of amyloidosis | Corticosteroids, etanercept, anakinra, canakinumab, tocilizumab |
| MKD [31, 32] | MVK 12q24 | Mevalonate kinase | AR | Fever, widespread polymorphous rash, arthralgias, abdominal pain, diarrhea, lymph node enlargement, oral aphthosis | NSAIDs, anakinra, corticosteroids |
| CAPS [33, 34] | FCAS | NLRP3 1q44 | Cryopyrin | AD | Fever, cold-induced urticaria-like rash, conjunctivitis, arthralgias, fatigue |
Anakinra, canakinumab, rilonacept | MWS | Fever, urticaria-like rash, conjunctivitis, arthralgias, neurosensorial deafness, risk of amyloidosis | CINCAs | Fever, urticaria-like rash, uveitis, papilledema, deforming arthritis involving large joints, neurosensorial deafness, aseptic chronic meningopathy, risk of amyloidosis |
| PAPAs [35–37] | PSTPIP1 15q24-q25.1 | CD2BP1 | AD | Pauciarticular pyogenic arthritis, osteocartilaginous erosions of joints, cystic acne, pyogenic abscesses | Infliximab, anakinra |
| MAJEEDs [38–40] | LPIN2 18p11.31 | Lipin 2 | AR | Recurrent multifocal osteomyelitis, congenital dyserythropoietic anemia, chronic dermatosis resembling Sweet’s syndrome | NSAIDs, corticosteroids, anakinra, canakinumab |
| BS [41–43] | NOD2 (CARD15) 16q12.1-13 | Nod2 (Card15) | AD | Intermittent fevers, granulomatous dermatitis with ichthyosis-like changes, symmetrical granulomatous polyarthritis, recurrent severe granulomatous panuveitis | Corticosteroids, immunosuppressive agents, anti-TNF-α drugs, anakinra |
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