Distinguishing Clinical Features and Response to Therapy in AID.
Distinguishing Clinical Features and Response to Therapy in AID.
| FMF | TRAPS | MKD | CAPS | PFAPA | |
|---|---|---|---|---|---|
| Mutant Gene | MEFV | TNFRSF1A | MVK | NLRP3 | Unknown |
| Duration of Recurrent Fever | 1–3 days | 3 weeks | 4–7 days | FCAS, triggered by cold, usually 24 h; MWS, variable; NOMID, variable | Constant intervals (usually ~28 days); Median duration, 4 days |
| Abdominal Pain | Yes | Yes | Yes | Rarely | Sometimes |
| Pleurisy | Yes | Yes | No | No | No |
| Arthritis/Arthralgia | Yes | Yes | Yes | FCAS, no; MWS, NOMID, yes | Rare/absent |
| Skin | No | Yes | Yes | Yes | Rarely |
| Ocular | No | Yes | Yes | Yes | No |
| Responds to Steroids | No (with the exception of PFM) | Yes | Yes | Yes | Yes |
| Response to Colchicine | Yes | No | No | No | No/possible role in prevention |
| Treatment | Colchicine, anti-IL-1 | Steroids, anti-TNF, anti-IL1 | NSAIDs, steroids, anti-IL1 | Anti-IL1 | Steroids, tonsillectomy, anti-IL1 |
CAPS, cryopyrin-associated periodic fever syndrome; FCAS, familial cold autoinflammatory syndrome; FMF, familial Mediterranean fever; IL1, interleukin 1; MKD, mevalonate kinase deficiency; MWS, Muckle–Wells syndrome; NOMID, neonatal onset multisystem inflammatory disease; NSAIDs, non-steroidal anti-inflammatory drugs; PFAPA, periodic fever, aphthous stomatitis, pharyngitis, adenitis; PFM, protracted febrile myalgia; TNF, tumor necrosis factor; TRAPS, tumor necrosis factor-associated periodic fever syndrome.