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| | Familial Mediterranean fever | Mevalonate kinase deficiency syndrome | Tumor necrosis factor receptor-associated periodic syndrome | Cryopyrin-associated periodic syndrome |
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| Onset | Childhood or adolescence | Infancy (first year of life) | 3–20 years | Neonatal period-childhood |
| Usual ethnicity | Armenian, nonsephardic Jews, Arab, Turkish people | Dutch and other Northern European populations | Firstly recognized in Northern European (Ireland and Scotland) people; any ethnicity | Panethnic |
| Fever duration | 1–4 days | 3–7 days | 1 or even 3-4 weeks, usually responding to corticosteroids | Subcontinuous/variable with circadian periodism and intermittent flares |
| Abdominal distress | Very common (in the form of sterile peritonitis) | Very common (abdominal pain, vomiting, diarrhea) | Common (abdominal pain, diarrhea, constipation) | Uncommon |
| Chest involvement | Pleurisy, often unilateral | Infrequent | Pleuritis | Absent |
| Skin involvement | Erysipelas-like rash on feet/ankles | Polymorphic rash, erythema elevatum diutinum, disseminated superficial actinic porokeratosis | Painful migratory eruption, edematous plaques | Neutrophilic urticaria-like skin eruption of variable severity and extension (either induced by cold exposure or constant) |
| Osteoarticular involvement | Arthralgias or arthritides | Arthralgias | Migratory arthralgias, nonerosive arthritides | Arthralgias of variable severity, deforming osteoarthritis involving large joints and contiguous bones (in CINCA syndrome) |
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