| Syndrome |
Abbreviation |
Inheritance |
Gene locus / chromosome / protein |
Days of Fever |
Clinical presentation |
Secondary AA Amyloidosis |
| Familial Mediterranean Fever |
FMF |
Autosomal recessive |
MEFV / 16p13.3 / pyrin (marenostrin) |
1-3 |
Abdominal pain, eresypela-like rash, serositis, synovitis, arthralgias |
++- (*) |
| Periodic fever with aphthous stomatitis, pharyngitis, and cervical adenitis |
PFAPA |
Undefined |
Undefined |
3-4 |
Aphtous stomatitis, lymphoadenopathy (cervical), abdominal pain (mild) |
- |
| Hyperimmunoglobulin D syndrome |
HIDS |
Autosomal recessive |
MEVK / 12p / mevalonate kinase |
3-7 |
Lymphoadenopathy (cervical), abdominal pain, arthralgias, arthritis, splenomegaly, rash |
+-- |
| Tumor necrosis factor alpha receptor 1 associated syndrome |
TRAPS |
Autosomal dominant |
TNFR1 / 12p / tumor necrosis factor alpha receptor-1 |
Longer than 5 |
Abdominal pain, conjuntivitis, arthralgias,arthritis, myalgial (local), rash |
++- |
(*) see text for details