From: Autoinflammatory diseases: a possible cause of thrombosis?
Monogenic disease | Pathogenesis |
---|---|
Periodic fever diseases | Â |
Familial Mediterranean fever, FMF | Defects of IL-1Î’ family regulation |
Hypepr IgD Syndrome, HIDS | Defects of IL-1Î’ family regulation |
TNF-receptor-associated periodic syndrome, TRAPS | Protein mis-folding |
Familial cold autoinflammatory syndrome 2, FCAS2 | NFk-B activation |
Cryopirin-associated periodic syndromes | Defects of IL-1Î’ family regulation |
Diseases with pyogenic lesions | Â |
Deficiency of IL-1 receptor antagonist, DIRA | Defects of IL-1Î’ family regulation |
Pyogenic arthritis, pyoderma gangrenosum and acne syndrome, PAPA | Defects of IL-1Î’ family regulation |
Majeed syndrome | Defects of IL-1Î’ family regulation |
Diseases with granulomatous lesions | Â |
Blau syndrome | NFk-B activation |
Diseases with psoriasis | Â |
Deficiency of IL-36 receptor antagonist | Altered IL-36 regulation |
Diseases with panniculitis-induced lipodistrophy | Â |
Joint contractures, muscle atrophy and panniculitis-induced lipodistrophy syndrome, JMP | Disease linked to the proteasome and/or IFN-γ |
Chronic atypical neutrophilic dermatosis with lipodistrophy and elevated temperature, CANDLE | Disease linked to the proteasome and/or IFN-γ |
Nakajo-Nishimura syndrome, NNS | Disease linked to the proteasome and/or IFN-γ |
Others | Â |
PLCγ2-associated antibody deficiency and immune dysregulation syndrome, APLAID | Alteration of inflammation signalling and immune response |
Poligenic diseases | Â |
Gout | IL-1B activation |
Schnitzler syndrome | IL-1B activation |
Behçet disease | Spontaneous and/or induced overexpression of pro-inflammatory Th1 type cytokines – HLA-B51 |
Systemic-onset juvenile idiopathic arthritis (JIA) | IL-1B activation |
Crohn’s disease | Altered innate and adaptive immune system (Th1 and Th17 mediated process) |
Aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome | Altered innate and adaptive immune system (IL-1B–18 activation and Th1 chemokines induction) |