Table 4 The main differences between autoimmune, monogenic autoinflammatory disorders and (major histocompatibility complex)-I associated disorders [20]
Variable | Autoimmune diseases | Mono genic autoinflammatory diseases | MHC-1- opathy |
|---|---|---|---|
Epidemiology | Common | Rare | Rare |
Gender | Female predominance | None | Diseases dependent |
Age of onset | Diseases dependent | Generally young | Generally young |
Primary site of disease | Lymphoid organ | Tissue target | Tissue target |
Immunopathogenesis | Predominant Adaptive system impairment | Predominant Innate system impairment | Innate system dysregulation with secondary MHC-I /ERAP-1 associated IL23/17 cytokine axis dysregulation |
Main cellular involvement | B and T cells | Neutrophils , Macrophages | Myeloid cell Innate lymphocyte CD8 T cell |
Genetic predisposition | MHC-II associations | Cytokine and bacterial sensing pathways | MHC-1 ERAP-1/2 IL-23/IL-17 axis |
Therapy | B cell depletion DMARDs | Anti-cytokines as (anti-IL-1} | Anti-cytokines IL-23/IL-17 but not B cell depletion |
Natural history | Progressive | Recurrent | Waxing and waning |