Table 1.

Shared Features of Autoinflammatory, HLA-B27 Associated, and Autoimmune Diseases

Feature	Autoinflammatory Diseases	HLA-B27 Associated Diseases	Autoimmune Diseases
Age of onset	Very young	Young	Varies
Sex	F = M	M > F	F > M
Fevers	Prominent feature	Rare	Rare
Disease course	Flares and remits	Flares and remits	Continuous and progressive
Ocular findings	Uveitis, conjunctivitis	Uveitis, conjunctivitis	Varied, scleritis, episcleritis, uveitis less common
Sacroiliitis	Present but not common	Prominent feature	Rare
Acute phase reactants	Greatly elevated	Typically normal	Elevated
Presence of autoantibodies	No	No	Yes
Histology	Neutrophils	Neutrophils and mixed inflammatory cells	Mixed inflammatory cells
Genetics	Typically monogenic mutations in innate pathways	Polygenic: MHC-I, ERAP1, IL-23/IL-17	Polygenic: MHC-II, PTPN22, CTLA4, TNFAIP3
Effective Treatments	Cytokine blockade, not B-cell blockade, not corticosteroids	Cytokine blockade, not B-cell blockade, not corticosteroids	Cytokine blockade, B-cell blockade, corticosteroids