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 |