Proposed mechanisms of autoimmunity in various diseases, autoimmune phenomena, and current treatment options
| Proposed mechanism of autoimmunity . | Diseases . | Autoimmune phenomena . | Treatment options . |
|---|---|---|---|
| Hypersensitivity to persisting viral antigens, especially EBV (T-lymphocyte mediated) | CHS, XLP, ? Omenn syndrome | Virus-associated HLH | Steroids, ATG, MTX, CyA, then BMT |
| Steroids, BMT | |||
| WAS | Vasculitis, arthritis | Steroids, CyA, BMT | |
| ? ALPS | Autoimmune cytopenias | ||
| Hypersensitivity to persisting parasitic infection (Cryptosporidium) (T-lymphocyte mediated) | CD40L deficiency, MHC II deficiency | Sclerosing cholangitis | BMT, liver transplantation |
| Hypersensitivity response to bacterial antigens, especially gut flora (neutrophil, NK-cell mediated) | CGD | Granulomatous inflammation of lung, gut, and liver | Antibiotics, steroids, interferon-γ, CyA, BMT |
| LAD, BLS-I | Leukocytoclastic vasculitis | Antibiotics, BMT (for LAD) | |
| Defective induction of T-cell tolerance in thymus (T-lymphocyte mediated) | APECED | Organ-specific autoimmunity | Alleviation of symptoms |
| Defective clearance of antibodies (immune complex mediated) | C1q, C1r, C1s, C4 deficiencies | SLE | Steroids, cyclophosphamide |
| Proposed mechanism of autoimmunity . | Diseases . | Autoimmune phenomena . | Treatment options . |
|---|---|---|---|
| Hypersensitivity to persisting viral antigens, especially EBV (T-lymphocyte mediated) | CHS, XLP, ? Omenn syndrome | Virus-associated HLH | Steroids, ATG, MTX, CyA, then BMT |
| Steroids, BMT | |||
| WAS | Vasculitis, arthritis | Steroids, CyA, BMT | |
| ? ALPS | Autoimmune cytopenias | ||
| Hypersensitivity to persisting parasitic infection (Cryptosporidium) (T-lymphocyte mediated) | CD40L deficiency, MHC II deficiency | Sclerosing cholangitis | BMT, liver transplantation |
| Hypersensitivity response to bacterial antigens, especially gut flora (neutrophil, NK-cell mediated) | CGD | Granulomatous inflammation of lung, gut, and liver | Antibiotics, steroids, interferon-γ, CyA, BMT |
| LAD, BLS-I | Leukocytoclastic vasculitis | Antibiotics, BMT (for LAD) | |
| Defective induction of T-cell tolerance in thymus (T-lymphocyte mediated) | APECED | Organ-specific autoimmunity | Alleviation of symptoms |
| Defective clearance of antibodies (immune complex mediated) | C1q, C1r, C1s, C4 deficiencies | SLE | Steroids, cyclophosphamide |
EBV indicates Epstein-Barr virus; HLH, hemophagocytic lymphohistiocytosis; ATG, antithymocyte globulin; MTX, methotrexate; CyA, cyclosporine; BMT, bone marrow transplantation; NK, natural killer; BLS-I, bare lymphocyte syndrome type I; and SLE, systemic lupus erythematosus. For explanation of other abbreviations, see Table1.