| |
Autoinflammatory disease |
Autoimmune disease |
| Pathogenetic basis |
Innate immunity |
Adaptive immunity |
| Competent cells |
Dendritic cells, Monocytes/Macrophages,
Neutrophils |
CD4+T cells - Th1/Th2/Th17/Treg, CD8+T cells, Macrophages, B cells |
| Activators |
PAMPs/DAMPs |
Antigens |
| Receptors |
TLR/MDA5/RIG-I/NLR |
TCR |
| Reaction specificity |
Comprehensive/Pattern recognition |
MHC-restricted/Antigen-specific |
| Gene products |
Type I interferon |
Antibodies Inflammatory cytokines |
| Disease due to gene mutation |
Congenital innate immunodeficiency (MyD88 deficiency, NEMO, DIRA, IkBα deficiency, IRAK-4 deficiency, etc.) |
Primary immunodeficiency diseases (CVID, agamma globulinemia, WAS, SCID, HIDS, HIES, CGD, etc.) |
| Representative |
Autoinflammatory diseases
Periodic fever syndrome ( FMF, PFAPA,TRAPS, MVK, CAPS), Systemic-onset JIA Juvenile dematomyositis, Chronic recurrent multifocal osteomyelitis, Behcet's disease, etc. |
Autoimmune diseases
Systemic lupus erythematosus, Mixed connective tissue disease, Kawasaki disease, Sjogren syndrome, Juvenile systemic sclerosis, etc. |
| Therapy |
Removal of inflammatory cytokines,
Suppression of activated macrophages/Dendritic cells |
Immunosuppressive therapy, Depletion of B cells |
Abbreviations: PAMPs: Pathogen-associated molecular patterns; DAMPs:
Damage-associated molecular patterns; TLR: Toll-like receptor; MDA5: Melanoma
differentiation-associated gene-5; RIG-I: Retinoic acid-inducible gene-I; NLR:
Nod-like receptor; NEMO: NF-kB essential modulator; DIRA: Deficiency of the
interleukin-1 receptor antagonist; FMF: Familial Mediterranean fever; PFAPA:
Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis; TRAPS:
Tumor necrosis factor receptor-associated periodic syndrome; MVK: Mevalonate
kinase deficiency; CAPS: Cryopyrin-associated periodic syndrome; JIA: Juvenile
idiopathic arthritis; TCR: T cell receptor; CVID: Common variable immunodeficiency;
WAS: Wiscott-Aldrich syndrome; SCID: Severe combined immunodeficiency;
HIES: Hyper-immunoglobulin E syndrome; CGD: Chronic granulomatous disease. |