Table 3 The differences between autoinflammatory and autoimmune diseases

Factors determining disease manifestations

Innate immune activation

Local tissue factors at disease-prone sites, including tissue trauma, necrosis, mechanical factors, bacteria or their constituent molecules

Adaptive immune activation

Clinical disease expression determined by events taking place in primary lymphoid tissues, including bone marrow, the thymus, lymph nodes and spleen

Key theory relating to disease expression

Various danger signals with tissue-specific factors determining disease localization

Breakdown of immunological tolerance and aberrant self–non-self-discrimination

Immunological basis

Genetically related to perturbations of innate immune function, including proinflammatory cytokine signaling/bacterial sensing/local tissue abnormalities

Acquired immune perturbation is key to disease expression: infiltration of Rheumatoid arthritis synovium, proliferation of macrophage-like synoviocytes and fibroblast-like synoviocytes, and infiltration of inflammatory cells, including B and T lymphocytes, and dendritic cells

Cellular basis

Expression determined by cells of the innate immune system, including neutrophils and macrophages or non-immune cells

An antigen-independent induction phase–expression is mainly determined by factors affecting B- and T cell activity

Genetic basis

Cytokine and sensing bacterial pathways

MHC class II association and adaptive response gene

Therapy

Anti-cytokine(Il-1, IL-6, TNF )

Anti-B and T cell

Classification

Monogenic: hereditary periodic fever

Polygenic: as Crohn’s diseases, spondyloarthropathies

Monogenic: immune dysregulation polyendocrinopathy enteropathy x-linked syndrome

Polygenic: rheumatoid arthritis, systematic lupus erythematosus