<unk>  (AIDs) are a group of rare disorders caused by a dysfunction of the innate immune system.They are characterised by a perdiodic or chronic systemic inflammation usually without the involvement of adaptive immunity. It is necessary to point out, that<unk>  are not autoimmune diseases. Both are characterised by an immune system malfunction which causes the symptoms, they share some of the symptoms, such as rash, swelling or fatigue, but the cardinal cause or mechanism of the diseases are different. Probably the most important difference is, that there is a malfunction of innate immune system in AIDs, while in autoimmune diseases there is a malfunction of adaptive immune system. It is also needed to be said, that in many conditions the boundaries between autoinflammation (overacitivy of the innate immunity), autoimmunity (overactivity of the adaptive immunity), and immunodeficiency (decreased activity of the innate or adaptive immunity) are fluid, and often they are crossed. The clinical phenotypes are driven by the cell type most affected by a particular mutation: Excessive activation of neutrophils, monocytes/macrophages, and dendritic cells leads to<unk>  symptoms; T cell and B cell dysfunction leads to autoimmunity. Failure of innate and/or adaptive immune cells to appropriately activate and recognize and clear infectious agents causes immunodeficiency and vulnerability to infections.
Autoinflammatory diseases
Immune-mediated inflammatory diseases
Central nervous system disease
Risk factors for tuberculosis
Skin manifestations of sarcoidosis
Cranial nerve disease
Oral manifestations of systemic disease
Autoimmune disease