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Immune dysregulation is any proposed or confirmed breakdown or maladaptive change in molecular control of immune system processes. For example, dysregulation is a component in the pathogenesis of autoimmune diseases and some cancers .
The complement system is part of the innate as well as the adaptive immune system; it is a group of circulating proteins that can bind pathogens and form a membrane attack complex. Complement deficiencies are the result of a lack of any of these proteins. They may predispose to infections but also to autoimmune conditions. [7]
In healthy people, Tregs maintain immune homeostasis. [8] When there is a deleterious FOXP3 mutation, Tregs do not function properly and cause autoimmunity. [8] [9] IPEX onset usually happens in infancy. If left untreated, it is often fatal by the age of 2 or 3. [10] [11] A bone marrow transplant is generally considered the best treatment ...
PLAID syndrome is an inherited condition characterised by antibody deficiency and immune dysregulation, first described in 2012. The name is an acronym of "PLCG2-associated antibody deficiency and immune dysregulation". It is characterised by cold-induced urticaria, autoimmunity, atopy and humoral immune deficiency. [1]
An immune-mediated inflammatory disease (IMID) is any of a group of conditions or diseases that lack a definitive etiology, but which are characterized by common inflammatory pathways leading to inflammation, and which may result from, or be triggered by, a dysregulation of the normal immune response.
ICD-10 is the 10th revision of the International Classification of Diseases (ICD), a medical classification list by the World Health Organization (WHO). It contains codes for diseases, signs and symptoms, abnormal findings, complaints, social circumstances, and external causes of injury or diseases. [1]
Another example is the inability of the anti-inflammatory cytokines, such as IL-10, to signal through its receptor. That, again, can lead to systemic inflammation and severe inflammatory bowel disease (IBD). This shows that even single-cytokine dysregulation can cause autoinflammatory diseases.
An immune system disorder but not autoimmune. Idiopathic giant-cell myocarditis: No consistent evidence of autoimmune cause though the disease has been found comorbid with other autoimmune diseases. [124] Idiopathic pulmonary fibrosis: Autoantibodies: SFTPA1, SFTPA2, TERT, and TERC. IgA nephropathy: An immune system disorder but not an ...