Type I Autoimmune Polyendocrinopathy

Thymocytes undergo four major processes before they mature. Thymic cells enter the thymus and cause positive selection of double positive thymocytes within the cortex, positive selection of thymocytes within the cortex, and negative selection of thymocytes within the medulla, and production of mature T cells from the thymus to the periphery. The negative selection of thymocytes depends on the expression of autoantigens in thymic medullary epithelial cells (MEC). Autoimmune regulatory factor (AIRE) is a transcription factor that initiates the expression of these autoantigens in MEC, so defects in the AIRE protein can lead to autoimmunity (Metzger and Anderson, 2011).

Caforio et al. (1991) reported 17% of organ-specific anti-cardiac autoantibodies in the sera of patients with autoimmune multioenocarcinosis, evidence of no evident echocardiography, and ECG and / or cardiac dysfunction (Table 2.2) . However, in antibody positive patients, higher frequency of systemic arterial hypertension was seen. The authors conclude that antibodies may represent markers of autoimmune hypertension, but prospective studies are necessary to confirm their hypothesis.

Autoimmune destruction of pancreatic β cells can be a small factor in type 2 diabetic patients and is called adult latent autoimmune diabetes syndrome. This group may occupy up to 10% of Scandinavian type 2 diabetic patients and has been confirmed in recent UK studies, but is not well characterized by other populations (4-6, 30 ). In conclusion, the first phase of insulin secretion has some diagnostic significance, but it does not seem to play a role in the pathogenesis of type 2 diabetes. Depending on patients with early-onset type 2 diabetes (perhaps up to 20%) (4, 5) there may be insufficient insulin secretion. This may be due to autoimmune destruction of beta cells, not by glucokinase genes. Missing

Diabetes mellitus is similar but not identical to human type I (insulin deficiency): This disease occurs in middle-aged Samoyed dogs and the average age at diagnosis is 7 years. Cause is chronic inflammation of the pancreas and / or autoimmune destruction of beta cells of the islet of Langerhans. Furthermore, the autoantibody observed in the affected dog was insulin. Several genetic markers are discussed as possible causes. Progressive retinal atrophy (PRA) caused by frameshift mutations at the RPRG locus on the X chromosome This disease retards visual acuity and ultimately causes blindness. Early symptoms appear between 2 and 5 years old. This disease corresponds to PRA 3 type linked to human X.