Autoimmunity classifications

In view of the variety of possible immunological reactions directed towards the canaliculi or bile-duct epithelia and the difficulties involved in making a definitive histological diagnosis and classification, it would be feasible to include a so-called overlap syndrome or conversion syndrome in the hypothesis of autoimmune cholangitis. 

McFarlane, I.G. Definition and classification of autoimmune hepatitis. Semin. Liver Dis. 2002 22 317-324... 

Approximately 5% to 10% of aU cases of diabetes mellitus are in this category. Patients usually have abrupt onset of symptoms (e.g., polyuria, polydipsia, and rapid weight loss). Patients have insulinopenia (a deficiency of insulin) caused by loss of pancreatic islet (3-ceUs and are dependent on insulin to sustain life and prevent ketosis. Most patients have antibodies that identify an autoimmune process (see later discussion) some patients have no evidence of autoimmunity and are classified as type 1 idiopathic. The peak incidence is in childliood and adolescence. Approximately 75% acquire the disease before age 30 years, but the onset in the remainder may occur at any age. Age at presentation is not a criterion for classification. 

The heterogeneity of most of the systemic but also organ-specific autoimmune diseases is an additional important factor that complicates genetic analyses. Careful disease classification is necessary, and differentiation of subgroups according to clinical presentation, autoantibody production, ethnic background, as well as environmental exposures may be helpful. The risk associated with one genetic risk factor for an autoimmune disease may be... 

The least well characterized autoimmune polyglandular syndrome is the type 3 syndrome. This syndrome is defined by the presence of autoimmune thyroid disease with another autoimmune disease, such as diabetes mellitus type l, autoimmune gastritis, or myasthenia gravis, but in the absence of Addison disease. APGS type 3 primarily involves females (the female to male ratio is 7 1) who have HLA-DR3-associated autoimmune disease and is probably the most common of the autoimmune polyglandular syndrome disorders. Since different and multiple clinical combinations can be found, the classification of this type of autoimmune polyglandular syndrome is probably more complicated than originally anticipated. 

Sjogren syndrome. Chronic inflammatory autoimmune disease of the exocrine glands of unknown etiology. Its primary symptoms are keratoconjunctivitis sicca and xerostomia. Two types of Sjogren syndrome are distinguished a primary (isolated) type and a secondary type associated with another underlying autoimmune disease (e.g. rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, primary biliary cirrhosis, autoimmune hepatitis, multiple sclerosis, thyroiditis, autoimmune, etc.). Ro/SS-A and La/SS-B autoantibodies are used as classification criteria. 

Systemic lupus erythematosus (SLE). A chronic, remitting-relapsing inflammatory autoimmune disease affecting multiple organ systems, such as the skin, joints, serosal membranes, kidneys, blood cells, and central nervous system. The disease is very heterogeneous in clinical expression and serological factors. Autoantibodies directed against nuclear components ( - antinuclear antibodies) are typically detected. Anti-dsDNA, anti-Sm, and antiphospholipid antibodies are used as classification criteria. 

Autoimmunity. Autoimmune diseases are disorders of immune regulation in which several different factors (e.g. viral, genetic, hormonal, environmental) may each play a role. Autoimmune diseases may belong to any of the four Coombs and Cell classifications of hypersensitivity and include the production of autoantibodies, destructive inflammatory cell infiltrates in various organs, and deposition of immune complexes in vascular beds. Chemically induced autoimmunity may result from any of several possible mechanisms. These include the alteration or release of autoantigens, or the cross-reaction of the chemical with autoantigens, or alternatively a direct effect on the immune system via lymphocytes or macrophages (60). 

In conclusion, classical myxoedematous cretinism is found in the Qinghai endemia. Nevertheless, all cretins displayed degrees of neurological abnormality. In this endemia the traditional classification was not useful and the clinical syndrome of cretinism was modified by the severity and duration of ongoing hypothyroidism. The factors that influence postnatal thyroid function are unidentified, but the finding of thyroid atrophy implies an autoimmune process. 

In hemolytic anemia, the life span of the red cell is considerably reduced. Anemia in the newborn usually results from either the presence of an abnormal hemoglobin or the development of an autoimmune process. In the adult, hemolytic anemia may be the consequence of drug administration or be caused by an autoimmune reaction. A classification of hemolytic anemia has been presented, and a detailed description of the primary injury appears in several other chapters. 

Locht H, Pelck R, Manthorpe R. Clinical manifestations correlated to the prevalence of autoantibodies in a large (n = 321) cohort of patients with primary Sjogren s syndrome a comparison of patients initially diagnosed according to the Copenhagen classification criteria with the American-European consensus criteria. Autoimmun Rev 2005 4 276 81. 

Nguyen VT, Ndoye A, Bassler KD, et al. Classification, clinical manifestations, and immunopathological mechanisms of the epithelial variant of paraneoplastic autoimmune multiorgan syndrome a reappraisal of paraneoplastic pemphigus. Arch Dermatol 2001 137 193-206. 

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