title: Autoimmune primary ovarian insufficiency.
abstract: Primary ovarian insufficiency (POI) is defined as sustained amenorrhea, increased follicle-stimulating hormone and low estrogen levels, whereas diminished ovarian reserve (DOR) is characterized as regular menses and alterations of ovarian reserve tests. POI of autoimmune origin may be associated with adrenal autoimmunity, non-adrenal autoimmunity or isolated. This autoimmune disease is characterized by serum ovarian, adrenocortical or steroidogenic cell autoantibodies. POI of adrenal autoimmune origin is the most frequent type observed in 60-80% of patients. Clinically, amenorrhea is the hallmark of POI, however before menstruation stops completely, irregular cycles occur. Infertility, hot flushes, vaginal atrophy, and dyspareunia are also common. Autoimmune oophoritis is characterized by mononuclear inflammatory cell infiltrate in the theca cells of growing follicles, with early stage follicles without lymphocytic infiltration. This infiltrate includes plasma, B and T-cells. A novel classification criterion for autoimmune POI/DOR is proposed subdividing in three distinct categories (possible, probable and confirmed) according to autoantibodies, autoimmune disease and ovarian histology. Unfortunately, up to date guidelines for the treatment of autoimmune oophoritis are not available. Strategies to POI treatment include hormone replacement and infertility therapy. Assisted conception with donated oocytes has been proven to achieve pregnancy by intra cytoplasmic sperm injection in POI women.
authors.affiliation: Pediatric Rheumatology Unit, Department of Pediatric, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil. Electronic address: clovis.silva@hc.fm.usp.br.
authors.forename: C A
authors.initials: CA
authors.lastname: Silva
authors.affiliation: Division of Gynecology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: L Y S
authors.initials: LY
authors.lastname: Yamakami
authors.affiliation: Pediatric Rheumatology Unit, Department of Pediatric, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: N E
authors.initials: NE
authors.lastname: Aikawa
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil; Division of Rheumatology, Faculdade de Medicina da Universidade Federal de Pelotas, Rio Grande do Sul, Brazil.
authors.forename: D B
authors.initials: DB
authors.lastname: Araujo
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade da Bahia, Bahia, Brazil.
authors.forename: J F
authors.initials: JF
authors.lastname: Carvalho
authors.affiliation: Division of Rheumatology, Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil.
authors.forename: E
authors.initials: E
authors.lastname: Bonfá
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000097:blood / Q000276:immunology; D015551:Autoimmunity / Q000276:immunology; D001402:B-Lymphocytes / Q000276:immunology; D005260:Female; D006801:Humans; D007246:Infertility / Q000276:immunology / Q000473:pathology; D016649:Primary Ovarian Insufficiency / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology / Q000276:immunology*; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D001323:Autoantibodies
keywords: Anti-Müllerian hormone; Autoimmune oophoritis; Autoimmune primary ovarian insufficiency; Fertility; Ovarian reserve; Premature ovarian failure