Clinical features and diagnosis of autoimmune primary ovarian insufficiency (premature ovarian failure)
- Lawrence M Nelson, MD
Lawrence M Nelson, MD
- National Institutes of Health
- Section Editors
- Robert L Barbieri, MD
Robert L Barbieri, MD
- Editor-in-Chief — Obstetrics, Gynecology and Women's Health
- Section Editor — General Gynecology and Female Reproductive Endocrinology
- Kate Macy Ladd Professor of Obstetrics, Gynecology and Reproductive Biology
- Harvard Medical School
- William F Crowley, Jr, MD
William F Crowley, Jr, MD
- Section Editor — Female Reproductive Endocrinology
- Daniel K Podolsky Professor of Medicine
- Harvard Medical School
46,XX primary ovarian insufficiency (POI) is defined as the development of primary hypogonadism before the age of 40 years in women who have a normal karyotype. The presenting symptoms are similar to those of menopause. The condition was previously referred to as “premature menopause” and “premature ovarian failure.” The age-specific incidence of spontaneous POI is approximately 1 in 250 by age 35 years and 1 in 100 by age 40 years.
Autoimmune POI due to autoimmune oophoritis is one of the rare causes of POI.
This topic review will discuss the pathogenesis, diagnosis, and treatment of autoimmune POI. An overview of the evaluation and treatment of spontaneous POI are reviewed separately. (See "Clinical manifestations and evaluation of spontaneous primary ovarian insufficiency (premature ovarian failure)" and "Management of spontaneous primary ovarian insufficiency (premature ovarian failure)".)
Autoimmune oophoritis is found in approximately 4 percent of women who present with spontaneous primary ovarian insufficiency (POI) . It was first confirmed histologically in a woman with coexisting autoimmune adrenal insufficiency  and subsequently in additional series of women with similar presentations. There is strong histologic evidence that POI, when it occurs in association with adrenal autoimmunity, is autoimmune-mediated ovarian insufficiency. However, there is only circumstantial evidence to suggest that autoimmune POI occurs in the absence of steroid cell autoimmunity .
Autoimmune oophoritis may occur as part of type I and type II syndromes of polyglandular autoimmune failure, which are associated with autoantibodies to multiple endocrine and other organs (table 1). (See "Causes of primary adrenal insufficiency (Addison's disease)", section on 'Polyglandular autoimmune syndrome type 1' and "Causes of primary adrenal insufficiency (Addison's disease)", section on 'Polyglandular autoimmune syndrome type 2'.)
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