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Ovary - nontumor

Inflammatory disorders - noninfectious

Autoimmune oophoritis


Reviewer: Mohiedean Ghofrani, M.D., Southwest Washington Medical Center (see Reviewers page)
Revised: 25 August 2011, last major update August 2010
Copyright: (c) 2002-2011, PathologyOutlines.com, Inc.

Description
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Primary ovarian insufficiency in a woman with autoimmune features, including:
- Serum adrenal cortical or steroid cell antibodies (StCA) or serum antibodies to adrenal or ovarian steroidogenic enzymes (21OHAb, 17OHAb, P450sccAb) identified by indirect immunofluorescence
- Associated with polymorphisms of genes influencing the function of the immune system, i.e. HLA complex
- Selective mononuclear cell infiltration into the theca layer of developing follicles of the ovary
- Association with other autoimmune diseases such as Addison disease, autoimmune thyroiditis and type I diabetes mellitus

Epidemiology
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● If normal karyotype, primary ovarian insufficiency occurs in 1 of 1,000 women before age 30 and 1 of 10,000 before age 20
● Up to 30% of cases of primary ovarian insufficiency are suggested to be autoimmune in origin, but documented ovarian autoimmune reaction is demonstrated in only 45% of women with ovarian insufficiency

Etiology
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● Selective mononuclear cell infiltration into the theca layer of large, antral follicles, with earlier stage follicles consistently free of lymphocytic infiltration
● Destruction of theca cells with preservation of granulosa cells (that produce low amounts of estradiol because of lack of substrates) results in a subsequent increase in FSH levels, which stimulates viable granulosa cells to produce increased amounts of inhibins (J Clin Endocrinol Metab 2009; 94:3816)

Clinical features
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● Secondary amenorrhea (rarely primary) with associated infertility
● Usually associated with other autoimmune diseases
● In teenagers, associated with autoimmune polyendocrine syndrome type I or II (Ann N Y Acad Sci 2008;1135:118)

Laboratory
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● Low estradiol, high FSH
● Increased concentrations of serum inhibins compared to healthy fertile women and women with other forms of ovarian insufficiency (J Clin Endocrinol Metab 2008; 93:1263)
● Histologic evidence of autoimmune oophoritis is only seen in women positive for ovarian or adrenal autoantibodies; therefore, there is no indication to perform an ovarian biopsy solely to confirm an ovarian autoimmunity disorder

Case reports
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● Mother and daughter with nonmosaic Turner's syndrome (Fertil Steril 2004;82:923)

Treatment
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● Hormone replacement therapy
● Steroids are not standard treatment
● Plans for clinical trials of immunotherapy aimed at preserving the residual functional tissue or delaying the progression of the destructive ovarian autoimmune process
● Future techniques of in vitro folliculogenesis in which a portion of primordial and primary follicles are preserved

Gross description (Macroscopy)
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● Cystic ovaries

Micro description (Histopathology)
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● Lymphocytes and plasma cells infiltrate developing follicles and corpora lutea but not primordial follicles
● Preservation of granulosa cells

Micro images
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Lymphocytic oophoritis

Differential diagnosis
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● Other causes of primary ovarian insufficiency

Additional references
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Curr Opin Obstet Gynecol 2010;22:277; Fertil Steril 2005;84:958, Johns Hopkins

End of Ovary - nontumor > Inflammatory disorders - noninfectious > Autoimmune oophoritis


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