Ovary - nontumor
Inflammatory disorders - noninfectious
Autoimmune oophoritis

Author: Mohiedean Ghofrani, M.D. (see Authors page)

Revised: 15 September 2017, last major update August 2010

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PubMed Search: Autoimmune oophoritis

Cite this page: Ghofrani, M. Autoimmune oophoritis. PathologyOutlines.com website. http://pathologyoutlines.com/topic/ovarynontumorautoimmuneoophoritis.html. Accessed September 21st, 2017.
Definition / general
  • 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
  • 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
  • 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
  • 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
  • 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
Treatment
  • 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
  • Cystic ovaries
Microscopic (histologic) description
  • Lymphocytes and plasma cells infiltrate developing follicles and corpora lutea but not primordial follicles
  • Preservation of granulosa cells
Microscopic (histologic) images

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Lymphocytic oophoritis

Differential diagnosis
  • Other causes of primary ovarian insufficiency