Autoimmune oophoritis is a rare

autoimmune disease An autoimmune disease is a condition arising from an abnormal immune response to a functioning body part. At least 80 types of autoimmune diseases have been identified, with some evidence suggesting that there may be more than 100 types. Nearly a ...

where the body's own

immune system The immune system is a network of biological processes that protects an organism from diseases. It detects and responds to a wide variety of pathogens, from viruses to parasitic worms, as well as cancer cells and objects such as wood splinte ...

attacks the

ovaries The ovary is an organ in the female reproductive system that produces an ovum. When released, this travels down the fallopian tube into the uterus, where it may become fertilized by a sperm. There is an ovary () found on each side of the body. T ...

. This causes the ovaries to have inflammation,

atrophy Atrophy is the partial or complete wasting away of a part of the body. Causes of atrophy include mutations (which can destroy the gene to build up the organ), poor nourishment, poor circulation, loss of hormonal support, loss of nerve supply t ...

, and fibrosis. Such changes in the ovaries can cause them to not function properly. This disease is caused by primary ovarian insufficiency (POI), where reproduction and hormonal function of the ovaries stops before the age of 40.

Signs and symptoms

Autoimmune oophoritis can present with a wide variety of symptoms. It begins with the main symptom, which is amenorrhea, where there is an irregular or no menstrual period at all. Other symptoms are related to

ovarian cyst An ovarian cyst is a fluid-filled sac within the ovary. Often they cause no symptoms. Occasionally they may produce bloating, lower abdominal pain, or lower back pain. The majority of cysts are harmless. If the cyst either breaks open or causes ...

s, and more common ones are also listed below. A variation of symptoms can occur together, however, that depends on the person and the severity of the disease. *

Primary amenorrhea Amenorrhea is the absence of a menstrual period in a woman of reproductive age. Physiological states of amenorrhoea are seen, most commonly, during pregnancy and lactation (breastfeeding). Outside the reproductive years, there is absence of menses ...

**This amenorrhea is more specific to

menstruation Menstruation (also known as a period, among other colloquial terms) is the regular discharge of blood and mucosal tissue from the inner lining of the uterus through the vagina. The menstrual cycle is characterized by the rise and fall of hor ...

never occurring before. *

Secondary amenorrhea Amenorrhea is the absence of a menstrual period in a woman of reproductive age. Physiological states of amenorrhoea are seen, most commonly, during pregnancy and lactation (breastfeeding). Outside the reproductive years, there is absence of menses ...

**This type of amenorrhea is where menstruation occurred once puberty began but then suddenly stopped later on. *

Infertility Infertility is the inability of a person, animal or plant to reproduce by natural means. It is usually not the natural state of a healthy adult, except notably among certain eusocial species (mostly haplodiploid insects). It is the normal state ...

* Cramping *Bloating *Nausea *Vomiting * Sex hormone deficiency *

Hot flushes Hot flashes (also known as hot flushes) are a form of flushing, often caused by the changing hormone levels that are characteristic of menopause. They are typically experienced as a feeling of intense heat with sweating and rapid heartbeat, and ...

* Lower abdominal pain *

Fever Fever, also referred to as pyrexia, is defined as having a body temperature, temperature above the human body temperature, normal range due to an increase in the body's temperature Human body temperature#Fever, set point. There is not a single ...

Malaise As a medical term, malaise is a feeling of general discomfort, uneasiness or lack of wellbeing and often the first sign of an infection or other disease. The word has existed in French since at least the 12th century. The term is often used ...

* Vaginal discharge * Symptoms associated with other coexisting autoimmune conditions

Causes

The underlying cause of autoimmune oophoritis is yet unknown. However, it is known that it can co-exit with

lupus Lupus, technically known as systemic lupus erythematosus (SLE), is an autoimmune disease in which the body's immune system mistakenly attacks healthy tissue in many parts of the body. Symptoms vary among people and may be mild to severe. Comm ...

pernicious anemia Pernicious anemia is a type of vitamin B12 deficiency anemia, a disease in which not enough red blood cells are produced due to the malabsorption of vitamin B12. Malabsorption in pernicious anemia results from the lack or loss of intrinsic fa ...

myasthenia gravis Myasthenia gravis (MG) is a long-term neuromuscular junction disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, dro ...

, or other autoimmune conditions. Autoimmune oophoritis can also be associated with autoimmune endocrinopathy syndrome type I and type II. These conditions can coexists because they are all autoimmune diseases, this is called polyautoimmunity. There are some environmental causes for autoimmune diseases that have been discovered in different studies. Those consist of exposures to chemicals in the environment, such as vinyl chloride, metals, mycotoxins, and organic compounds.

Genetics

As Autoimmune oophoritis often occurs in the setting of autoimmune polyendocrine syndromes (APS), mutations in '' AIRE'' or ''HLA-DR3/4'' can contribute to its pathology.

Mechanism/ Pathophysiology

There is no set mechanism specifically for autoimmune oophoritis, but it is known that microscopic examinations reveal a picture of inflammatory infiltrate. Selectively attacking the developing follicles and

corpus luteum The corpus luteum (Latin for "yellow body"; plural corpora lutea) is a temporary endocrine structure in female ovaries involved in the production of relatively high levels of progesterone, and moderate levels of estradiol, and inhibin A. It is t ...

with sparing of the primordial follicles. However, since it is an autoimmune disease, it occurs when the immune system develops a response against one or more of the body's normal functions as if they were harmful. Then, the immune system fails to differentiate between "self" and "non-self", therefore it begins to develop antibodies. Antibodies target its own cells, tissues, and organs. Antibodies are made by the immune system as a response to an infection. They are produced by B cells, which are made in the bone marrow and found in the blood. Antibodies are Y-shaped which allows them to work properly because that is how their unique sites bind with a matching site on antigens. That is where they destroy them. Research has shown that Theca cells could be part of a potential mechanism but more research should be done to verify the results.

Diagnosis

Diagnosis includes a blood test that looks for anti-steroid or anti-ovarian

antibodies An antibody (Ab), also known as an immunoglobulin (Ig), is a large, Y-shaped protein used by the immune system to identify and neutralize foreign objects such as pathogenic bacteria and viruses. The antibody recognizes a unique molecule of the ...

in the bloodstream to confirm infertility in the female. Antibodies result as a response to an infection that the body has already fought. Anti-ovarian antibodies are found against the ovaries, they bind to the working sites of the ovaries. According to research anti-ovarian antibodies cause malfunctions in the ovulation process.

Pelvic ultrasound Medical ultrasound includes diagnostic techniques (mainly imaging techniques) using ultrasound, as well as therapeutic applications of ultrasound. In diagnosis, it is used to create an image of internal body structures such as tendons, musc ...

s are also done to look for enlarged cystic ovaries. In other cases biopsies may be required in order to confirm the diagnosis. Other type of tests could be done to rule out other issues that can be a part of primary ovarian insufficiency (POI).

Prevention/Treatment

There is no specific treatment for autoimmune oophoritis yet. Nor is there an immunosuppressive that has shown to be effective and safe. However, affected women are usually started on hormone replacement therapies, or corticosteroids to achieve immunosuppression. A few risk factors associated with the hormone replacement therapies are: breast cancer, endometrial cancer, and ovarian cancer. Women with this disease need a lot of emotional support and should maintain a management of other autoimmune conditions.

Prognosis

The prognosis is difficult to evaluate given the rarity of this condition, some patients with autoimmune oophoritis may have intermittent ovarian follicle function and may ovulate spontaneously. Allowing them to menstruate normally and even achieve pregnancy after therapy.

Epidemiology

Autoimmune oophoritis accounts for almost 4% of women who present with primary ovarian insufficiency (POI). However, due to the lack of awareness and systematic studies, the ethnicity related prevalence is not known yet. Since autoimmune oophoritis is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the

National Institutes of Health The National Institutes of Health, commonly referred to as NIH (with each letter pronounced individually), is the primary agency of the United States government responsible for biomedical and public health research. It was founded in the late ...

(NIH), it means that less than 200,000 women in the United States are affected by it.

Research

In 2015 a research was done on the role of autoimmunity in premature ovarian failure. In 2014 there was an ovarian autoimmune disease research that revealed at least two mechanisms that protect the ovary from an autoimmune attack. Research showed that Theca cells were targeting the autoimmune deficiency within the ovary. And in 2011, a research was done on a patient who suffered from

(MG) in conjunction with autoimmune oophoritis whose premature ovarian insufficiency was cured without hormonal therapy, only after

thymectomy A thymectomy is an operation to remove the thymus. It usually results in remission of myasthenia gravis with the help of medication including steroids. However, this remission may not be permanent. Thymectomy is indicated when thymoma are present ...

. The study suggests treatment of MG including thymectomy could cure ovarian failure and hormonal therapy may not be necessary.

References

External links

{{Medical resources , DiseasesDB = , ICD10 = , ICD9 = , MedlinePlus = , MeshID = Inflammatory diseases of female pelvic organs Autoimmune diseases