Autoimmune hypophysitis - Revision history

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			{{Infobox medical condition
			\| name = Autoimmune hypophysitis
			\| synonyms = Lymphocytic hypophysitis
			\| field = [[Endocrinology]]
			\| symptoms = [[Headache]], [[visual field defects]], [[hormonal deficiencies]]
			\| complications = [[Adrenal insufficiency]], [[hypothyroidism]], [[diabetes insipidus]]
			\| onset = Typically in [[pregnancy]] or [[postpartum]]
			\| duration = Chronic
			\| causes = [[Autoimmune disease]]
			\| risks = [[Pregnancy]], [[female sex]]
			\| diagnosis = [[Magnetic resonance imaging\|MRI]], [[hormonal assay]]
			\| differential = [[Pituitary adenoma]], [[Rathke's cleft cyst]], [[sarcoidosis]]
			\| treatment = [[Glucocorticoids]], [[immunosuppressive therapy]], [[surgery]]
			\| prognosis = Variable, depends on response to treatment
			\| frequency = Rare
			}}
	'''Other Names:''' LYH; LH; Lymphocytic hypophysitis		'''Other Names:''' LYH; LH; Lymphocytic hypophysitis

	Lymphocytic hypophysitis (LH) is a condition in which the [[pituitary gland]] becomes infiltrated by [[lymphocytes]], resulting in pituitary enlargement and impaired function.		Lymphocytic hypophysitis (LH) is a condition in which the [[pituitary gland]] becomes infiltrated by [[lymphocytes]], resulting in pituitary enlargement and impaired function.
	It most often occurs in women in late pregnancy or the postpartum period, but can also occur in prepubertal or post-menopausal women, and in men.		It most often occurs in women in late pregnancy or the postpartum period, but can also occur in prepubertal or post-menopausal women, and in men.

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	== '''Epidemiology''' ==		== '''Epidemiology''' ==
	A large scale study on cadavers done in Sweden, performed biopsies on hundreds of pituitary glands. The study indicated that perhaps as much as 5% of the population experiences some amount of autoimmune pituitary destruction. It is further hypothesized that perhaps half that many show, or may experience, clinical manifestations.		A large scale study on cadavers done in Sweden, performed biopsies on hundreds of pituitary glands. The study indicated that perhaps as much as 5% of the population experiences some amount of autoimmune pituitary destruction. It is further hypothesized that perhaps half that many show, or may experience, clinical manifestations.

	== '''Cause''' ==		== '''Cause''' ==
	The exact cause of lymphocytic hypophysitis (LH) is still under debate and remains unclear. It has been suggested that LH has an autoimmune etiology. This has generally been supported by the frequent postpartum occurrence and the lymphocytic infiltration, as well as several histopathological, laboratory and clinical findings.		The exact cause of lymphocytic hypophysitis (LH) is still under debate and remains unclear. It has been suggested that LH has an autoimmune etiology. This has generally been supported by the frequent postpartum occurrence and the lymphocytic infiltration, as well as several histopathological, laboratory and clinical findings.

	== '''Signs and symptoms''' ==		== '''Signs and symptoms''' ==
	Individuals affected with lymphocytic hypophysitis typically have headaches as their first symptom. This usually precedes or occurs with visual field impairment; rarely, double vision ([[diplopia]]) may be present. [[Hyperprolactinemia]] affects approximately one third of individuals, causing [[amenorrhea]] (absence of menstruation) or [[galactorrhea]] (production of breast milk) in women and sexual dysfunction in men.		Individuals affected with lymphocytic hypophysitis typically have headaches as their first symptom. This usually precedes or occurs with visual field impairment; rarely, double vision ([[diplopia]]) may be present. [[Hyperprolactinemia]] affects approximately one third of individuals, causing [[amenorrhea]] (absence of menstruation) or [[galactorrhea]] (production of breast milk) in women and sexual dysfunction in men.

	Other features that occur more rarely and are related to alterations in pituitary secretions may include:		Other features that occur more rarely and are related to alterations in pituitary secretions may include:
	* hypoprolactinemia (deficiency of prolactin)		* hypoprolactinemia (deficiency of prolactin)
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	* [[hypogonadotropic hypogonadism]] (usually diagnosed only in males)		* [[hypogonadotropic hypogonadism]] (usually diagnosed only in males)
	* isolated [[growth hormone]] (GH) deficiency		* isolated [[growth hormone]] (GH) deficiency

	== '''Diagnosis''' ==		== '''Diagnosis''' ==
	[[Biopsy]] is the only means of accurate diagnosis as no autoantigen has been discovered. Biopsy of the [[pituitary gland]] is not easily performed with safety as it sits under the brain, however, a test does exist to detect antibodies to the pituitary without biopsy: autoantibodies to M(r) 49,000 pituitary cytosolic protein may represent markers for an immunological process affecting the pituitary gland. Tests for normal pituitary gland hormone production tend to be expensive and in some cases difficult to administer. In addition, certain hormone levels vary largely throughout the day and in response to metabolic factors, making abnormal levels difficult to ~~calibrate—further~~ hampering diagnosis.		[[Biopsy]] is the only means of accurate diagnosis as no autoantigen has been discovered. Biopsy of the [[pituitary gland]] is not easily performed with safety as it sits under the brain, however, a test does exist to detect antibodies to the pituitary without biopsy: autoantibodies to M(r) 49,000 pituitary cytosolic protein may represent markers for an immunological process affecting the pituitary gland. Tests for normal pituitary gland hormone production tend to be expensive and in some cases difficult to administer. In addition, certain hormone levels vary largely throughout the day and in response to metabolic factors, making abnormal levels difficult to calibrate‚Äîfurther hampering diagnosis.
	[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary biopsy is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.		[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary biopsy is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.

	== '''Treatment''' ==		== '''Treatment''' ==
	Lymphocytic hypophysitis can evolve differently among affected individuals, so different treatment strategies may be required. Some individuals experience spontaneous remission (improvement or reversal of the condition without any formal treatment). Careful follow-up is typically recommended.		Lymphocytic hypophysitis can evolve differently among affected individuals, so different treatment strategies may be required. Some individuals experience spontaneous remission (improvement or reversal of the condition without any formal treatment). Careful follow-up is typically recommended.
	Many affected individuals only have headaches as a manifesting symptom, and there have been multiple reports of these individuals improving with [[glucocorticoids]] alone. Although the use of glucocorticoids or other anti-inflammatory and immunosuppressive drugs have been suggested as medical treatment, their long-term efficacy still needs to be confirmed. High-dose [[methylprednisolone]] pulse therapy (short, intensive administration given at set intervals) seems to be effective in about 30% of treated patients.		Many affected individuals only have headaches as a manifesting symptom, and there have been multiple reports of these individuals improving with [[glucocorticoids]] alone. Although the use of glucocorticoids or other anti-inflammatory and immunosuppressive drugs have been suggested as medical treatment, their long-term efficacy still needs to be confirmed. High-dose [[methylprednisolone]] pulse therapy (short, intensive administration given at set intervals) seems to be effective in about 30% of treated patients.

	[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary [[biopsy]] is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.		[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary [[biopsy]] is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.

	{{Pituitary disease}}		{{Pituitary disease}}
	{{Hypersensitivity and autoimmune diseases}}		{{Hypersensitivity and autoimmune diseases}}

	[[Category:Pituitary disorders]]		[[Category:Pituitary disorders]]
	[[Category:Autoimmune diseases]]		[[Category:Autoimmune diseases]]

Deepika vegiraju at 11:21, 4 November 2020

2020-11-04T11:21:03Z

New page

'''Other Names:''' LYH; LH; Lymphocytic hypophysitis

Lymphocytic hypophysitis (LH) is a condition in which the [[pituitary gland]] becomes infiltrated by [[lymphocytes]], resulting in pituitary enlargement and impaired function.
It most often occurs in women in late pregnancy or the postpartum period, but can also occur in prepubertal or post-menopausal women, and in men.

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== '''Epidemiology''' ==
A large scale study on cadavers done in Sweden, performed biopsies on hundreds of pituitary glands. The study indicated that perhaps as much as 5% of the population experiences some amount of autoimmune pituitary destruction. It is further hypothesized that perhaps half that many show, or may experience, clinical manifestations.

== '''Cause''' ==
The exact cause of lymphocytic hypophysitis (LH) is still under debate and remains unclear. It has been suggested that LH has an autoimmune etiology. This has generally been supported by the frequent postpartum occurrence and the lymphocytic infiltration, as well as several histopathological, laboratory and clinical findings.

== '''Signs and symptoms''' ==
Individuals affected with lymphocytic hypophysitis typically have headaches as their first symptom. This usually precedes or occurs with visual field impairment; rarely, double vision ([[diplopia]]) may be present. [[Hyperprolactinemia]] affects approximately one third of individuals, causing [[amenorrhea]] (absence of menstruation) or [[galactorrhea]] (production of breast milk) in women and sexual dysfunction in men.

Other features that occur more rarely and are related to alterations in pituitary secretions may include:
* hypoprolactinemia (deficiency of prolactin)
* subclinical [[hypopituitarism]] (decreased production of pituitary hormones with no symptoms)
* ACTH deficiency
* acute adrenal crisis
* [[hypogonadotropic hypogonadism]] (usually diagnosed only in males)
* isolated [[growth hormone]] (GH) deficiency

== '''Diagnosis''' ==
[[Biopsy]] is the only means of accurate diagnosis as no autoantigen has been discovered. Biopsy of the [[pituitary gland]] is not easily performed with safety as it sits under the brain, however, a test does exist to detect antibodies to the pituitary without biopsy: autoantibodies to M(r) 49,000 pituitary cytosolic protein may represent markers for an immunological process affecting the pituitary gland. Tests for normal pituitary gland hormone production tend to be expensive and in some cases difficult to administer. In addition, certain hormone levels vary largely throughout the day and in response to metabolic factors, making abnormal levels difficult to calibrate—further hampering diagnosis.
[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary biopsy is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.

== '''Treatment''' ==
Lymphocytic hypophysitis can evolve differently among affected individuals, so different treatment strategies may be required. Some individuals experience spontaneous remission (improvement or reversal of the condition without any formal treatment). Careful follow-up is typically recommended.
Many affected individuals only have headaches as a manifesting symptom, and there have been multiple reports of these individuals improving with [[glucocorticoids]] alone. Although the use of glucocorticoids or other anti-inflammatory and immunosuppressive drugs have been suggested as medical treatment, their long-term efficacy still needs to be confirmed. High-dose [[methylprednisolone]] pulse therapy (short, intensive administration given at set intervals) seems to be effective in about 30% of treated patients.

[[Transsphenoidal surgery]] (accessed "through the nose") to confirm diagnosis and save viable pituitary tissue may be required in individuals with symptoms or signs of severe compression. Visual improvement following decompression with this type of surgery has been reported. In some cases, pituitary [[biopsy]] is both diagnostic and therapeutic, because after this procedure a progressive recovery of pituitary function may occur.

{{Pituitary disease}}
{{Hypersensitivity and autoimmune diseases}}

[[Category:Pituitary disorders]]
[[Category:Autoimmune diseases]]
[[Category:IgG4-related disease]]
{{rarediseases}}
{{stub}}