Hypophysitis

Hypophysitis

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List of terms related to Hypophysitis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Synonyms and keywords:Autoimmune hypophysitis, lymphocytic hypophysitis

Overview

Hypophysitis is the inflammation of the pituitary gland that typically results in some degree of pituitary dysfunction, ranging from under secretion of some pituitary hormones to complete panhypopituitarism requiring lifelong hormone supplementation. While hypophysitis by itself is seldom life-threatening, the sequelae of pituitary inflammation, particularly adrenal insufficiency and severe hypothyroidism, may be fatal if unrecognized and left untreated. There are several recognized causes of hypophysitis, including lymphocytic infiltration, granulomatous infiltration (e.g. in the setting of a systemic granulomatous disease such as sarcoidosis or granulomatosis with polyangiitis), and cell-mediated autoimmune reactivity in the setting of treatment of certain solid tumors with immunomodulatory checkpoint inhibitors (i.e. CTLA-4 inhibitors and PD-1 inhibitors). Although there are several different subtypes of hypophysitis, there are many commonalities in the diagnostic evaluation and treatment of this rare endocrinopathy.

Historical Perspective

The first documented case of hypophysitis occurred in a 22-year-old woman who died of circulatory collapse within 8 hours of appendectomy for gangrenous appendicitis in 1962. Autopsy was notable for an enlarged thyroid, grossly normal pituitary, and absent adrenal glands. Histologic examination of the thyroid was consistent with Hashimoto's thyroiditis, and microscopic examination of the pituitary revealed extensive lymphocytic infiltration of the anterior lobe. The authors of the case report posited an autoimmune etiology to the patient's apparent thyroid and pituitary dysfunction.

Causes

Life Threatening Causes

Life-threatening causes include conditions which may result in death or permanent disability within 24 hours if left untreated.

Causes by Organ System

Cardiovascular	No underlying causes
Chemical / poisoning	No underlying causes
Dermatologic	No underlying causes
Drug Side Effect	No underlying causes
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	No underlying causes
Genetic	No underlying causes
Hematologic	No underlying causes
Iatrogenic	No underlying causes
Infectious Disease	No underlying causes
Musculoskeletal / Ortho	No underlying causes
Neurologic	No underlying causes
Nutritional / Metabolic	No underlying causes
Obstetric/Gynecologic	No underlying causes
Oncologic	No underlying causes
Opthalmologic	No underlying causes
Overdose / Toxicity	No underlying causes
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal / Electrolyte	No underlying causes
Rheum / Immune / Allergy	No underlying causes
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Dental	No underlying causes
Miscellaneous	No underlying causes

Causes in Alphabetical Order

• A...
• Z...

Make sure that each diagnosis is linked to a page.

Differentiating Hypophysitis From Other Diseases

Hypophysitis should be differentiated from other diseases causing severe headache for example:

Disease	Symptoms			Gold Standard	CT/MRI	Other Investigation Findings
	Headache		Other features
	Onset	Characterstics
Subarachnoid hemorrhage	Sudden	Severe headache Thunderclap Described as the worst headache of life	Double vision Nausea and vomiting Symptoms of meningeal irritation Sudden decreased level of consciousness	Digital subtraction angiography	The modality of choice for diagnosis of subarachnoid hemorrhage is noncontrast head computed tomography (CT), with or without lumbar puncture.[1] CT shows hyperattenuating material filling the subarachnoid space.	Lumbar puncture (LP) is necessary when there is a strong suspicion of subarachnoid hemorrhage. LP will show: Elevated opening pressure Elevated red blood cell (RBC) Xanthochromia
Meningitis	Sudden	Headache is associated with: Fever Neck stiffness	Photophobia    Phonophobia  Irritability Altered mental status	Lumbar puncture for CSF	CT scan of the head may be performed before LP to determine the risk of herniation.	Diagnosis is based on clinical presentation in combination with CSF analysis. CSF analysis is the investigation of choice. For more information on CSF analysis in meningitis please click here.
Intracranial mass	Gradual	Morning headache	Nausea Vomiting Change in mental status Seizures Focal neurological deficits	MRI	CT or MRI is the initial test to detect intracranial lesions (ring enhancing lesions). These imaging tests determine the location of intracranial mass lesion(s) and help in guiding therapy.	Biopsy of the lesion may be done to identify the nature of the lesion such as: Tumor Abscess X- ray of the skull is a non specific test, but useful if any of the lesions are calcified.
Cerebral hemorrhage	Sudden	Rapidly progressing headache	Symptoms of increased intracranial pressure (ICP) Focal neurological deficits	CT without contrast (differentiate ischemic stroke from hemorrhagic stroke.)	CT is very sensitive for identifying acute hemorrhage which appears as hyperattenuating clot. Gradient echo and T2 susceptibility-weighted MRI are as sensitive as CT for detection of acute hemorrhage and are more sensitive for identification of prior hemorrhage.	PT/ INR and aPTT should be checked to rule out coagulopathy.
Intracranial venous thrombosis	Gradual	Diffuse headache Headache can be the only symptom of cerebral venous thrombosis	Focal neurological deficits Seizures Depressed level of consciousness	Digital subtraction angiography	The classic finding of sinus thrombosis on unenhanced CT images is a hyperattenuating thrombus in the occluded sinus. CT and MRI may identify Cerebral edema and venous infarction may be apparent.	CT venography detects the thrombus, computed tomography with radiocontrast in the venous phase (CT venography or CTV) has a detection rate that in some regards exceeds that of MRI. Cerebral angiography may demonstrate smaller clots, and obstructed veins may give the "corkscrew appearance".
Migraine	Sudden	Severe to moderate headache One-sided Pulsating Lasts between several hours to three days.	Nausea and vomiting Preceding aura Photophobia Phonophobia	---	CT and MRI may be needed to rule out other suspected possible causes of headache.	Migraine is a clinical diagnosis that does not require any laboratory tests. Laboratory tests can be ordered to rule out any suspected coexistent metabolic problems or to determine the baseline status of the patient before initiation of migraine therapy.
Head injury	Sudden	Dull Throbbing One sided or all around	Confusion Drowsiness Personality change Seizures Nausea and vomiting Loss of consciousness Lucid interval	CT scan without contrast	CT scan is the first test performed and identifies cerebral hemorrhage (appears as hyperattenuating clot) following head injury. MRI is more sensitive, takes more time and is done in patients with symptoms unexplained by CT scan.	The Glasgow Coma Scale is a tool for measuring degree of unconsciousness and is a useful tool for determining severity of injury. The Pediatric Glasgow Coma Scale is used in young children.
Lymphocytic hypophysitis	Sudden	Generalized Retro-orbital or Bitemporal	Most often seen in late pregnancy or the postpartum period Mass lesion effect such as visual field defects Hypopituitarism	Pituitary biopsy	CT & MRI typically reveal features of a pituitary mass.	The most accurate test is pituitary biopsy which will show lymphocytic infiltration.

Hypophysitis should be differentiated from other diseases causing hypopituitarism.

Diseases	Onset	Manifestations					Diagnosis
		History and Symptoms				Physical examination	Laboratory findings	Gold standard	Imaging	Other investigation findings
		Trumatic delivery	Lactation failure	Menstrual irregularities	Other features
Sheehan's syndrome	Acute	++	++	Oligo/amenorrhea	Symptoms of: Adrenal insufficiency Hypothyroidism	Breast tissue atrophy Decreased axillary and pubic hair growth	Pancytopenia Eosinophilia Hyponatremia Low fasting plasma glucose Decreased levels of anterior pituitary hormones in blood	Clinical diagnosis Most senitive test: Low baseline prolactin levels w/o response to TRH	CT/MRI: Sequential changes of pituitary enlargement followed by: Shrinkage and necrosis leading to decreased sellar volume or empty sella	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Lymphocytic hypophysitis	Acute	+/-	+	Oligo/amenorrhea	Associated with autoimmune conditions Generalized headache Retro-orbital or Bitemporal pain Mass lesion effect such as visual field defects	DI Autoimmune thyroiditis	Decreased pituitary hormones(Gonadotropins most common) Hyperprolactinemia(40%) GH excess	Pituitary biopsy: lymphocytic infiltration	CT & MRI: Features of a pituitary mass Diffuse and homogeneous contrast enhancement	Assays for: Anti-TPO Anti-Tg Ab
Pituitary apoplexy	Acute	+/-	++	Oligo/amenorrhea	Severe headache Nausea and vomiting Paralysis of eye muscles (diplopia) Changes in vision	Visual acuity defects CN palsies (nerves III, IV, V , and VI)	Decreased levels of anterior pituitary hormones in blood.	MRI	CT scan without contrast: Hemorrhage on CT presents as a hyperdense lesion MRI: If inconclusive CT	Blood tests may be done to check: PT/INR and aPTT Pituitary hormonal assay
Empty sella syndrome	Chronic	-	+	Oligo/amenorrhea	Erectile dysfunction Headache Low libido	Signs of raised intracranial pressure may be present Nipple discharge	Decreased levels of pituitary hormones in blood.	MRI	Empty sella containing CSF	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Simmonds' disease/Pituitary cachexia	Chronic	+/-	+	Oligo/amenorrhea	Cachexia Premature aging	Progressive emaciation Loss of body hair	Decreased levels of anterior pituitary hormones in blood.	MRI	Done to rule out any pituitary cause	Pituitary hormone stimulation tests (Metoclopramide and clomiphene citrate stimulation tests)
Hypothyroidism	Chronic	+/-	-	Oligomenorrhea/menorrhagia	Cold intolerance Constipation	Dry skin Bradycardia Hair loss Myxedema Delayed relaxation phase of deep tendon reflexes	Low T3,T4 Normal/ low TSH Rest of pituitary hormone levels WNL	TSH levels	Done to rule out any pituitary cause	Assays for anti-TPO and anti-Tg Ab FNA biopsy
Hypogonadotropic hypogonadism	Chronic	-	-	Oligo/amenorrhea	Hot flushes Energy and mood changes Decreased libido	Breast tissue atrophy Decreased maturation of vaginal mucosa	Low estrogen, testosterone High FSH/LH	FSH LH	Done to rule out any pituitary cause	Genetic tests  (karyotype) Measurement of total and free testosterone and 17-hydroxyprogesterone concentrations
Hypoprolactinemia	Chronic	-	+	-	Infertility Subfertiliy	Puerperal agalactogenesis	No workup is necessary	Decreased prolactin levels	Done to rule out any pituitary cause	Prolactin assay in 3rd trimester LH, FSH Thyrotropin and free thyroxine
Panhypopituitarism	Chronic	-	+	Oligo/amenorrhea	Polyuria Polydipsia Features of hypothyroidism and hypoadrenalism	Growth failure B/L hemianopsia Papilledema	All pituitary hormones decreased	MRI	Done to rule out any pituitary cause	Left hand and wrist radiograph for bone age
Primary adrenal insufficiency/Addison's disease	Chronic	-	-	-	Hypoglycemia Hypotension	Dehydration Hyperpigmentation loss of pubic and axillary hair	Hyponatremia with/without hyperkalemia Plasma renin activity to aldosterone ratio	Abdominal CT	Abdominal CT	Serum cortisol testing Serum ACTH testing Anti-adrenal Ab testing
Menopause	Chronic	-	+/-	Oligo/amenorrhea	Hot flashes Insomnia Weight gain and bloating Mood changes	Vaginal atrophy Loss of pelvic muscle tone	↑ FSH ↓ Estradiol and inhibin	FSH > LH	Normal	Endometrial biopsy

References

Epidemiology and Demographics

Age

Gender

Race

Developed Countries

Developing Countries

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Criteria

If available, the diagnostic criteria are provided here.

History

A directed history should be obtained to ascertain

Symptoms

"Type symptom here" is pathognomonic of the "type disease name here".

"Type non specific symptoms" may be present.

Past Medical History

Family History

Social History

Occupational

Alcohol

The frequency and amount of alcohol consumption should be characterized.

Drug Use

Smoking

Allergies

Physical Examination

Appearance of the Patient

Vital Signs

Skin

Head

Eyes

Ear

Nose

Mouth

Throat

Heart

Lungs

Abdomen

Extremities

Neurologic

Genitals

Other

Laboratory Findings

Electrolyte and Biomarker Studies

Electrocardiogram

Chest X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Pharmacotherapy

Acute Pharmacotherapies

Chronic Pharmacotherapies

Surgery and Device Based Therapy

Indications for Surgery

Pre-Operative Assessment

Post-Operative Management

Transplantation

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

References

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