Pituitary Inflammation (Hypophysitis)

What is Pituitary Inflammation (Hypophysitis)?

Overview

Hypophysitis is inflammation of the pituitary gland or pituitary stalk and typically results in varying degrees of pituitary gland failure.

There are several types or possible causes of pituitary gland inflammation (also known as hypophysitis). The most common type is lymphocytic hypophysitis followed by granulomatous hypophysitis.

Who is Affected?

Classic lymphocytic hypophysitis occurs predominantly in women during or after pregnancy, but it can occur in men as well, and across a wide age spectrum. A relatively new form of hypophysitis has recently been described in patients undergoing immunotherapy as a cancer treatment, most commonly in patients with metastatic melanoma being treated with ipilimumab (a human monoclonal antibody against cytotoxic T-lymphocyte antigen 4).

Pituitary Inflammation Symptomsrn& Causes

Symptoms

The most common symptoms and signs of hypophysitis are anterior and posterior pituitary gland failure (which may include adrenal insufficiency, hypothyroidism, hypogonadism, growth hormone deficiency, as well as diabetes insipidus).

Other common symptoms include headache and visual loss.

In some cases, hypophysitis may present with additional symptoms that are specific to certain types or underlying causes.

For instance, lymphocytic hypophysitis, which is the most common form, often manifests with symptoms related to autoimmune activity in the body. These symptoms may include fatigue, joint pain, muscle weakness, and general malaise.

Other less frequent types of hypophysitis, such as granulomatous hypophysitis or xanthomatous hypophysitis, may have distinct clinical features associated with the nature of the inflammation or the presence of granulomas or lipid-laden cells, respectively.

Causes

The causes of hypophysitis vary by type:

• Lymphocytic hypophysitis: Often autoimmune in nature and associated with pregnancy.
• Granulomatous hypophysitis: Linked to granulomatous diseases such as sarcoidosis or infections.
• Xanthomatous hypophysitis: A rare form with lipid-rich inflammatory cells.
• IgG4-related hypophysitis: Associated with systemic IgG4-related disease.
• Medication-induced hypophysitis: Most often seen with immune checkpoint inhibitors used in cancer therapy.

When to See a Doctor

As hypophysitis is a relatively uncommon condition, it is important for individuals experiencing persistent hormonal disturbances, severe headaches, or visual changes to consult a healthcare professional for a comprehensive evaluation.

Early diagnosis and proper management can help alleviate symptoms, improve quality of life, and prevent potential complications related to hormonal imbalances or pressure on surrounding structures in the brain.

Pituitary Inflammation Diagnosis

A presumptive diagnosis of hypophysitis can be made in a patient with otherwise unexplained hypopituitarism with characteristic findings on pituitary MRI of diffuse pituitary gland enlargement and/or a thickened pituitary stalk (infundibulum).

In a minority of patients with presumed hypophysitis, a pituitary biopsy may be warranted to confirm the diagnosis if MRI findings are not completely convincing. Such a pituitary biopsy can be safely done with an endoscopic endonasal approach.

Pituitary Inflammation Treatmentrn& Outcomes

Treatment Options

Treatment of hypophysitis is typically with hormone replacement therapy and with high dose glucocorticoids (steroids) to reduce inflammation. Despite long-term treatment with steroids, most patients do develop some degree of permanent pituitary gland failure.

Pituitary surgery using an endoscopic endonasal approach may also be indicated for patients with visual loss from the enlarged pituitary gland impinging upon the optic nerves or optic chiasm.

Patient Outcomes

While steroids can reduce inflammation and improve symptoms, many patients develop some degree of permanent pituitary gland failure, requiring lifelong hormone replacement therapy. With proper treatment, most patients achieve good symptom control and improved quality of life.

Managing Pituitary Inflammation

Ongoing management of hypophysitis often includes:

Meet our Expert Specialists & Surgeons

Abbas A. Anwar, MD

ENT Surgery, Neurorhinology

Garni Barkhoudarian, MD

Neurosurgery

Walavan Sivakumar, MD

Neurosurgery

Chester F. Griffiths, MD

ENT Surgery

Daniel F. Kelly, MD

Neurosurgery

Noa Tal, MD

Endocrinology

Michael Yong, MD, MPH, MBA

ENT Surgery, Neuro-rhinology

Alexander M. Solomon, MD

Neuro-ophthalmology

Howard R. Krauss, MD

Neuro-ophthalmology

Experience Compassionate, Expert Care

At the Pacific Pituitary Disorders Center we have one of the world’s largest and most extensive experiences in diagnosing and treating patients with hypopituitarism of all causes. Our team of pituitary endocrinologists and neurosurgeons work closely together to preserve or restore pituitary gland function and quality of life.

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