IgG4-related hypophysitis

Last revised by Mostafa Elfeky on 16 Jul 2023

IgG4-related hypophysitis is a rare cause of inflammation of the pituitary gland and an uncommon manifestation of the systemic IgG4-related disease (IgG4-RD).

Although IgG4-RD tends to present in middle-aged and elderly men, the gender ratio is more balanced in patients with IgG4-related hypophysitis and other head and neck manifestations of IgG4-RD 1

Clinical features depend on the part of the pituitary gland involved 1-3:

IgG4-related hypophysitis is characterized by chronic inflammation of the pituitary gland with histology revealing lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells 1,2. This classic histological finding differentiates it from the histological appearances of other etiologies of hypophysitis, those being:

Although this is a very rare entity, common radiographic findings that have been reported include non-specific enlargement of the pituitary gland with or without infundibulum enlargement that is visible on both CT and MRI 1,4,5. Post-contrast T1-weighted MR images may further reveal enhancement 1,4,5.

Other associated head and neck manifestations of IgG4-RD, such as IgG4-related hypertrophic pachymeningitis, may also be seen 1,4,5.  

Based on case series-level evidence, good response is seen to corticosteroids such as prednisolone or hydrocortisone 1,2. Treatment of endocrine hormone deficiencies (e.g. giving desmopressin for diabetes insipidus) is also prudent 1,2.  

The differential diagnosis is primarily that of other pituitary region masses. Considerations include:

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