Endocrine Abstracts

Lymphocytic hypophysitis

SJB Aylwin

King’s College Hospital, London, United Kingdom.

Lymphocytic hypophysitis (LYH) is an unusual pituitary disorder in which an inflammatory process compromises normal anterior and posterior pituitary function, and produces a mass effect on adjacent structures.

Clinical features and diagnosis: LYH typically presents in the peripartum period, although the condition may be encountered in middle-aged women and in male subjects. LYH may be suspected in patients presenting during or following pregnancy, in subjects with diabetes insipidus or with severe headache. MRI findings include infiltration of the infundibulum, enhancement after gadolinium and extension of the infiltrate into the surrounding dura (‘dural tail’). Surgical biopsy remains the diagnostic gold standard.

Pathology/Classification: LYH is characterised by a lymphoplasmacytic infiltrate; the presence of multinucleated giant cells or xanthomatous macrophages support a diagnosis of granulomatous and xanthomatous hypophysitis, respectively. It remains unclear whether these and LYH are distinct disease entities. LYH is frequently encountered in patients with other auto-immune diseases, most commonly thyroiditis.

Management: Excision biopsy may offer relief from mass effect in addition to securing a histological diagnosis, and hormone supplementation may be required. Corticosteroids and other immuno-modulatory regimens have been employed as adjuvant therapy, but their efficacy has to be considered against a background of a relapsing – remitting condition of variable natural history. We, and others, have described patients who have responded favourably to radiotherapy.

Future Perspective: The identification of specific auto-antigens would further our understanding of the pathogenesis, whereas non-invasive diagnostic criteria might facilitate evaluation of non-surgical treatment. The diagnostic use of anti-pituitary antibodies may broaden the spectrum of LYH to include pituitary deficiency currently considered sporadic.

Endocrine Abstracts (2005) 10 S32