Pituitary and stalk lesions (infundibulo-hypophysitis) associated with immunoglobulin G4-related systemic disease: an emerging clinical entity.

Abstract:

:Inflammatory lesions of the pituitary gland are rarely encountered. Recently, the concept of immunoglobulin G4 (IgG4)-related systemic disease was proposed in Japan, and more than 20 cases have been reported as possibly associated with infundibulo-hypophysitis since 2000. We herein review such case reports in the published literature and in the abstracts of scientific meetings. Almost all cases involved middle-aged to elderly men presenting with various degrees of hypopituitarism and diabetes insipidus and demonstrating a thickened pituitary stalk and/or pituitary mass. These structures shrank remarkably in response to glucocorticoid therapy, even in a lower dose range similar to that prescribed as a replacement for adrenocortical insufficiency. Some of the anterior pituitary insufficiencies were also resolved by glucocorticoid administration. The presence of IgG4-related systemic disease and an elevated serum IgG4 level before glucocorticoid therapy were the main clues to a correct diagnosis of IgG4-related infundibulo-hypophysitis. Autoimmunity is suggested but not yet established to play a role in the pathogenesis for IgG4-related systemic disease. The fact that hypertrophic pachymeningitis and para-sinusitis accompanied some cases suggested that both sellar and parasellar structures are involved in the chronic inflammation. We therefore classify this disorder not as a variant form of primary autoimmune hypophysitis but as a secondary form of infundibulo-hypophysitis associated with IgG4-related systemic disease.

journal_name

Endocr J

journal_title

Endocrine journal

authors

Shimatsu A,Oki Y,Fujisawa I,Sano T

doi

10.1507/endocrj.k09e-277

subject

Has Abstract

pub_date

2009-01-01 00:00:00

pages

1033-41

issue

9

eissn

0918-8959

issn

1348-4540

pii

JST.JSTAGE/endocrj/K09E-277

journal_volume

56

pub_type

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