Primary hypophysitis: clinical-pathological correlations.
OBJECTIVE
Primary hypophysitis comprises of three distinct histomorphological entities: lymphocytic, granulomatous and xanthomatous. Clinical features of the three subtypes for diagnostic and treatment strategies have yet not been well characterized.


METHODS
Endocrine function, visual fields and acuity as well as magnetic resonance imaging characteristics were assessed before and after transphenoidal surgery in the largest series of 31 patients with primary hypophysitis (21 lymphocytic, 6 granulomatous, and 4 xanthomatous cases).


RESULTS
Only lymphocytic hypophysitis occurred during pregnancy (30%) and was associated with other autoimmune diseases (24%). Visual fields and acuity abnormalities were not seen in xanthomatous hypophysitis. Lymphocytic and granulomatous hypophysitis most often resulted in severe dysfunction of the adrenal, gonadal and thyroidal axes as well as diabetes insipidus. For patients presenting with xanthomatous hypophysitis most often, mild anterior pituitary axis failure was documented and posterior pituitary involvement was hardly found. The outcome after transphenoidal biopsy was generally favorable. Pre- or postsurgical glucocorticoid treatment was very effective in 75% of the lymphocytic form in reducing the pituitary size. In contrast, glucocorticoid therapy was less effective in granulomatous or xanthomatous hypophysitis.


CONCLUSIONS
Diffuse destruction of the complete pituitary gland including the infundibulum has to be considered in lymphocytic and granulomatous hypophysitis, whereas in xanthomatous, a circumscribed anterior pituitary lesion leading to compression of the pituitary gland without alteration of the pituitary stalk and optic chiasm can be assumed.
Gutenberg
Angelika
A
Department of Neurosurgery, Georg August University Göttingen, Göttingen, Germany. agutenberg@med.uni-goettingen.de
Hans
Volkmar
V
Puchner
Maximilian J A
MJ
Kreutzer
Jürgen
J
Brück
Wolfgang
W
Caturegli
Patrizio
P
Buchfelder
Michael
M
D000328:Adult; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006099:Granuloma / Q000473:pathology; D020249:Hormone Replacement Therapy; D006728:Hormones / Q000097:blood; D006801:Humans; D007016:Hypophysectomy; D008214:Lymphocytes / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D008581:Meningitis / Q000209:etiology; D008875:Middle Aged; D010900:Pituitary Diseases / Q000150:complications* / Q000473:pathology*; D010901:Pituitary Function Tests; D010902:Pituitary Gland / Q000473:pathology* / Q000503:physiopathology; D011247:Pregnancy; D014792:Visual Acuity / Q000502:physiology; D014794:Visual Fields / Q000502:physiology; D014973:Xanthomatosis / Q000473:pathology
D005938:Glucocorticoids; D006728:Hormones