Diabetes insipidus is an unfavorable prognostic factor for response to glucocorticoids in patients with autoimmune hypophysitis

Autoimmune hypophysitis (AH) has a variable clinical presentation and natural history; likewise, its response to glucocorticoid therapy is often unpredictable. To identify clinical and radiological findings associated with response to glucocorticoids. 12 consecutive patients with AH, evaluated from...

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Published in:European journal of endocrinology Vol. 177; no. 2; p. 127
Main Authors: Lupi, Isabella, Cosottini, Mirco, Caturegli, Patrizio, Manetti, Luca, Urbani, Claudio, Cappellani, Daniele, Scattina, Ilaria, Martino, Enio, Marcocci, Claudio, Bogazzi, Fausto
Format: Journal Article
Language:English
Published: England 01.08.2017
Subjects:
Adult
Aged
Autoimmune Hypophysitis - blood
Autoimmune Hypophysitis - diagnostic imaging
Autoimmune Hypophysitis - drug therapy
Cohort Studies
Diabetes Insipidus - blood
Diabetes Insipidus - diagnostic imaging
Diabetes Insipidus - drug therapy
Female
Follow-Up Studies
Glucocorticoids - therapeutic use
Humans
Magnetic Resonance Imaging - methods
Male
Middle Aged
Prognosis
Retrospective Studies
Treatment Outcome
ISSN:1479-683X, 1479-683X
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Summary:Autoimmune hypophysitis (AH) has a variable clinical presentation and natural history; likewise, its response to glucocorticoid therapy is often unpredictable. To identify clinical and radiological findings associated with response to glucocorticoids. 12 consecutive patients with AH, evaluated from 2008 to 2016. AH was the exclusion diagnosis after ruling out other pituitary masses and secondary causes of hypophysitis. Mean follow-up time was 30 ± 27 months (range 12-96 months). MRI identified two main patterns of presentation: global enlargement of the pituitary gland or panhypophysitis (  = 4, PH), and pituitary stalk abnormality only, or infundibulo-neuro-hypophysitis (  = 8, INH). Multiple tropin defects were more common in PH (100%) than those in INH (28%  = 0.014), whereas diabetes insipidus was more common in INH (100%) than that in PH (50%;  = 0.028). All 4 PH and 4 out of 8 INH were treated with glucocorticoids. Pituitary volume significantly reduced in all PH patients (  = 0.012), defective anterior pituitary function recovered only in the two patients without diabetes insipidus (50%) and panhypopituitarism persisted, along with diabetes insipidus, in the remaining 2 (50%). In all INH patients, either treated or untreated, pituitary stalk diameter reduced (  = 0.008) but diabetes insipidus persisted in all. Glucocorticoid therapy may improve anterior pituitary function in a subset of patients but has no effect on restoring posterior pituitary function. Diabetes insipidus appears as a negative prognostic factor for response to glucocorticoids.
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ISSN:1479-683X
1479-683X
DOI:10.1530/EJE-17-0123