Hypothalamo-pituitary sarcoidosis: a multicenter study of 24 patients

• Published in: QJM : An International Journal of Medicine Vol. 105; no. 10; pp. 981 - 995
• Main Authors: LANGRAND, C, BIHAN, H, BROUSSOLLE, C, COTTON, F, VALEYRE, D, SEVE, P, RAVEROT, G, VARRON, L, ANDRODIAS, G, BORSON-CHAZOT, F, BRUE, T, CATHEBRAS, P, PINEDE, L, MULLER, G
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.10.2012
• Subjects: Adult; Aged; Biological and medical sciences; Biopsy; Case-Control Studies; Central Nervous System Diseases - diagnosis; Central Nervous System Diseases - drug therapy; Central Nervous System Diseases - metabolism; Central Nervous System Diseases - physiopathology; Child; Drug Monitoring; Female; General aspects; Glucocorticoids - administration & dosage; Humans; Hypothalamic Diseases - diagnosis; Hypothalamic Diseases - drug therapy; Hypothalamic Diseases - metabolism; Hypothalamic Diseases - physiopathology; Hypothalamic Hormones - analysis; Hypothalamic Hormones - secretion; Hypothalamo-Hypophyseal System - drug effects; Hypothalamo-Hypophyseal System - physiopathology; Hypothalamus - metabolism; Hypothalamus - pathology; Magnetic Resonance Imaging - methods; Male; Medical sciences; Pituitary Gland - metabolism; Pituitary Gland - pathology; Pituitary Hormones - analysis; Pituitary Hormones - secretion; Prednisone - administration & dosage; Sarcoidosis - diagnosis; Sarcoidosis - drug therapy; Sarcoidosis - metabolism; Sarcoidosis - physiopathology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Treatment Outcome; Human; Medicine; Endocrine gland; Sarcoidosis; Multicenter study; Systemic disease; Pituitary gland; Patient
• ISSN: 1460-2725; 1460-2393
• DOI: 10.1093/qjmed/hcs121

To assess clinical features, treatment and outcome of Hypothalamo-pituitary (HP) sarcoidosis and to determine whether HP is associated with a particular clinical phenotype of sarcoidosis. Multicentric retrospective study. Retrospective chart review. Each patient was matched with two controls. Twenty-four patients were identified (10 women, 14 men). Their median age at the sarcoidosis diagnosis was 31.5 years (range: 8-69 years). HP involvement occurred in the course of a previously known sarcoidosis in 11 cases (46%), whereas it preceded the diagnosis in 13 patients (54%). All but two patients had anterior pituitary dysfunction, 12 patients presented with diabetes insipidus. The most common hormonal features were gonadotropin deficiency (n=21), TSH deficiency (n=15) and hyperprolactinemia (n=12). Magnetic Resonance Imaging (MRI) revealed infundibulum involvement (n=8), pituitary stalk thickness (n=12) and involvement of the pituitary gland (n=14). All but two patients received prednisone. After a mean follow-up of 4 years, only two patients recovered from hormonal deficiencies. MRI abnormalities improved or disappeared in 12 cases under corticosteroid. There was no correlation between the hormonal dysfunctions and the radiologic outcomes. Patients with HP sarcoidosis had significantly more frequent sinonasal localizations and neurosarcoidosis and required a systemic treatment more frequently than controls. Although HP sarcoidosis is unusual, physicians should be aware that such specific localization could be the first manifestation of sarcoidosis. HP involvement is associated with general severity of sarcoidosis. MRI abnormalities can improve or disappear under corticosteroid treatment, but most endocrine defects are irreversible.