title: Hypothalamo-pituitary sarcoidosis: a multicenter study of 24 patients.
abstract: OBJECTIVE
  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.
  
  
  METHODS
  Multicentric retrospective study.
  
  
  METHODS
  Retrospective chart review. Each patient was matched with two controls.
  
  
  RESULTS
  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.
  
  
  CONCLUSIONS
  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.
authors: Department of Endocrinology, Fédération d’Endocrinologie, Groupement hospitalier Est, and University of Claude Bernard Lyon 1, 59 Boulevard Pinel 69677 Bron Cedex, France.
authors: C
authors: C
authors: Langrand
authors: H
authors: H
authors: Bihan
authors: G
authors: G
authors: Raverot
authors: L
authors: L
authors: Varron
authors: G
authors: G
authors: Androdias
authors: F
authors: F
authors: Borson-Chazot
authors: T
authors: T
authors: Brue
authors: P
authors: P
authors: Cathebras
authors: L
authors: L
authors: Pinede
authors: G
authors: G
authors: Muller
authors: C
authors: C
authors: Broussolle
authors: F
authors: F
authors: Cotton
authors: D
authors: D
authors: Valeyre
authors: P
authors: P
authors: Seve
mesh_terms: D000328:Adult; D000368:Aged; D001706:Biopsy; D016022:Case-Control Studies; D002493:Central Nervous System Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology; D002648:Child; D016903:Drug Monitoring; D005260:Female; D005938:Glucocorticoids / Q000008:administration & dosage; D006801:Humans; D007027:Hypothalamic Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology; D007028:Hypothalamic Hormones / Q000032:analysis / Q000378:metabolism; D007030:Hypothalamo-Hypophyseal System / Q000187:drug effects* / Q000503:physiopathology; D007031:Hypothalamus / Q000378:metabolism / Q000473:pathology; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010902:Pituitary Gland / Q000378:metabolism / Q000473:pathology; D010907:Pituitary Hormones / Q000032:analysis / Q000378:metabolism; D011241:Prednisone / Q000008:administration & dosage; D012507:Sarcoidosis / Q000175:diagnosis / Q000188:drug therapy / Q000378:metabolism / Q000503:physiopathology; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007028:Hypothalamic Hormones; D010907:Pituitary Hormones; D011241:Prednisone