Hypothalamo-pituitary sarcoidosis: a multicenter study of 24 patients
Authors: Langrand, C.1; Bihan, H.2; Raverot, G.1; Varron, L.3; Androdias, G.4; Borson-Chazot, F.1; Brue, T.5; Cathebras, P.6; Pinede, L.7; Muller, G.8; Broussolle, C.3; Cotton, F.9; Valeyre, D.10; Seve, P.3
Source: QJM: An International Journal of Medicine, Volume 105, Number 10, 26 October 2012 , pp. 981-995(15)
Publisher: Oxford University Press
Abstract:Aim: 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.
Design: 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: 869 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 (n21), TSH deficiency (n15) and hyperprolactinemia (n12). Magnetic Resonance Imaging (MRI) revealed infundibulum involvement (n8), pituitary stalk thickness (n12) and involvement of the pituitary gland (n14). 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.
Conclusion: 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.
Document Type: Research Article
Affiliations: 1: Department of Endocrinology, Fédération d’Endocrinologie, Groupement hospitalier Est, and University of Claude Bernard Lyon 1, 59 Boulevard Pinel 69677 Bron Cedex, , 2: Department of Internal Medicine and Endocrinology, Hôpital Avicenne and University of Paris, 125 rue de Stalingrad 93009 Bobigny Cedex, , 3: Department of Internal Medicine, Hôpital de La Croix-Rousse and University of Claude Bernard Lyon 1, 103 Grande rue de la Croix-Rousse 69317 Lyon Cedex 04, , 4: Department of Neurology A and EDMUS Coordinating Center, Hôpital Neurologique and INSERM U842, University of Claude Bernard Lyon 1, 59 Boulevard Pinel 69500 Bron, , 5: Department of Endocrinology, Hôpital de la Timone, Centre de Référence des Maladies Rares d’Origine Hypophysaire, Assistance Publique-Hôpitaux de Marseille and University of Aix-Marseille, 13385 Marseille, , 6: Department of Internal Medicine, Hôpital Nord, Avenue Albert Raimond 42270 Saint-Priest-en-Jarez, , 7: Department of Internal Medicine, Infirmerie protestante, 3 Chemin Penthod 69300 Caluire-et-Cuire, , 8: Department of Internal Medicine, Hôpital Général, 2 rue de l’hôpital et 3 rue du faubourg Raines 21033 Dijon, , 9: Department of Radiology, Centre Hospitalier Lyon Sud and University of Claude Bernard Lyon 1, 165 chemin du grand revoyet 69495 Pierre-Bénite Cedex and , 10: Department of Pneumology, Hôpital Avicenne and University of Paris, 125 rue de Stalingrad 93009 Bobigny Cedex, France,
Publication date: 2012-10-26
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