title: [Subclinical adrenal diseases: silent pheochromocytoma and subclinical Addison's disease].
abstract: The silent pheochromocytoma, a hidden form of pheochromocytoma, exposes the patient to an increased risk of mortality if the diagnosis is not established on time. Biological diagnosis of pheochromocytoma can be difficult. Catecholamine secretion is dependent on tumor size and a large number of physiological, pharmacological, lifestyle modifications and sampling conditions influence the measurement of urinary and plasma metanephrines. The prevalence of pheochromocytoma is 2% among adrenal incidentaloma smaller than 3 cm (2/3 of tumors). Recent studies suggest the almost zero risk of pheochromocytoma among these tumors if they are hypodense (<10 housefield units) on adrenal tomography. Addison's disease is a pathology affecting about 1 in 8000. Immunopathology is still unknown, but some elements advocated the hypothesis of a predominant cell-mediated immunity in particular Interferon-gamma production by CD4 T lymphocytes in the presence of an epitope from the 21-hydroxylase, as well as IgG1 subtype produced by activated B lymphocytes, autoantibodies do appear to be a simple marker of the disease. Subclinical Addison's disease is defined by the presence of anti-21-hydroxylase autoantibodies, without clinical symptoms. It evolves faster to the clinical phase in young subjects, male, having high levels of autoantibodies and with an initially impaired adrenal function. Dosage of ACTH, plasma renin active, and basal cortisol and after Synacthen allow to discriminate the subjects with low or high risk of evolution and establish an appropriate monitoring.
authors.affiliation: Service d'Endocrinologie CHU Brest, Boulevard Tanguy Prigent, 29609 Brest cedex, France. philippe.thuillier@chubrest.fr
authors.forename: P
authors.initials: P
authors.lastname: Thuillier
authors.forename: V
authors.initials: V
authors.lastname: Kerlan
mesh_terms: D000224:Addison Disease / Q000175:diagnosis / Q000276:immunology / Q000503:physiopathology; D000310:Adrenal Gland Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D000311:Adrenal Glands / Q000503:physiopathology; D000324:Adrenocorticotropic Hormone / Q000097:blood; D001323:Autoantibodies / Q000097:blood; D015496:CD4-Positive T-Lymphocytes / Q000276:immunology; D003366:Cosyntropin; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007111:Immunity, Cellular; D007371:Interferon-gamma / Q000096:biosynthesis; D008297:Male; D008676:Metanephrine / Q000097:blood / Q000652:urine; D010673:Pheochromocytoma / Q000175:diagnosis / Q000453:epidemiology / Q000473:pathology; D012083:Renin / Q000097:blood; D013255:Steroid 21-Hydroxylase / Q000276:immunology
chemical_list: D001323:Autoantibodies; D003366:Cosyntropin; D008676:Metanephrine; C006418:adrenocorticotropin zinc; D007371:Interferon-gamma; D000324:Adrenocorticotropic Hormone; D013255:Steroid 21-Hydroxylase; D012083:Renin; D006854:Hydrocortisone