title: [Polyendocrinopathy combined with primary pulmonary arterial hypertension].
abstract: We report the case of a woman who had premature menopause, adrenal insufficiency and hypothyroidism by peripheral gland lesion, all most probably due to an autoimmune disorder, and who subsequently developed primary pulmonary arterial hypertension. The causes of this hypertension are ill-defined, but an autoimmune origin has often been envisaged since primary pulmonary arterial hypertension is not unfrequently associated with connective tissue diseases. Its association with an autoimmune polyendocrinopathy has only been reported, to our knowledge, on four occasions; in all 4 cases the thyroid gland was involved, and a connective tissue disease was present in 3 of them. Our case, which includes adrenal insufficiency and premature menopause is, as far as we know, unique. The possible link between these various diseases is their autoimmune nature, in which case primary pulmonary arterial hypertension would belong to the category of organ-specific autoimmune diseases. Our case supports the hypothesis that a number of isolated primary pulmonary arterial hypertensions could be of autoimmune origin.
authors.forename: Z
authors.initials: Z
authors.lastname: Barrou
authors.forename: M
authors.initials: M
authors.lastname: Pehuet-Figoni
authors.forename: S
authors.initials: S
authors.lastname: Weber
authors.forename: A
authors.initials: A
authors.lastname: Lockhart
authors.forename: J P
authors.initials: JP
authors.lastname: Luton
mesh_terms: D001327:Autoimmune Diseases / Q000209:etiology*; D006328:Cardiac Catheterization; D004700:Endocrine System Diseases / Q000150:complications*; D005260:Female; D006728:Hormones / Q000032:analysis; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000000981:diagnostic imaging; D008594:Menopause, Premature; D008875:Middle Aged; D011859:Radiography
chemical_list: D006728:Hormones
vernacular_title: Polyendocrinopathie associée à une hypertension artérielle pulmonaire primitive.