title: A case of scleroderma spectrum disorder with anticentriole antibody and pulmonary hypertension.
abstract: We describe the case of a patient with anticentriole antibody-positive scleroderma spectrum disorder (SSD) who developed pulmonary hypertension. A 54-year-old woman had noticed Raynaud's phenomenon and digital ulcers during the winter for the past 10 years. Although sclerodactyly was not present, digital ulcers, swelling of her hands, and phalangeal contracture were observed. An indirect immunofluorescence test revealed anticentriole antibody. Other SSc-specific antoantibodies were negative. An echocardiogram demonstrated that the estimated right ventricular systolic pressure was increased to 51 mmHg. She was diagnosed as SSD with pulmonary hypertension. This is the first case of SSD with anticentriole antibody to develop pulmonary hypertension.
authors.affiliation: Department of Dermatology, Kanazawa University, Graduate School of Medical Science, 13-1 Takaramachi, Ishikawa 920-8641 Kanazawa, Japan.
authors.forename: Ikuko
authors.initials: I
authors.lastname: Hayakawa
authors.forename: Shinichi
authors.initials: S
authors.lastname: Sato
authors.forename: Minoru
authors.initials: M
authors.lastname: Hasegawa
authors.forename: Takeshi
authors.initials: T
authors.lastname: Echigo
authors.forename: Kazuhiko
authors.initials: K
authors.lastname: Takehara
mesh_terms: D001323:Autoantibodies / Q000276:immunology*; D002502:Centrioles / Q000276:immunology*; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology*; D008875:Middle Aged; D012595:Scleroderma, Systemic / Q000150:complications / Q000276:immunology*
chemical_list: D001323:Autoantibodies