title: Severe pulmonary hypertension associated with primary Sjögren's syndrome.
abstract: Severe pulmonary hypertension is one of the fetal complications in various connective tissue diseases. We report a case of severe pulmonary hypertension associated with primary Sjögren's syndrome. In a lung biopsy specimen, there were findings of intimal and medial hypertrophy with narrowing vessel lumina and plexiform lesions. Moreover, deposits of immunoglobulin M, immunoglobulin A and complement protein C1q were found in the pulmonary arterial walls. Although pulmonary hypertension was refractory to oral prostacyclin, steroid therapy improved the clinical and hemodynamic conditions. In the present case, the immunological etiology may be related to the mechanisms of pulmonary hypertension associated with Sjögren's syndrome.
authors.affiliation: First Department of Medicine, Asahikawa Medical College, Asahikawa.
authors.forename: Naoki
authors.initials: N
authors.lastname: Nakagawa
authors.forename: Shinobu
authors.initials: S
authors.lastname: Osanai
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ide
authors.forename: Yutaka
authors.initials: Y
authors.lastname: Nishigaki
authors.forename: Saori
authors.initials: S
authors.lastname: Takahashi
authors.forename: Hitoshi
authors.initials: H
authors.lastname: Nakano
authors.forename: Yoshinobu
authors.initials: Y
authors.lastname: Ohsaki
authors.forename: Kenjiro
authors.initials: K
authors.lastname: Kikuchi
authors.forename: Yoshihiko
authors.initials: Y
authors.lastname: Tokusashi
authors.forename: Hiromi
authors.initials: H
authors.lastname: Obata
mesh_terms: D000328:Adult; D000936:Antigen-Antibody Complex / Q000378:metabolism; D015922:Complement C1q / Q000378:metabolism; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism / Q000473:pathology; D007070:Immunoglobulin A / Q000378:metabolism; D007075:Immunoglobulin M / Q000378:metabolism; D007150:Immunohistochemistry; D008168:Lung / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011651:Pulmonary Artery / Q000378:metabolism / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications* / Q000378:metabolism
chemical_list: D000936:Antigen-Antibody Complex; D005938:Glucocorticoids; D007070:Immunoglobulin A; D007075:Immunoglobulin M; D015922:Complement C1q; D011239:Prednisolone