title: Pulmonary arterial hypertension associated with IgG4-related disease.
abstract: A 22-year-old woman with generalized lymphadenopathy and symmetrical swelling of the lacrimal and submandibular glands was diagnosed with IgG4-related disease. Biopsy specimens of the lips, lymph nodes, gastrointestinal tract and bronchus showed IgG4-positive plasma cell infiltration. Echocardiography and right heart catheterization revealed a high mean pulmonary arterial pressure. The patient was treated with 50 mg of prednisolone daily and rapidly improved. This is the first reported case of pulmonary arterial hypertension associated with IgG4-related disease.
authors.affiliation: Department of Internal Medicine and Rheumatology, Clinical Research Institute, National Hospital Organization Kyushu Medical Center, Japan.
authors.forename: Motoko
authors.initials: M
authors.lastname: Ishida
authors.forename: Tomoya
authors.initials: T
authors.lastname: Miyamura
authors.forename: Shinji
authors.initials: S
authors.lastname: Sato
authors.forename: Daisaku
authors.initials: D
authors.lastname: Kimura
authors.forename: Eiichi
authors.initials: E
authors.lastname: Suematsu
mesh_terms: D000888:Antibodies, Anti-Idiotypic / Q000276:immunology*; D001327:Autoimmune Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D006976:Hypertension, Pulmonary / Q000209:etiology* / Q000276:immunology / Q000503:physiopathology; D007074:Immunoglobulin G / Q000276:immunology*; D008206:Lymphatic Diseases / Q000150:complications* / Q000188:drug therapy / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D055815:Young Adult
chemical_list: D000888:Antibodies, Anti-Idiotypic; D005938:Glucocorticoids; D007074:Immunoglobulin G; D011239:Prednisolone