title: Long-term clinical course of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension.
abstract: We report a case of a patient with anti PL-12 antibody accompanied by interstitial pneumonia and severe pulmonary hypertension. At first presentation, hyperkeratotic skin lesions were found, although the diagnosis of CVD was not conclusive. Lung histology showed diffuse fibrosing interstitial pneumonia predominantly in the subpleural regions. During the seven-year follow-up period, severe pulmonary hypertension developed, although the progression of lung fibrosis was relatively limited. Anti-PL12 antibody was detected, and therefore the patient was diagnosed as having antisynthetase syndrome. Lung histology and pulmonary arteriogram suggested that vascular involvement of the disease contributed to the development of severe pulmonary hypertension.
authors.affiliation: Department of Respiratory Medicine, Graduate School of Medicine, Kyoto University, Kyoto.
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Handa
authors.forename: Sonoko
authors.initials: S
authors.lastname: Nagai
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kawabata
authors.forename: Taishi
authors.initials: T
authors.lastname: Nagao
authors.forename: Masaya
authors.initials: M
authors.lastname: Takemura
authors.forename: Masanori
authors.initials: M
authors.lastname: Kitaichi
authors.forename: Takateru
authors.initials: T
authors.lastname: Izumi
authors.forename: Tsuneyo
authors.initials: T
authors.lastname: Mimori
authors.forename: Michiaki
authors.initials: M
authors.lastname: Mishima
mesh_terms: D000328:Adult; D000412:Alanine-tRNA Ligase / Q000276:immunology*; D000792:Angiography; D000906:Antibodies / Q000276:immunology*; D001706:Biopsy; D003095:Collagen Diseases / Q000150:complications / Q000201:enzymology* / Q000473:pathology; D003937:Diagnosis, Differential; D005500:Follow-Up Studies; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000503:physiopathology; D047468:Immunoprecipitation; D017563:Lung Diseases, Interstitial / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D008297:Male; D011651:Pulmonary Artery / Q000000981:diagnostic imaging / Q000473:pathology; D011667:Pulmonary Veins / Q000000981:diagnostic imaging / Q000473:pathology; D011669:Pulmonary Wedge Pressure / Q000502:physiology; D013902:Radiography, Thoracic; D012867:Skin / Q000473:pathology; D013997:Time Factors; D014057:Tomography, X-Ray Computed
chemical_list: D000906:Antibodies; D000412:Alanine-tRNA Ligase