title: Imatinib is partially effective for the treatment of pulmonary capillary hemangiomatosis.
abstract: A 43-year-old man presented with dyspnea on exertion. Right heart catheterization demonstrated pulmonary arterial hypertension (PAH). He was treated with bosentan, sildenafil and intravenous epoprostenol. Despite the administration of such intensive therapy, the patient's condition deteriorated to a World Health Organization functional class (WHO-FC) of IV. He participated in a clinical trial of imatinib for PAH. After three months of treatment with imatinib, the chest X-ray and echocardiography findings improved, and the WHO-FC class was III. One year after, however, the PAH worsened again, and the patient died 2.6 years after the first diagnosis. At autopsy, patchy capillary proliferation was observed in the lungs. The definitive diagnosis was pulmonary capillary hemangiomatosis.
authors.affiliation: Department of Cardiology, Nagoya University Graduate School of Medicine, Japan.
authors.forename: Shiro
authors.initials: S
authors.lastname: Adachi
authors.forename: Akihiro
authors.initials: A
authors.lastname: Hirashiki
authors.forename: Takahisa
authors.initials: T
authors.lastname: Kondo
authors.forename: Masato
authors.initials: M
authors.lastname: Nakaguro
authors.forename: Aiko
authors.initials: A
authors.lastname: Ogawa
authors.forename: Katsumasa
authors.initials: K
authors.lastname: Miyaji
authors.forename: Hiromi
authors.initials: H
authors.lastname: Matsubara
authors.forename: Toyoharu
authors.initials: T
authors.lastname: Yokoi
authors.forename: Toyoaki
authors.initials: T
authors.lastname: Murohara
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use*; D000970:Antineoplastic Agents / Q000008:administration & dosage*; D001344:Autopsy; D001549:Benzamides / Q000008:administration & dosage*; D000077300:Bosentan; D006328:Cardiac Catheterization; D004417:Dyspnea / Q000209:etiology; D004452:Echocardiography; D011464:Epoprostenol / Q000008:administration & dosage; D017809:Fatal Outcome; D018324:Hemangioma, Capillary / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications* / Q000188:drug therapy; D000068877:Imatinib Mesylate; D008168:Lung / Q000473:pathology* / Q000503:physiopathology; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology; D008297:Male; D010879:Piperazines / Q000008:administration & dosage*; D011687:Purines / Q000008:administration & dosage; D011743:Pyrimidines / Q000008:administration & dosage*; D013902:Radiography, Thoracic; D000068677:Sildenafil Citrate; D013449:Sulfonamides / Q000008:administration & dosage; D017211:Treatment Failure
chemical_list: D000959:Antihypertensive Agents; D000970:Antineoplastic Agents; D001549:Benzamides; D010879:Piperazines; D011687:Purines; D011743:Pyrimidines; D013449:Sulfonamides; D000068877:Imatinib Mesylate; D000068677:Sildenafil Citrate; D011464:Epoprostenol; D000077300:Bosentan