title: Lengthy diagnostic challenge in a rare case of pulmonary veno-occlusive disease: case report and review of the literature.
abstract: Pulmonary veno-occlusive disease (PVOD) is a rare and usually survival poor disorder. We report a patient with a long history of progressive dyspnea of over 8 years, who with a diagnosis of chronic cor pulmonale confirmed elsewhere, was ultimately diagnosed as PVOD via histological analysis of a lung biopsy. After treatment with combined bosentan, diuretics and digoxin, his symptoms and function improved. This case highlights that PVOD is an under-recognised and often misdiagnosed disease, especially in its chronic form. Understanding its pathogenesis, its poor response to medical therapy and its dismal prognosis remain challenges for the treatment of PVOD.
authors.affiliation: Department of Respiratory Diseases, the Second Affiliated Hospital of Nanchang University, China.
authors.forename: Xiao-qun
authors.initials: XQ
authors.lastname: Ye
authors.forename: Chun-song
authors.initials: CS
authors.lastname: Yan
authors.forename: Xin-yi
authors.initials: XY
authors.lastname: Zhang
authors.forename: Yin
authors.initials: Y
authors.lastname: Cai
authors.forename: Feng
authors.initials: F
authors.lastname: Guo
authors.forename: Jiu-long
authors.initials: JL
authors.lastname: Kuang
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000008:administration & dosage; D001706:Biopsy; D000077300:Bosentan; D002316:Cardiotonic Agents / Q000008:administration & dosage; D003937:Diagnosis, Differential; D004077:Digoxin / Q000008:administration & dosage; D004232:Diuretics / Q000008:administration & dosage; D004359:Drug Therapy, Combination; D004417:Dyspnea / Q000209:etiology; D006801:Humans; D006976:Hypertension, Pulmonary / Q000175:diagnosis; D008297:Male; D011660:Pulmonary Heart Disease / Q000175:diagnosis; D011668:Pulmonary Veno-Occlusive Disease / Q000175:diagnosis* / Q000188:drug therapy / Q000503:physiopathology; D013449:Sulfonamides / Q000008:administration & dosage; D013997:Time Factors; D014057:Tomography, X-Ray Computed
chemical_list: D000959:Antihypertensive Agents; D002316:Cardiotonic Agents; D004232:Diuretics; D013449:Sulfonamides; D004077:Digoxin; D000077300:Bosentan