title: World Health Organization group 5 pulmonary hypertension.
abstract: World Health Organization (WHO) group 5 pulmonary hypertension (PH) entails a heterogeneous group of disorders that may cause PH by unclear and/or multiple mechanisms. In particular, group 5 includes PH caused by hematologic disorders, systemic diseases, metabolic disorders, chronic renal failure, and disorders leading to pulmonary vascular occlusion or compression. This article discusses common pathogenic mechanisms leading to group 5 PH, followed by a detailed overview of epidemiology, pathogenesis, and disease-specific management of the individual group 5 conditions. Off-label use of vasomodulatory therapies, typically indicated for pulmonary arterial hypertension (WHO group 1 PH), in group 5 conditions is also discussed.
authors.affiliation: Division of Pulmonary, Allergy, Critical Care, Occupational and Sleep Medicine, Department of Medicine, Richard L. Roudebush VA Medical Center, Center for Immunobiology, Indiana University School of Medicine, 980 West Walnut Street, Room C400, Indianapolis, IN 46202, USA. Electronic address: tlahm@iu.edu.
authors.forename: Tim
authors.initials: T
authors.lastname: Lahm
authors.forename: Murali M
authors.initials: MM
authors.lastname: Chakinala
mesh_terms: D006801:Humans; D006976:Hypertension, Pulmonary / Q000145:classification* / Q000209:etiology / Q000628:therapy; D013156:Splenectomy / Q000009:adverse effects; D014665:Vasodilator Agents / Q000627:therapeutic use; D014944:World Health Organization
chemical_list: D014665:Vasodilator Agents
keywords: Fibrosing mediastinitis; Lymphangioleiomyomatosis; Metabolic disorders; Myeloproliferative disorders; Pulmonary Langerhans cell histiocytosis; Renal failure; Sarcoidosis; Splenectomy