Journal
CLINICS IN CHEST MEDICINE
Volume 34, Issue 4, Pages 753-+Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.ccm.2013.08.005
Keywords
Sarcoidosis; Lymphangioleiomyomatosis; Pulmonary Langerhans cell histiocytosis; Renal failure; Splenectomy; Fibrosing mediastinitis; Myeloproliferative disorders; Metabolic disorders
Categories
Funding
- Pfizer
- Gilead
- Actelion
- United Therapeutics
- Novartis
- Medtronics
- Bayer
- Ikaria
- Aires
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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.
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