Causes of Pulmonary Hypertension

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Altitude and Hypoxia
Antiphospholipid Antibodies
Endothelial Dysfunction
Estrogen and Enzyme CYP1B1
Vascular Disease/Scleroderma


There are dozens of causes of pulmonary hypertension, ranging from genetics, medications or street drugs, connective tissue diseases (such as scleroderma), heart and lung diseases, and blood disorders.

Causes of Pulmonary Hypertension. Depending on the type, pulmonary hypertension can have many potential causes. MayoClinic.

Altitude and Hypoxia

Effects of Iron Supplementation and Depletion on Hypoxic Pulmonary Hypertension Two Randomized Controlled Trials. Hypoxia is a major cause of pulmonary hypertension in respiratory disease and at high altitude. Hypoxic pulmonary hypertension may be attenuated by iron supplementation and exacerbated by iron depletion. Thomas G. Smith, MBBS, DPhil. JAMA. (Also see Hypoxia, and Vitamins/Minerals)

Antiphospholipid Antibodies (APLA)

Prevalence and Clinical Associations of Antiphospholipid Antibodies in Systemic Sclerosis: New Data From a French Cross-Sectional Study, Systematic Review, and Meta-Analysis. These data provide additional insights into the role of aPL in the vasculopathy observed in SSc. PubMed, Front Immunol, 2018 Nov 2;9:2457. (Also see Antiphospholipid Syndrome, Raynaud's and Digital Ulcers)

Endothelial Dysfunction

Endothelin and Systemic Scleroderma. Endothelin is a protein that helps to regulate contraction and relaxation of the smooth muscle found in the walls of blood vessels and internal organs (except the heart). An overproduction of endothelin, whether by illness or medication, can cause blood vessels to constrict and blood pressure to increase. In scleroderma, it can cause pulmonary arterial hypertension (PAH). ISN.

Endothelin and Systemic Scleroderma Endothelin Receptor Antagonists

Estrogen and Enzyme CYP1B1

Estrogen Metabolites in a Small Cohort of Patients With Idiopathic Pulmonary Arterial Hypertension (iPAH). We show that the estradiol and metabolite profile is altered in iPAH. PubMed, Pulm Circ, 2020 Mar 13;10(1):2045894020908783.


Genes behind deadly heart condition found, scientists say. Scientists say they have identified genes that cause a deadly heart condition that can only be cured by transplants of the heart or lungs. BBC News, 04/15/2018.


Fli1 deficiency induces endothelial adipsin expression, contributing to the onset of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Adipsin is up-regulated at least partially by Fli1 deficiency in endothelial cells, potentially contributing to the development of pulmonary vascular involvement in SSc. PubMed, Rheumatology (Oxford), 2020 Aug 1;59(8):2005-2015.

Medications that Cause Pulmonary Hypertension

Dasatinib-induced pulmonary arterial hypertension. Dasatinib-induced PAH usually seems to be reversible with the cessation of the drug, and sometimes with PAH-specific treatment strategies. PubMed, BR J Clin Pharmacol, 2018 Jan 15.

Vascular Disease and Scleroderma

Vascular Disease and Systemic Scleroderma. The vascular system is our circulatory system. It consists of all the vessels that carry blood and lymph through the body, to and from organs. Vessels include arteries, veins, and capillaries. Any condition that affects the vascular system, all of in part, is considered a vascular disease. ISN.

Vascular in Scleroderma
Types in Scleroderma
In Other CTDs
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Related Information
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