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Pulmonary Hypertension Research

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview
Antibodies
Ineffective Therapies
Prevalence

Overview

Active research for pulmonary hypertension is largely focusing on causes, antibodies, and treatments. (Also see Pulmonary Hypertension Overview, Scleroderma Lung Involvement, What is Scleroderma?, Types of Scleroderma, and Systemic Sclerosis)

Recent advances in scleroderma-associated (SSc) - pulmonary hypertension. Although there have been many recent advances in SSc-pulmonary hypertension, further research is needed in order to prevent/cure this deadly complication. PubMed, Curr Opin Rheumatol.

Antibodies

Interferon and alternative activation of macrophage/monocytes in systemic sclerosis-associated pulmonary arterial hypertension. This study explores the relationship between biomarkers of pulmonary arterial hypertension (PAH), interferon-regulated gene expression and alternatively activated macrophages (AAM) in systemic sclerosis (SSc). Romy B. Christmann MD, PhD, Arthritis and Rheumatism.

Ineffective Therapies

Calcium channel blockers do not protect scleroderma patients from developing pulmonary hypertension.

Immunosuppressives do not prevent pulmonary hypertension in scleroderma patients.

Prevalence of PH/PAH in Scleroderma (Systemic Sclerosis, SSc)

Elevated levels of pentraxin 3 (PTX3) in systemic sclerosis (SSc): associations with vascular manifestations and defective vasculogenesis. In SSc patients, exposure to high concentrations of PTX3 may suppress EPC–mediated vasculogenesis and promote vascular manifestations such as digital ulcers and pulmonary arterial hypertension. PubMed, Arthritis Rheumatol, 2015 Feb;67(2):498-507. (Also see Research on Digital Ulcers)

Prevalence of pulmonary arterial hypertension (PAH) in patients with connective tissue diseases: a systematic review of the literature. Prevalence of PAH in patients with connective tissue diseases was substantially higher than that of idiopathic PAH based on pooled prevalence estimates. PubMed, Clin Rheumatol. (Also see Connective Tissue Disease)

"PAH in SSc occurs in a significant proportion of (systemic scleroderma) patients without any "red flag signs" in early stages." Journal of Association of Physicians of India.

Go to Secondary Pulmonary Hypertension
 

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