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Pulmonary Hypertension
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Pulmonary Hypertension Research

Author: Shelley Ensz. Scleroderma is highly variable. See Types of Scleroderma. Read Disclaimer
Overview of PH Research
Antibodies
Causes of PH
Ethnic Disparities
Ineffective Therapies
Prevalence

Overview of Pulmonary Hypertension Research

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)
New study will explore impact of exercise on pulmonary hypertension (PAH). The objective of this study will be finding identifiers that separate PAH patients who will benefit from exercise from those whose condition will worsen from it. University of Wisconsin-Madison News. 08/26/11.
Central command system for pulmonary hypertension discovered. "We believe this finding opens the door to a new strategy and the development of a new stream of drugs to treat this complex and lethal disease," said Dr. Evangelos Michelakis. The team has previously shown that dichloroacetate (DCA) also reverses pulmonary hypertension in animal models. News-Medical.Net, 07-05-07.

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, 18 MAR 2011.

Causes of Pulmonary Hypertension

What Causes Pulmonary Hypertension? Certain factors appear to increase your chances of developing pulmonary arterial hypertension (PAH). National Heart, Blood and Lung Institute.
Inflammation and Pulmonary Hypertension (PH). Evidence is accumulating to suggest that inflammation plays a significant role in the pathogenesis of PH. Endothelial cells play an important role in inflammation and immune reactions, and inflammatory cytokines cause endothelial dysfunction. (AHA) R. Mathew, MD. Cardiology in Review. March/April 2010.
Significance of serum uric acid (UA) in pulmonary hypertension (PH) due to systemic sclerosis: a pilot study. Among patients with PH, UA values were inversely correlated with the SMWT (6-minute walk test) distance. Serum UA values increased in proportion to the functional capacity in PH patients with scleroderma.T Dimitroulas. (PubMed) Rheumatol Int. 2010 Jul 25.

Ethnic Disparities

Ethnic Disparities Among Patients with Pulmonary Hypertension Associated with Systemic Sclerosis. In this cohort of patients, African Americans were more likely to have diffuse scleroderma. Among patients with pulmonary hypertension, African Americans presented at a younger age than their Caucasian counterparts. Incidence of diastolic dysfunction was higher in the pulmonary hypertension population. J Rheumatol 2007 June;34:1277-82. (Also see: Types of Scleroderma, Diffuse Scleroderma, and Causes of Scleroderma: Race)

Ineffective Therapies

Calcium channel blockers do not protect scleroderma patients from developing pulmonary hypertension
Treatment of Connective Tissue Disease-associated Pulmonary Arterial Hypertension - Where Do New Oral Therapies Fit In? There is no evidence supporting the use of calcium channel blockers (CCBs) in SSc-PAH. Acute vasodilator responses are rare in SSc-PAH,15 and in the few patients in whom these are observed there is to date no evidence that a sustained response occurs. European Respiratory Disease 2007 - Issue I.
Immunosuppressives do not prevent pulmonary hypertension in scleroderma patients.
Immunosuppressive Therapy in Connective Tissue Diseases-Associated Pulmonary Arterial Hypertension. No patients with systemic sclerosis responded, while 5 of 12 patients with SLE and 3 of 8 patients with MCTD did respond. PAH associated with SLE or MCTD might respond to a treatment combining glucocorticosteroids and cyclophosphamide. CHEST July 2006 vol. 130.

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

Prevalence and Predictors of Pulmonary Artery Hypertension (PAH) in Systemic Sclerosis (SSc). PAH was found in 32% patients on 2D-echocardiography. Prevalence of PAH did not differ between patients with limited cutaneous SSc (1cSSc) and patients with diffuse cutaneous SSc (dcSSc). PAH in SSc occurs in a significant proportion of patients without any "red flag signs" in early stages. Non-invasive screening of patients with SSc for PAH will help in early diagnosis and appropriate timely therapeutic intervention before significant end-organ damage occurs. Journal of Association of Physicians of India, June 2008.
 
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