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Pulmonary Hypertension Research
This page was written by Shelley Ensz, and has not been medically edited. Scleroderma (SD) affects everyone differently. Just because something is listed here does not mean an individual patient will ever experience it. See Disclaimer
Overview of PH Research
Antibodies
Causes of PH
Ethnic Disparities
Ineffective Therapies
Prevalence
Overview of Pulmonary Hypertension Research
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
Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Immunoglobulin G from patients with idiopathic pulmonary arterial hypertension or scleroderma-associated pulmonary arterial hypertension express distinct reactivity profiles with fibroblasts antigens, suggesting distinct target antigens. PubMed. Eur Respir J. 2006 Oct;28(4):799-807. (Also see: Antibodies )
Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury. We found that the prevalence of antiphospholipid antibodies in SSc patients was low. However, anticardiolipin (aCL) antibodies were associated with PAH and endothelial injury. PubMed. Clin Exp Rheumatol. 2005 Mar-Apr;23(2):199-204. (Also see: Antiphospholipid Syndrome)
Relationship of Pulmonary Hypertension with Level of Skin Involvement and Autoantibody Profile in Systemic Sclerosis. Positive anti Scl-70 antibodies were significantly associated with signs of pulmonary hypertension in our patients. However, there was no correlation among extension of skin involvement and presence of ACA on one side, and signs of pulmonary hypertension and restrictive lung disease on the other. M. B. Zlatanovic. FRI0116 EULAR 2005. (Also see: Antibodies)
A clue for telangiectasis in systemic sclerosis: elevated serum soluble endoglin levels in patients with the limited cutaneous form of the disease. Pulmonary artery pressure was positively correlated with sEndoglin levels in patients with limited scleroderma. PubMed. Dermatology. 2006;213(2):88-92. (Also see: Telangiectasia, Limited Scleroderma, and Causes of Scleroderma: Molecular Defect )
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.
Methamphetamine use and pulmonary arterial hypertension (PAH). “We are seeing more and more PAH patients come into the clinic that are current or former methamphetamine users." Medical Health News and Articles. 03/25/08.
Pulmonary complications from cocaine and cocaine-based substances: imaging manifestations. A wide variety of pulmonary complications including interstitial pneumonitis, fibrosis, pulmonary hypertension, alveolar hemorrhage, asthma exacerbation, barotrauma, thermal airway injury, hilar lymphadenopathies, and bullous emphysema may be associated with the inhalation of crack cocaine or of associated substances such as talc, silica, and lactose. (PubMed) Radiographics. 2007 Jul-Aug;27(4):941-56.
Antidepressants May Harm Infants' Lungs. Expectant mothers who took antidepressants like Prozac late in their pregnancy were significantly more likely to give birth to an infant with a rare but serious breathing problem, pulmonary hypertension. The New York Times. 02-09-06. (Also see: Scleroderma and Pregnancy, and Depression)
Discovery Links Blood Cell Defect To Common Lung Disorder, Leads To Novel Treatment. Howard Hughes Medical Institute researchers at Duke University Medical Center have found through studies in people and animals that inhalation of a 'souped up' form of nitric oxide, which targets red blood cells, reverses the blood abnormality to restore normal lung pressure. Science Daily 10-11-05.
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. Do Calcium Channel Blockers Protect Patients with Systemic Sclerosis from Developing Pulmonary Arterial Hypertension? The previously established protective effect of prior CCB treatment for the development of PAH in SSc could not be confirmed in our cohort of prospectively followed patients with SSc. M. C. Vonk. FRI0082 EULAR 2005.
Immunosuppressives do not prevent pulmonary hypertension in scleroderma patients. Is Prior Immunosuppresive Therapy Protective for the Development of Pulmonary Arterial Hypertension in Patients with Systemic Sclerosis? We found no evidence of a protective effect of prior use of immunosuppressive agents for the development of PAH. This finding is consistent with recent data from the literature. M. C. Vonk. FRI0081 EULAR 2005.
Pulmonary involvement in systemic sclerosis due to therapy and as a complication. We report a case of a patient affected by SS, with isolated pulmonary hypertension, who developed bronchiolitis secondary to penicillamine. PubMed. Monaldi Arch Chest Dis. 2005 Jun;63(2):111-3. (Also see: Ineffective and Unproven Treatments: Penicillamine)
Prevalence of PH/PAH in Scleroderma (Systemic Sclerosis, SSc)
Prevalence of Pulmonary Arterial Hypertension in a Prospectively Followed Cohort of Patients with Systemic Sclerosis (SSc). The prevalence of PAH in 15% of this cohort of patients with SSc is similar to the prevalence of this complication reported in recent literature. Diminished pulmonary function test results, especially KCO, can be of value for early detection of PAH in SSc. M. C. Vonk. FRI0076 EULAR 2005.
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