2022 Western Medical Research Conference



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New Insights Into Systemic Lupus Erythematosus-associated Pulmonary Arterial Hypertension

A team from Peking Union Medical College Hospital in Beijing, China, have explored changes in the characteristics, treatment, and 5-year survival for SLE-associated PAH in the last decade, and possible reasons for survival change. To date, this is the largest multi-center prospective SLE-PAH cohort to describe disease characteristics and prognosis. The results have been shared at the 2023 annual congress of EULAR—the European Alliance of Associations for Rheumatology.

A multi-center prospective cohort of 610 patients with SLE-associated PAH was established, and divided into cohort A and B according to the date that people underwent a right heart catheterization procedure. Cohort A was 2011 to 2016, and cohort B was 2016 to 2021. In tandem, a single-center cohort of 104 idiopathic pulmonary arterial hypertension (IPAH) was recruited as a control group in order to describe baseline characteristics and survival. Disease characteristics, treatment regimen, and all-cause mortality were compared between cohorts A and B.

In their abstract, Dong and colleagues report that SLE PAH patients were overall younger, predominantly female, and had lower levels of NTproBNP—an important cardiac biomarker. They also had better functional status, better hemodynamics, and higher 5-year survival than IPAH patients.

Compared with cohort A, those in cohort B showed lower mean pulmonary arterial pressure and pulmonary vascular resistance. They were also more likely to receive intensive immunosuppressants and PAH targeted medication. The 5-year survival rate was higher in cohort B: 88.1% versus 72.9% in cohort A. Analysis showed that treatment goal achievement of PAH and reaching lupus low disease activity state (LLDAS) were both independently associated with lower mortality.

This study shows that survival has improved significantly for SLE-associated PAH. The findings demonstrate that early detection of PAH in SLE patients and achieving treatment goals for both PAH and SLE contribute to improved survival.

More information: Conference: congress.Eular.Org/

Provided by European Alliance of Associations for Rheumatology (EULAR)

Citation: New insights into systemic lupus erythematosus-associated pulmonary arterial hypertension (2023, June 2) retrieved 2 June 2023 from https://medicalxpress.Com/news/2023-06-insights-lupus-erythematosus-associated-pulmonary-arterial.Html

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Pulmonary Hypertension Warning Signs: What To Look Out For

Pulmonary hypertension image © Provided by Getty Images Pulmonary hypertension image

Overview

A condition in which high blood pressure affects arteries of the lungs, and right side of the heart. This results in chest pain and shortness of breath.

Symptoms

If you or someone you know is exhibiting symptoms of a Pulmonary hypertension, seek medical attention immediately

The symptoms include

→ Common treatment options

→ How is this diagnosed?

Causes

There are several causes and may include:

  • Parasitic infection – schistosomiasis, blood vessels in the lungs becomes infected with the parasite and causes pulmonary arterial hypertension
  • Back flow of blood – stacking of blood can lead to this condition
  • Drugs or medication: certain Drugs like dexfenfluramine (Redux) and Fen/Phen can cause this condition
  • Genetic disorders – Caused due to gene mutations
  • Blood clots – blood clots in the lungs can restrict blood flow
  • Tumor – An abnormal growth in the lungs might compress the arteries and result in reduced blood flow and pressure build up
  • A range of heart or lung diseases can contribute to pulmonary hypertension
  • Chronic pulmonary emboli blockage in the pulmonary arteries
  • Hypoxic pulmonary vasoconstriction might also be the cause of pulmonary hypertension

    • → Do you have a health question on your mind for pulmonary hypertension? Ask professionals from across the world

    → Interested to know more? Check out the full article here


    Is Pulmonary Arterial Hypertension Reversible?

    There is no cure for pulmonary arterial hypertension (PAH). However, treatment can lead to reduced symptoms and improved quality of life.

    PAH occurs when the pressure within the blood vessels linking the heart and the lungs is high. This high pressure can cause the narrowing of tiny arteries in the lungs, restricting blood flow and damaging the right-hand side of the heart.

    Symptoms of PAH may include:

    PAH increases the risk of right heart failure and death. It is extremely rare. According to the American Lung Association, approximately 500-1,000 people receive a diagnosis of PAH each year in the United States.

    Continue reading to learn whether PAH is reversible, what treatment options exist, and the outlook for a person with PAH.

    While there is no cure for PAH, treatment options may improve the quality of life and outcomes in people with the condition.

    Developments in medical therapies have also shown evidence of potentially reversing PAH.

    For example, a 2018 study investigating the effects of a drug on the HIF-2α gene found that the drug effectively reversed right heart failure and vascular remodeling in rodents with PAH. The HIF-2α gene promotes arterial wall thickening, which is a key step in the development of PAH. However, the drug does not cure PAH.

    Vascular remodeling is a change to the structure of the cells and fibroblasts in the arteries.

    However, PAH is often idiopathic, meaning it occurs spontaneously, without any underlying or known cause. Idiopathic PAH or PAH associated with underlying lung or connective tissue disease is more difficult to reverse or treat.

    Treatment options for PAH aim to improve symptoms, increase exercise capacity and slow disease progression. The suitability of a treatment option will depend on a number of factors, including the severity of PAH.

    Medication

    A number of drugs exist to treat PAH. These include:

    Oxygen therapy

    Oxygen therapy involves administering oxygen to a person with low blood oxygen levels.

    A 2021 study found that supplemental oxygen may have therapeutic benefits for people with PAH. This is because oxygen is a pulmonary vasodilator, meaning it expands the vessels of the lungs.

    Lung transplantation

    Lung transplantation involves the replacement of a diseased lung with a healthy lung from a donor. Transplantation may involve the replacement of a single lung or both lungs.

    A small 2018 study found that lung transplantation provides good long-term and short-term survival for individuals with PAH.

    Surgery

    Some surgical options may provide better symptom control and exercise capability in people with PAH.

    For example, atrial septostomy is a surgical procedure that involves creating a hole between the upper chambers of the heart. This allows the blood to flow from the right side of the heart to the left side, reducing pressure on the right side.

    A small 2023 retrospective study involving 12 people with PAH and severe right heart failure found that this surgical option improved survival rates.

    Pulmonary rehabilitation

    Pulmonary rehabilitation is a program that typically involves breathing techniques, exercises, and education to help improve the physical and psychological health of individuals with respiratory conditions and diseases.

    Pulmonary rehabilitation aims to improve physical function, reduce symptom impact, and improve quality of life.

    A 2020 study found that treatment programs that incorporate exercise significantly improve exercise capacity and quality of life in individuals with PAH.

    PAH can cause death if left untreated.

    The mean survival rate for untreated idiopathic PAH is 2-3 years from the PAH diagnosis.

    PAH is a condition that affects the pressure in the blood vessels between the heart and the lungs.

    It might be possible to reverse PAH that has a known cause by treating the underlying condition.

    Treatment options for PAH include medication, surgery, and oxygen therapy.





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