Pulmonary arterial hypertension (PAH) is a complex and often misunderstood condition that disproportionately impacts those with systemic sclerosis (scleroderma). 

Dr Zoe Brown, a researcher at the forefront of PAH and systemic sclerosis, is aiming to change the way we diagnose and treat this life-altering condition. 

Funded by Janssen Australia and working in close collaboration with specialists across Australia, Dr Brown is piloting a groundbreaking screening algorithm that promises early detection and better management of PAH in scleroderma patients.

Collaborations and experience

Dr Brown is pushing the envelope by integrating the Australian Scleroderma Interest Group (ASIG) algorithm into her research. 

ASIG, a multidisciplinary team including rheumatologists, immunologists, and geneticists, focuses on enhancing the life quality of systemic sclerosis patients. 

Dr Brown’s approach involves annual respiratory function tests coupled with a specific blood marker test (NT-Pro BNP) for early detection of PAH.

With a rich background in Cardiology at St. Vincent’s Hospital in Melbourne, Dr Brown collaborates with pulmonary hypertension specialists in Queensland and Victoria. 

These partnerships aim to harmonise knowledge and refine treatment practices across different medical landscapes.

In addition, the cost of the essential NT-Pro BNP test will be covered in Australia starting November 1st of the current year, a significant step toward early diagnosis.

Understanding PAH in systemic sclerosis

Pulmonary arterial hypertension (PAH)  is characterised by high blood pressure in the blood vessels that supply the lungs, leading to complications such as stiffening or narrowing of these vessels. 

The symptoms of pulmonary hypertension (PH) are often non-specific and typically manifest in the late stages of the condition. They can include:

  • Fatigue
  • Reduced exercise tolerance or ability
  • Shortness of breath
  • Swelling in the ankles

What causes Pulmonary Hypertension (PH)?

  • Scleroderma: This autoimmune disease can lead to the development of PAH.
  • Idiopathic PAH: Sometimes, the cause of PAH remains unknown.
  • Heart Disease: Various types of heart disease can contribute to pulmonary hypertension, affecting the heart’s ability to pump blood effectively.
  • Emphysema: This lung condition can restrict airflow and contribute to PH.
  • Interstitial Lung Disease (ILD): Scarring and inflammation of the lung tissue can lead to PH.
  • CTEPH (Chronic Thromboembolic Pulmonary Hypertension): This is a type of PH caused by long-standing blood clots in the lungs.

For a comprehensive understanding of pulmonary hypertension, including diagnosis and treatment options, you may refer to the 2022 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension.

While PAH occurs in less than 1% of the general population, an alarming 1 in 10 people with scleroderma live with this condition according to data from the Australian Scleroderma Cohort Study (ASCS).

Findings from ASCS also reveal that PAH usually develops more than four years following a diagnosis of scleroderma.

Diagnosis vs. screening


Screening involves routinely applying a particular test or investigation to people who don’t show symptoms of the condition being examined. A positive result from a screening test doesn’t confirm the disease but suggests that further diagnostic tests are needed.


  • Mammograms for detecting breast cancer
  • Stool tests for identifying colorectal cancer


Diagnosis, on the other hand, is the application of investigative procedures that confirm a condition definitively.

One of the biggest challenges in diagnosing PAH is the slow onset of symptoms like reduced exercise tolerance and shortness of breath, which are often confused with other conditions like interstitial lung disease or anaemia.

While diagnostic tests like right-heart catheterisation provide definitive PAH diagnoses, Dr Brown emphasises the value of early screening tests. Early diagnosis is particularly vital in scleroderma patients, as it has been shown to lead to significantly better outcomes.


Treatment for PAH has evolved substantially over the last 25 years, with over 40 clinical trials shaping today’s treatment protocols. 

Current medications target the vasoconstriction or narrowing of blood vessels, with a focus on three chemical pathways: Nitric oxide, prostacyclin, and endothelin. These medications not only improve heart function but also enhance the patient’s overall quality of life.

Early detection and improved screening

What’s involved in Annual Screening for PAH?

Annual screening for PAH typically involves the following tests:

  • Heart Ultrasound/Echocardiography: To examine the heart’s structure and function
  • Respiratory Function Test: To assess lung function
  • Blood Test (NT-proBNP): To measure levels of the N-terminal prohormone of brain natriuretic peptide, which can be elevated in PAH

Advantages of Early Screening for PAH

Here’s why screening matters:

  • Earlier Diagnosis: Catches the condition before it advances
  • Less Advanced PAH: When caught early, the condition is generally less severe
  • Earlier Introduction of Treatments: Enables starting treatment plans sooner
  • Improved Outcomes: Leads to better health prospects overall

ASCS and Dr Brown are working toward more accurate and cost-effective screening methods, particularly for those in remote regions where access to specialised tests is limited. 

The new screening algorithm promises not just to identify PAH earlier but also to be more cost-effective than existing methods.

Challenges and goals

Access to specialised tests remains a challenge, especially in rural and remote areas. 

The overarching goal is to increase awareness and accessibility to PAH screening across Australia and improve early identification and management of this often-overlooked condition.

Dr Zoe Brown’s work is a beacon of hope for those struggling with PAH and systemic sclerosis. 

Her collaborative approach, the support from Janssen, and her incorporation of the ASIC algorithm herald a future where PAH can be diagnosed earlier and managed more effectively, promising a better quality of life for patients.

For those seeking more information on pulmonary hypertension and other lung diseases, Dr Brown recommends the Lung Foundation Australia’s website as an excellent resource. 

By demystifying PAH and advocating for early detection and targeted treatment, Dr Brown’s work has the potential to change countless lives for the better.