Australian Scleroderma Interest Group (ASIG)

The Australian Scleroderma Interest Group (ASIG) is a multidisciplinary collaboration of rheumatologists, immunologists, cardiologists and respiratory physicians around Australia with a special interest in improving the outcomes of patients with systemic sclerosis (SSc), commonly known as scleroderma, and the related scleroderma variant, mixed connective tissue disease (MCTD).

In 2007, ASIG established the Australian Scleroderma Cohort Study (ASCS), using a web-based platform to screen for heart and lung complications of these diseases as a service to patients with scleroderma and their rheumatologists. This is delivered in screening centres around Australia. Patients are screened annually and if they consent, participate in a longitudinal observational cohort that provides a framework for clinical and laboratory research resulting in collaborations with other Australian researchers and internationally.


The primary objective of the ASCS is to enhance clinical care by increasing the rate of screening for SSc-related PAH and ILD to allow earlier identification of patients at high risk, and institution of timely treatment. Another key objective is to identify and quantify risk and prognostic factors for cardiopulmonary outcomes in SSc and MCTD through the systematic prospective collection of clinical and laboratory data. Over time, the aims have expanded to:

Heart and lung complications of systemic sclerosis

SSc is a multi-organ autoimmune disease characterized by vasculopathy and fibrosis with a prevalence of around 20/100 000. Among the rheumatic diseases, SSc is associated with one of the greatest increases in mortality compared with age and sex-matched peers. Collectively, the pulmonary complications of ILD and PAH, are the leading cause of mortality. The use of advanced pulmonary vasodilators for PAH improves functional class, exercise capacity, haemodynamics, quality of life and survival. Patients who undergo screening for PAH in specialized clinics have better survival compared with those who are found to have SSc-PAH during routine clinical care.

PAH occurs in approximately 10% of patients with SSc but early detection is challenging as the disease is often clinically silent in the early stages. Annual screening of all SSc patients with non-invasive tests, such as transthoracic echocardiography (TTE), is recommended by the American College of Cardiology Foundation / American Heart Association (ACCF/ AHA), the European Society of Cardiology and European Respiratory Society (ESC/ERS) and the National Pulmonary Hypertension centres of the UK and Ireland. The aim of screening is to identify patients at highest risk of developing PAH who should then undergo right heart catheterization to confirm the diagnosis. TTE and other screening tests such as pulmonary function tests have limitations making risk stratification difficult. Even though a number of screening algorithms to identify patients at high risk have been developed, adherence to these guidelines may be limited by competing demands in the clinic setting. A survey of Australian rheumatologists in 2007 found that less than 40% of physicians screened asymptomatic SSc patients less than annually or not at all, confirming that screening according to an agreed protocol was not consistently offered in Australia.

Referral to Screening Centres

In 2015, PAH screening is delivered in 15 centres across Australia (ASIG screening centres). Most are government-designated PAH treatment centres, in which physicians are able to prescribe PAH-specific therapy according to criteria defined by the Pharmaceutical Benefits Scheme (PBS), although some are based in private practices. All centres agree to the Terms of Reference, to adopt the ASCS Screening and Research Protocol; and have a collaboration with a cardiologist and respiratory physician who agree in writing to provide support to screening, including right heart catheterisation in particular. New centres are welcomed and continue to join the collaboration.

Physicians outside the centres who care for patients with SSc and MCTD are invited to refer patients to the nearest centre for the screening service while ongoing care between screening visits remains their responsibility.

Screening algorithm for early detection of PAH

The screening algorithm (see link 7) for early detection of SSc-PAH is currently based on annual transthoracic echocardiogram, pulmonary function tests and clinical assessment. If abnormal findings suggest increased risk of heart or lung complications, definitive investigations such as right heart catheterisation (RHC) or high resolution CT san of the chest (HRCT chest) are indicated in a patient at high risk of PAH or ILD, respectively.

Enrolment in ASCS

All patients with SSc defined according to ACR/EULAR criteria and MCTD are eligible for enrolment in the ASCS. Patients provide written informed consent for prospective collection of de-identified data, retrospective chart review for specific projects, and collection of blood samples for storage of serum and extraction of DNA for future studies. The ASCS has been approved by the human research ethics committees of all participating centres. Comprehensive demographic and disease-related data are collected annually and entered into a custom-made database.

Structure of ASIG

The Executive Committee, chaired by A/Prof Susanna Proudman, first met in November 2005 and in 2007, it became a committee under the auspice of the Australian Rheumatology Association (ARA).

The Executive Committee comprises:

The ASCS project coordinator and database administrator was appointed in 2006 and is based at St. Vincent’s Hospital Melbourne Department of Rheumatology. ASIG meets by teleconference every two to three months throughout the year. A face to face meeting occurs once a year in conjunction with the ARA Annual Scientific Meeting.

In 2008, a Scientific Committee, chaired by A/Prof Susanna Proudman was established to:

• Devise research strategies for ASIG using ASCS data and patient samples
• Review and approve proposals from other members of ASIG
• Assist with applications for funding to continue the research activities long term
• To engage and supervise PhD student(s) in this field

All ASIG’s members are clinical rheumatologists many without formal academic appointments, who provide their time voluntarily to the group. Nonetheless, by establishing a supportive infrastructure and drawing on the various skills of the individuals, much has been achieved. These achievements owe much to the rheumatologists outside ASIG who have kindly entrusted their patients to ASIG for screening and specialised care if required. This has been the most rewarding collaboration of all.

For further information about ASIG or the contact details for ASIG centres, contact

The ASIG Project Manager:
Or the ASIG Chair:
A/Prof Mandana Nikpour
Department of Medicine and Rheumatology
University of Melbourne
St Vincent’s Hospital
41 Victoria Parade
Tel: +613 9288 2211