Diseases of the immune system
Scleroderma
IMPORTANT INFORMATION
The information on the Rare Awareness Rare Education (RARE) Portal is intended for educational purposes only and does not replace professional advice.
Rare diseases typically display a high level of symptom complexity and variability. Individuals diagnosed with the same rare disease may be impacted differently and each person’s experience is unique. Please seek support from qualified healthcare professionals to learn more about the most suitable care and support options for you.
For more information on this disease, please refer to RVA Partner, Scleroderma Australia.1
If you are aware of any additional information that may benefit stakeholders with an interest in this page, or if you notice any broken links or inaccurate information, please let us know via the Contribute page.
Contributors
Information for this page has been supplied by RVA Partner, Scleroderma Australia.1
Quick Page Search:
Emergency Management | Clinical Care Guidelines | Synonyms | Summary | Personal Stories | Symptoms | Cause/Inheritance | Diagnosis | Treatment | Clinical Care | Research and Data | Rare Disease Organisation(s) | Support Services/Resources | Mental Health | Other | References
Emergency Management
There may be special considerations for the emergency management of individuals living with scleroderma presenting to emergency departments.
The following hospital care plan documents have been published by Scleroderma Australia1, with information produced in collaboration with Scleroderma/Lupus Resource Centre at the Royal Newcastle Hospital, NSW:
- Scleroderma Australia: A guide to using the Hospital Checklist Care Plan
- Scleroderma Australia: Hospital Checklist – Care Plan for Person with Scleroderma
Clinical Care Guidelines
Information for referring practitioners can be found here for some states:
- Queensland – Queensland Health: Clinical Prioritisation Criteria for Connective Tissue Disease – SLE, Scleroderma, MCTD, Sjogren’s Syndrome and undifferentiated or overlap CTDs
- Tasmania – Tasmanian Health Organisation: Scleroderma – Outpatient Clinics
International clinical care guidelines:
- BSR and BHPR guideline for the treatment of systemic sclerosis – developed by a representative group from the British Society of Rheumatology (BSR) and British Health Professionals in Rheumatology (BHPR)
Synonyms
ORPHA:801 Scleroderma
- ORPHA:90289 Localised scleroderma
- ORPHA:90291 Systemic sclerosis
ICD11: 4A42 Systemic sclerosis
ICD11: EB61.0 Plaque morphoea
Summary
Scleroderma is an autoimmune condition that affects connective tissues1, which joins and supports other tissues and organs in the body. ‘Scleroderma’ comes from the Greek words ‘skleros’ (hard) and ‘derma’ (skin), as this condition is often characterised by the hardening or thickening of skin, and in some cases, internal organs as well.1,2 This is caused by a reaction of the body’s immune system resulting in too much collagen, an important protein of the connective tissue, being produced. This overproduction of collagen can lead to scarring and damage to the skin and internal organs, such as the lungs, heart, kidneys, oesophagus and digestive system (gastrointestinal tract).1
Scleroderma can affect both men and women but is more common in women.1,2 It can occur at any age, particularly between 40-60 years, but is less likely in children. Scleroderma is not contagious or infectious.1 It is also not cancerous or malignant, but it can be life-threatening1, particularly if it affects the internal organs such as the lungs, heart, and kidney.
There are two main forms of scleroderma3:
- Systemic scleroderma, which affects the skin and internal organs
- Localised scleroderma, which affects the skin and, in some cases, the tissues or muscles beneath the skin but not internal organs
Please visit the Systemic Scleroderma page and Localised Scleroderma page on the RARE Portal for specific information about the different forms of scleroderma.
More information about scleroderma can be found at: Scleroderma Australia: Understanding and Managing Scleroderma.
Useful Links for Professionals
Scleroderma Australia: Resources – brochures with information relevant to medical professionals
Australian Scleroderma Interest Group (ASIG): Scleroderma fact sheet
Red flags in scleroderma (article reprinted from Australian Family Physician)
Symptoms
Please visit the Systemic Scleroderma page and Localised Scleroderma page on the RARE Portal for more information about the symptoms for the different forms of scleroderma.
Cause/Inheritance
Scleroderma is an autoimmune condition which causes the body to overproduce collagen. It is generally classified as one of the autoimmune rheumatic diseases.3 The exact cause of the autoimmune reaction is unknown.
Diagnosis
Diagnosis of scleroderma may be made based on clinical examination of symptoms/disease presentation, medical history and findings from laboratory tests and studies. It is important not just to confirm the diagnosis of scleroderma, but to determine the extent and severity of the disease, particularly whether there is involvement of internal organs.3
Please visit the Systemic Scleroderma page and Localised Scleroderma page on the RARE Portal for more information about specific diagnostic tests for the different forms of scleroderma.
Treatment
There is currently no curative treatment for scleroderma, but there are strategies to assist in the management of symptoms. This may involve different types of medication to suppress the immune system and to treat specific symptoms.1,4 Physiotherapy and exercise may help with joint mobility and muscles.3
Monitoring and regular assessment for red flags and the involvement of internal organs is important, as early treatment is more effective.3,4
Please speak with your medical team to learn more about management strategies for scleroderma and its associated symptoms. Treatment will depend on an individual’s specific symptoms.
Clinical Care
Healthcare professionals involved in the treatment of scleroderma may include general practitioners (GP), rheumatologists, dermatologists, pulmonologists, cardiologists, nephrologists, gastroenterologists, wound care and continence nurses, social workers, occupational therapists, physiotherapists, psychologists, and dentists.1 The need for different healthcare professionals may change over a person’s lifetime and extend beyond those listed here.
The Australian Scleroderma Interest Group (ASIG) is a collaboration of rheumatologists who operate multidisciplinary scleroderma centres around Australia.
- ASIG: Centres lists the available scleroderma centres around Australia
- ASIG: Screening Centre Locator shows available scleroderma screening centres by map
Research and Data
Information about research on scleroderma in Australia can be found at Scleroderma Australia: Australian research into Scleroderma.
The Australian Scleroderma Interest Group (ASIG) is a collaboration of rheumatologists, who in addition to operating multidisciplinary scleroderma centres around Australia, have designed the Australian Scleroderma Cohort Study (ASCS), aimed at creating a national database about people with scleroderma and mixed connective tissue disease (MCTD). They have also established a bio-bank to collect and store biological specimens from patients. More information about the ACSC and the bio-bank can be found at ASIG: ASCS Demographic & Biobank.
Scleroderma Australia: Clinical Trials Understanding Scleroderma provides an in-depth overview of what clinical trials may be like for those living with scleroderma.
Please visit Australian Clinical Trials to learn about clinical trials for scleroderma in Australia; there may not be any clinical trials currently available.
Information regarding clinical trials for scleroderma in other countries can be found at ClinicalTrials.gov; there may not be any clinical trials currently available.
It is best to discuss your interest in any clinical trials with your medical team to determine suitability and eligibility.
Rare Disease Organisation(s)
Scleroderma Australia
Website: https://www.sclerodermaaustralia.com.au/
Phone: 02 9990 5159
Email: [email protected]
Contact form: https://www.sclerodermaaustralia.com.au/contact-us/
Scleroderma Australia is the national representative voice in supporting the scleroderma community across Australia, with its governing committee made up of delegates from different states. Scleroderma Australia also produces a number of informative brochures that explain aspects of scleroderma for medical professionals, people living with scleroderma, their families and friends. These free resources can be found at: Scleroderma Australia: Free brochures and information.
There are different state organisations that provide support to people living with scleroderma:
The Scleroderma Association of New South Wales
Website: https://www.sclerodermansw.org/
Phone: 02 9798 7351
Email: [email protected]
Scleroderma Association of New South Wales provides support for those living in New South Wales, and the Australian Capital Territory.
Scleroderma Victoria
Website: https://sclerodermavictoria.com.au/
Phone: 03 9231 3651
Email: [email protected]
Scleroderma Victoria provides support for those living in Victoria, South Australia, Tasmania, and Western Australia.
Scleroderma Association of Queensland
Website: https://www.scleroderma.org.au/
Phone: 0468 801 021
Email: [email protected]
Scleroderma Queensland provides support for those living in Queensland, and the Northern Territory.
Please note that RVA does not necessarily monitor or endorse each group/organisation’s operational governance.
Support Services/Resources
Scleroderma Australia: Understanding Scleroderma and the NDIS provides information to help people with scleroderma apply for funding through the National Disability Insurance Scheme (NDIS) for support and services.
Please visit the Disability Support Information page on the RARE Portal for more information on disability supports, and the National and State Services pages for general information on other social services.
Mental Health
Please visit the ‘Mental Health’ sections listed on the National and State Services pages.
Other
Further information on scleroderma can be found at:
- Scleroderma Australia: Resources (Free brochures and information)
- healthdirect: Scleroderma
- Arthritis Australia: Scleroderma
- National Organization for Rare Disorders (NORD): Scleroderma
References
- Scleroderma Australia. Accessed 3 October 2023. https://www.sclerodermaaustralia.com.au/
- Australian Scleroderma Interest Group. Scleroderma: more than skin deep. 2019. 1 p. https://www.sclerodermaaustralia.com.au/wp-content/uploads/2020/05/ASIG_Fact_Sheet.pdf
- Scleroderma Australia. Understanding and Managing Scleroderma 6th 2021. 19p. https://www.sclerodermaaustralia.com.au/wp-content/uploads/2021/07/Understanding-and-Managing-Scleroderma-6th-Edition.pdf
- Li Q, Sahhar J, LittleJohn G. Red flags in scleroderma. Aust. Fam. Physician. 2008; 37(10): 831-834. https://www.racgp.org.au/getattachment/a6c7dcc0-3c03-49f7-a766-6125cd5c2510/200810Li.pdf
Page Last Updated
30/01/2024 12:19
