Systemic sclerosis (limited and diffuse scleroderma)
Limited versus diffuse disease, the antibodies that predict ILD, PAH and renal crisis, the annual screening that catches them early, and organ-based treatment from the EULAR 2023 and BSR 2024 guidance.
Tier 1Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- An autoimmune disease with three linked processes: small-vessel vasculopathy (Raynaud, digital ulcers, PAH, renal crisis), autoimmunity (specific antibodies), and fibrosis of skin and organs (ILD, gut, heart).
- Onset usually at 30 to 50, women about four times as often as men. Prevalence in Australia is around 20 per 100,000, among the higher rates reported worldwide (Australian Scleroderma Interest Group cohort).
- It has the highest mortality of the connective tissue diseases. ILD and PAH cause most deaths.
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Sources
- Therapeutic Guidelines: Rheumatology (systemic sclerosis and Raynaud phenomenon)AU/NZTherapeutic Guidelines Limited (eTG complete), current online version, accessed November 2026
- Services Australia: nintedanib for progressive fibrosing interstitial lung disease, PBS authorityAU/NZServices Australia, accessed November 2026
- Pharmaceutical Benefits Scheme: nintedanib for progressive fibrosing interstitial lung disease, item 12967L restrictionAU/NZAustralian Government Department of Health, Disability and Ageing, online schedule, accessed November 2026
- Del Galdo F et al. EULAR recommendations for the treatment of systemic sclerosis: 2023 update. Ann Rheum Dis 2025;84:29-40EULAR, 2023 update (published 2025)
- Denton CP et al. The 2024 British Society for Rheumatology guideline for management of systemic sclerosis. Rheumatology (Oxford) 2024;63(11):2956-75British Society for Rheumatology, 2024
- van den Hoogen F et al. 2013 classification criteria for systemic sclerosis: an ACR/EULAR collaborative initiative. Arthritis Rheum 2013;65:2737-47ACR and EULAR, 2013
- 2023 ACR/CHEST guideline for the screening and monitoring of interstitial lung disease in people with systemic autoimmune rheumatic diseasesAmerican College of Rheumatology and American College of Chest Physicians, 2023 (published 2024)
- Distler O et al. Nintedanib for systemic sclerosis-associated interstitial lung disease (SENSCIS). N Engl J Med 2019;380:2518-28New England Journal of Medicine, 2019
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Deploy to Cloudflare on push to main