What is Systemic Sclerosis / Scleroderma?
Systemic sclerosis is an autoimmune disease that causes thickening and tightening of the skin along with damage to small blood vessels and internal organs. It almost always begins with Raynaud's phenomenon and puffy fingers. Early identification matters because lung and kidney complications are far more treatable when caught before symptoms appear.
Also called: Scleroderma, SSc
Who gets Systemic Sclerosis / Scleroderma?
Women are affected four to five times more often than men, usually beginning between 30 and 50. It is uncommon, which contributes to diagnostic delay. Two broad patterns exist: limited cutaneous disease, where skin thickening stays below the elbows and knees and pulmonary hypertension is the main long-term risk; and diffuse cutaneous disease, which progresses faster, involves the trunk and upper arms, and carries a higher early risk of lung fibrosis and kidney crisis.
What are the symptoms of Systemic Sclerosis / Scleroderma?
Not everyone has every symptom below, and having one does not confirm the diagnosis. These are the features that most often lead to assessment:
- Fingers turning white, then blue, then red in cold or stress, often years before anything else
- Puffy, swollen fingers, later becoming tight, shiny and hard to bend
- Skin tightening around the mouth, reducing how wide it opens
- Small painful sores or pits at the fingertips
- Heartburn, reflux, or food sticking in the throat
- Bloating, alternating constipation and diarrhoea, unintended weight loss
- Dry cough or breathlessness on exertion
- Joint pain, tendon friction rubs, muscle weakness
- Visible small red spots on the face and hands (telangiectasia)
- Calcium deposits under the skin
- Sudden severe high blood pressure with headache — a medical emergency
How is Systemic Sclerosis / Scleroderma diagnosed?
Nail fold capillaroscopy is central: it shows enlarged and giant capillaries, capillary loss and disorganised vessels, and is often abnormal long before skin changes appear. This single painless test is what separates harmless Raynaud's from early systemic sclerosis.
Antibodies define the subtype and predict the risks: anti-centromere is associated with limited disease and pulmonary hypertension, anti-Scl-70 with diffuse disease and lung fibrosis, anti-RNA polymerase III with rapid skin progression and renal crisis.
Baseline organ screening is mandatory at diagnosis and repeated regularly: high-resolution CT of the chest, lung function tests, echocardiography, kidney function and blood pressure.
How is Systemic Sclerosis / Scleroderma treated?
Treatment targets the specific problems present rather than the disease as a whole.
For Raynaud's and digital ulcers: keeping the whole body warm rather than only the hands, calcium channel blockers, and for severe disease PDE5 inhibitors or iloprost infusions. Bosentan reduces new ulcers.
For reflux and gut involvement: proton pump inhibitors, often at higher doses than usual, and prokinetic agents.
For lung fibrosis: mycophenolate is first-line, with nintedanib or tocilizumab in selected patients.
For skin disease: methotrexate or mycophenolate in early diffuse disease.
For renal crisis: ACE inhibitors immediately — this transformed a once-fatal complication into a treatable one, and is why blood pressure is monitored so closely.
High-dose corticosteroids are avoided in diffuse disease because they can precipitate renal crisis.
Living with Systemic Sclerosis / Scleroderma
This is a condition where regular organ screening changes outcomes more than anything else, because lung and pulmonary artery involvement are silent until they are advanced. Annual lung function tests and echocardiography are routine. Practical measures matter: warmth, meticulous skin and fingertip care, small frequent meals, elevating the head of the bed, and avoiding smoking entirely.
When should you see a rheumatologist?
Raynaud's phenomenon that begins in adulthood, is asymmetrical, or comes with puffy fingers, fingertip ulcers, reflux or breathlessness should be assessed with capillaroscopy and antibody testing. Sudden severe hypertension in someone with scleroderma is an emergency.
Common questions about Systemic Sclerosis / Scleroderma
Is scleroderma fatal?
Outcomes vary considerably by subtype. Limited disease is often compatible with a normal lifespan. The risks come from lung fibrosis, pulmonary hypertension and renal crisis — all of which are now detectable early through routine screening and treatable when caught before symptoms appear.
Why do I need lung tests when I feel fine?
Lung fibrosis and pulmonary hypertension are silent until they are advanced. Annual lung function testing and echocardiography detect them at a stage where treatment preserves function, which is the single most valuable part of long-term care.
Can skin tightening be reversed?
Skin thickening in diffuse disease often peaks in the first two to three years and then softens spontaneously over subsequent years. Immunosuppression during the active phase reduces how far it progresses.
Will my Raynaud's definitely become scleroderma?
No. Most Raynaud's is primary and harmless. Capillaroscopy and antibody testing identify the minority at risk, and a normal result in both is strongly reassuring.
Why are steroids avoided?
High-dose corticosteroids can trigger scleroderma renal crisis, a dangerous rise in blood pressure with kidney failure. They are used only when clearly necessary and at the lowest possible dose.
This page is general information to support a consultation. It is not a diagnosis, and it does not replace an assessment by a doctor who has examined you.