Scleroderma Treatment: Options and What to Expect

How is scleroderma treated?

Scleroderma (also called systemic sclerosis) is a long-term autoimmune condition. The immune system triggers a build-up of collagen — scar-like tissue — that makes skin and other tissues tighten and harden, and it can also damage small blood vessels and internal organs. There is no cure yet, so treatment has several goals: slow the hardening, calm the immune attack, keep blood flowing, protect the organs, and relieve symptoms.

There is no single scleroderma drug. Because the disease affects people so differently, treatment is matched to which organs are involved and how active the disease is. Care is usually led by a rheumatologist working with other specialists — for the lungs, heart, kidneys, or skin — and is adjusted over time. Starting treatment early, before too much scarring sets in, gives the best chance of protecting the body.

Calming the immune system and slowing skin changes

For skin thickening and more widespread (diffuse) disease, doctors use medicines that calm the immune system and can slow the hardening:

• Mycophenolate mofetil. Often the first choice, because it works well for skin and lung disease and is generally well tolerated.
• Methotrexate. Commonly used for skin involvement, especially early on.
• Cyclophosphamide. Effective but stronger, so it’s used more selectively.
• Rituximab and tocilizumab. Targeted medicines used for active or progressing disease.
• Stem cell transplant. For a small number of people with severe, fast-progressing diffuse scleroderma, an autologous stem cell transplant can improve outcomes. Because it carries real risks, it’s reserved for carefully selected cases.

These medicines take time to work and need regular blood tests to watch for side effects.

Protecting the lungs

Lung involvement — scarring called interstitial lung disease, and high blood pressure in the lung arteries called pulmonary hypertension — is one of the most important things to watch for in scleroderma, which is why doctors screen for it regularly.

• Mycophenolate mofetil is widely used as a first medicine for scleroderma lung scarring.
• Nintedanib (Ofev) and tocilizumab (Actemra) are FDA-approved to slow the decline of lung function in scleroderma-related interstitial lung disease. Nintedanib is an antifibrotic (it targets scarring); tocilizumab blocks an immune signal called IL-6.
• Pulmonary hypertension is treated with medicines that relax and open the lung’s blood vessels, often used in combination.

Managing Raynaud’s and finger ulcers

Most people with scleroderma have Raynaud’s — fingers and toes that change color and go numb in the cold or with stress. Managing it protects the fingers from damage:

• Everyday steps: keep your whole body warm, wear gloves and layers, avoid sudden cold and stress, and don’t smoke.
• Medicines: calcium channel blockers such as nifedipine are usually tried first; other vasodilator medicines can be added if needed.
• Finger ulcers: sores at the fingertips may need wound care, stronger vasodilators, and sometimes procedures to protect the finger.

Other symptoms and organs

• Acid reflux and digestion. Acid-reducing medicines (such as PPIs) help heartburn, and other medicines can help move food through the gut.
• Kidneys. Blood pressure medicines called ACE inhibitors are important if the kidneys are affected. A sudden rise in blood pressure (scleroderma renal crisis) is an emergency that needs urgent treatment.
• Skin. Moisturizers help dry, tight skin, and small visible blood vessels (telangiectasia) can be treated with laser if desired.

Physical and occupational therapy

Medicine is only part of the picture. Physical therapy and hand exercises can improve hand function, ease stiffness, and help you keep moving as skin tightens. Occupational therapy offers tools and techniques that make daily tasks — dressing, cooking, writing — easier. Good skin and dental care, staying active, and protection from the cold all support the medical treatments.