Scleroderma has no cure, but treatments targeting specific symptoms and organ involvement can slow progression, reduce discomfort, and protect vital organs. Because scleroderma affects people differently, from skin-only forms to widespread systemic disease, treatment is tailored to which parts of the body are involved. The approach combines immune-suppressing medications, organ-specific therapies, lifestyle changes, and physical rehabilitation.
Localized vs. Systemic: Two Different Treatment Paths
Localized scleroderma (morphea) affects primarily the skin and underlying tissue. Treatment typically starts with prescription corticosteroid creams to reduce inflammation, though long-term use can thin the skin. For severe or widespread morphea, ultraviolet light therapy (phototherapy) is an effective option. Most people with localized scleroderma don’t need the aggressive immune-suppressing drugs used for systemic disease.
Systemic sclerosis is a different story. It can affect the skin, lungs, heart, kidneys, and digestive tract, so treatment usually involves multiple medications working on different fronts simultaneously. The rest of this article focuses on systemic disease, since that’s where treatment decisions are most complex.
Treating Skin Thickening and Fibrosis
Skin tightening is one of the earliest and most visible signs of systemic sclerosis. European treatment guidelines recommend three main immune-suppressing options for skin fibrosis: methotrexate, mycophenolate mofetil, and rituximab. These drugs work by dialing down the overactive immune response that drives excess collagen production. Mycophenolate mofetil has become particularly popular because it treats both skin and lung involvement at the same time, making it a practical choice for people dealing with both problems.
Protecting the Lungs
Lung disease is the leading cause of death in systemic sclerosis, taking two main forms: scarring of lung tissue (interstitial lung disease) and high blood pressure in the lung’s blood vessels (pulmonary arterial hypertension). Both require different treatments and regular monitoring with breathing tests and heart ultrasounds.
Interstitial Lung Disease
For lung scarring, mycophenolate mofetil and cyclophosphamide (an older, more potent immune suppressor) both improve lung function by a small but meaningful amount over two years. A major clinical trial comparing the two found that lung capacity improved by about 2% in the mycophenolate group and about 3% in the cyclophosphamide group, with no statistically significant difference between them. The critical distinction is tolerability: patients on cyclophosphamide had far higher rates of low blood cell counts, were more likely to stop treatment early, and had a higher death rate (15% vs. 7%) during the study period. For this reason, mycophenolate mofetil is generally preferred as the first option.
Two additional medications are FDA-approved specifically for scleroderma lung disease. Tocilizumab, which blocks a key inflammatory signal called IL-6, is approved to slow the decline in lung function. It’s given as a weekly injection under the skin. Nintedanib, originally developed for a different type of lung scarring, also slows the rate at which lung function worsens and is often used alongside immune-suppressing therapy.
Pulmonary Arterial Hypertension
When blood pressure rises in the lungs’ arteries, current guidelines recommend starting combination treatment right away rather than adding drugs one at a time. This typically involves medications that relax blood vessels in the lungs, improving blood flow and reducing strain on the heart.
Managing Raynaud’s Phenomenon
Nearly all people with systemic sclerosis experience Raynaud’s, where fingers and toes turn white or blue and become painful in response to cold or stress. The blood vessels in the fingers constrict too aggressively, and over time this can lead to painful sores (digital ulcers) on the fingertips.
Lifestyle measures come first: keeping your whole body warm (not just your hands), wearing insulated gloves, using hand warmers, and avoiding sudden temperature changes. Most people with scleroderma-related Raynaud’s will also need medication. Calcium channel blockers, particularly nifedipine in its extended-release form, are the standard first choice. These drugs relax blood vessel walls to improve circulation. When calcium channel blockers aren’t enough, PDE5 inhibitors (the same class of drug used for erectile dysfunction) can provide additional blood vessel relaxation. For digital ulcers that don’t heal, endothelin receptor antagonists like bosentan are sometimes prescribed.
Kidney Crisis: A Medical Emergency
Scleroderma renal crisis is a sudden, dangerous spike in blood pressure that can rapidly damage the kidneys. Before effective treatment existed, almost all patients with significant kidney involvement died within a year. ACE inhibitors transformed that outlook and remain the cornerstone of treatment. The goal is to bring blood pressure back to baseline within 72 hours.
One important nuance: while ACE inhibitors are essential during a renal crisis, taking them preventively before a crisis may actually increase the risk of developing one. Observational studies found that prior ACE inhibitor use was associated with roughly 2.5 times the risk of developing renal crisis. This means ACE inhibitors are reserved for active crisis management, not used as a preventive measure.
High-dose corticosteroids also significantly raise the risk of triggering a renal crisis. When steroids are unavoidable for other symptoms, doctors typically keep the dose as low as possible and use them for the shortest time they can.
Digestive Problems and Nutrition
Up to 90% of people with systemic sclerosis develop some form of gastrointestinal involvement. The digestive tract loses its ability to move food along normally, which can cause acid reflux, difficulty swallowing, bloating, nausea, constipation, or diarrhea. Bacterial overgrowth in the small intestine (SIBO) is common when gut motility slows down, because bacteria that normally stay in the lower intestine migrate upward and begin fermenting food prematurely.
Dietary adjustments make a real difference. For acid reflux and swallowing difficulty, eat frequent small meals, stay upright for one to two hours after eating, and elevate the head of your bed six to eight inches using wooden blocks or a wedge pillow. Avoid chocolate, caffeine, mint, fatty foods, and alcohol before bed, as these relax the valve between the stomach and esophagus. If you develop gastroparesis (delayed stomach emptying), shift toward lower-fat, lower-fiber meals with higher liquid content.
For SIBO, reducing simple sugars, fruit juices, sugar alcohols, and fiber supplements can decrease the fermentation that causes bloating and gas. Many people also develop secondary lactose intolerance, which means cutting back on dairy or using lactase supplements. If SIBO becomes a recurring problem, reducing acid-blocking medications when possible allows stomach acid to help keep bacterial levels in check.
Medications that stimulate gut movement, called prokinetics, can help in earlier stages of digestive involvement. These work by encouraging the intestinal muscles to contract more regularly. Maintaining good nutrition is critical, since malabsorption from a sluggish gut can lead to weight loss and nutritional deficiencies over time.
Hand Exercises and Physical Therapy
Skin tightening on the hands and fingers is one of the most functionally limiting aspects of scleroderma. Without regular stretching, the fingers can gradually curl inward and lose their range of motion. Occupational therapy focused specifically on the hands is a core part of treatment.
The Scleroderma Patient-centered Intervention Network (SPIN) developed a structured hand exercise program with four targeted modules: thumb flexibility and strength, finger bending (making a fist), finger extension (straightening), and wrist flexibility and strength. Each exercise is available at different difficulty levels depending on how much limitation you’re starting with. Consistency matters more than intensity. Daily stretching, even for just 10 to 15 minutes, helps maintain the range of motion you have and can slow the progression of contractures.
Beyond the hands, general exercise helps maintain joint flexibility, cardiovascular fitness, and overall wellbeing. Many people find that warm water exercises are especially comfortable because the warmth helps relax tight skin and stiff joints.
Stem Cell Transplant for Severe Cases
For people with rapidly progressing, severe systemic sclerosis who haven’t responded to standard treatments, autologous stem cell transplant is an option. This involves collecting your own stem cells, using high-dose chemotherapy to essentially reset the immune system, and then reinfusing the stem cells to rebuild it.
Three major clinical trials have shown striking results. In one trial, all 10 patients in the transplant group improved, compared to none in the chemotherapy-only group. A longer-term trial found 86% survival at six years for transplant patients versus 51% for those on standard chemotherapy alone. The tradeoff is significant upfront risk: the procedure carries a higher chance of serious complications in the first year or two, but long-term outcomes are substantially better for eligible patients.
Eligibility is limited to people with disease duration under four to five years, significant skin thickening, and lung function that’s impaired but not too severely. Heart function must be adequate to tolerate the intensive treatment. This approach is typically offered at specialized transplant centers with experience in autoimmune diseases.
Ongoing Monitoring
Because scleroderma can quietly damage organs before symptoms appear, regular screening is essential. Lung function tests measure how well your lungs are working and track whether scarring is progressing. Echocardiograms check for rising pressure in the lung arteries, which can develop gradually over years. Blood pressure monitoring at home helps catch early signs of renal crisis, particularly in the first few years of diffuse disease when the risk is highest. Blood work tracks kidney function, blood counts, and markers of inflammation. The specific screening schedule depends on your disease subtype and which organs are involved, but most people with systemic sclerosis need these assessments at least annually.