Scleroderma

What is Scleroderma?

Scleroderma is a chronic autoimmune connective tissue disorder characterised by excessive collagen production and accumulation, resulting in hardening and tightening of the skin and, in some cases, internal organs. Scleroderma can be limited to certain areas of the skin or may involve multiple organs throughout the body. Although there is currently no cure, treatment can help control symptoms, manage complications, and improve quality of life.

What are the Types of Scleroderma?

Scleroderma can occur in several forms, such as:

Stages of Scleroderma

Scleroderma is broadly divided into 5 major stages such as:

How Common is Scleroderma in India?

Scleroderma is considered an uncommon condition in India, although its exact prevalence is difficult to determine because mild and early cases may go undiagnosed. It can affect people of different ages but is more frequently diagnosed in adults, particularly women. Around 66.7% of patients were reported to have joint involvement, while 52.6% had internal organ involvement. Oral and dental manifestations are also reported in up to 80% of people with systemic sclerosis, although these symptoms may receive less attention because of other systemic manifestations.

What are the Symptoms of Scleroderma?

The symptoms of scleroderma can be different depending on the type and which organs are affected. Here are some common early and advanced symptoms:

What Causes Scleroderma?

Some possible causes of scleroderma include:

• Abnormal Immune Response: Scleroderma occurs when the immune system becomes overactive and triggers inflammation and excessive production of collagen.

• Genetic Factors: Certain genetic factors may increase susceptibility to autoimmune diseases, although having a genetic predisposition does not mean that a person will necessarily develop scleroderma.

• Environmental Exposure: Exposure to certain chemicals, solvents, silica, and other environmental substances has been associated with an increased risk of developing some forms of systemic sclerosis.

• Hormonal Factors: Scleroderma is more common in women, suggesting that hormonal factors may play a role in disease development.

• Autoimmune Predisposition: People with certain other autoimmune diseases may have a greater susceptibility to developing systemic sclerosis or related connective tissue disorders.

• Vascular Abnormalities: Changes in the small blood vessels and abnormal vascular responses, including those associated with Raynaud's phenomenon, are important features of systemic sclerosis.

What are the Complications of Scleroderma?

Scleroderma can sometimes affect more than just the skin, particularly in systemic forms of the disease. Possible complications include problems such as:

• Lung Problems: Scleroderma can affect the lungs and may lead to interstitial lung disease or pulmonary arterial hypertension, causing breathlessness and reduced exercise tolerance.

• Heart Problems: In some people, scleroderma can affect the heart and surrounding tissues, potentially causing irregular heart rhythms or reduced heart function.

• Digestive Problems: Changes in the digestive tract may cause acid reflux, difficulty swallowing, bloating, constipation, or diarrhoea.

• Circulation Problems: Reduced blood flow associated with Raynaud's phenomenon can cause painful fingers or toes, skin ulcers, and slow wound healing.

• Skin Ulcers and Scarring: Tightened skin and poor circulation can sometimes lead to sores or ulcers, particularly on the fingers and other areas with reduced blood flow.

• Joint and Movement Problems: Tight skin and changes in the tissues around joints may cause stiffness, pain, and difficulty moving affected areas comfortably.

• Dental and Mouth Problems: Tightening of the skin around the mouth and dry mouth can sometimes make eating, speaking, or maintaining oral hygiene more difficult.

Related Diseases Comparison

Scleroderma can have symptoms that are similar to those of other autoimmune and connective tissue diseases. The table below points out some important differences.

Scleroderma vs Rheumatoid Arthritis vs Systemic Lupus Erythematosus (SLE)

How is Scleroderma Diagnosed?

Doctors use different tests to confirm whether there is a chance of scleroderma and rule out other conditions. Such diagnostic methods include:

How is Scleroderma Managed?

There is currently no single treatment that cures scleroderma. Treatment is tailored to the disease type, symptoms, and organs affected. Various ways to treat and manage scleroderma are as follows:

• Skin Care: Regularly moisturising the skin can help manage dryness and discomfort caused by skin tightening. Gentle, fragrance-free products may be more suitable for sensitive skin.

• Managing Digestive Symptoms: Eating smaller meals and avoiding foods that trigger reflux may help reduce digestive discomfort. Doctors may also recommend medicines for heartburn, acid reflux, constipation, or other digestive problems.

• Managing Raynaud's Phenomenon: To manage Raynaud's Phenomenon, try to keep your hands and feet warm to help prevent episodes caused by cold weather. If you often have circulation problems or they are severe, your doctor might prescribe medicine.

• Physical Activity and Physiotherapy: Gentle stretching and regular physical activity can help you stay flexible and reduce stiffness. If you have trouble moving because of tight skin or joint problems, your doctor may suggest physiotherapy.

• Monitoring Internal Organs: If you have systemic sclerosis, you may need regular check-ups for your lungs, heart, kidneys, and digestive system. These check-ups help your doctor find any problems early and adjust your treatment if needed.

• Regular Medical Follow-up: Since scleroderma is a long-term condition, it is important to keep up with regular medical visits, even if your symptoms are stable. Let your healthcare provider know right away if you notice any new or worsening symptoms.

Medicines Used to Treat Scleroderma

Medicines for scleroderma are selected based on the type of disease, symptoms and organs affected. Commonly used medicines include:

• Mycophenolate Mofetil: An immunosuppressive medicine used to control active or progressive skin thickening and certain forms of organ involvement, such as interstitial lung disease.

• Methotrexate: An immunosuppressive medicine that may be used when scleroderma causes inflammatory arthritis or other inflammatory symptoms.

• Cyclophosphamide: A stronger immunosuppressive medicine that may be considered for certain severe or progressive forms of scleroderma, particularly when the lungs are involved.

• Tocilizumab: An immunosuppressive medicine that may be used in selected patients with active systemic disease.

• Rituximab: A biologic medicine that targets specific immune cells and may be considered for some patients with systemic scleroderma.

• Amlodipine: A calcium channel blocker that helps relax blood vessels and may reduce the frequency of Raynaud’s phenomenon attacks.

• Nifedipine: Another calcium channel blocker used to improve blood flow and help reduce Raynaud’s attacks and the occurrence of ulcers.

Can Scleroderma Be Cured?

The outlook for scleroderma depends on the type of disease, how much skin is involved, and if any internal organs are affected. Localised scleroderma usually follows a different path than systemic sclerosis, which can affect organs like the lungs, heart, kidneys, and digestive system. While there is no cure right now, getting diagnosed early, starting the right treatment, and having regular check-ups can help manage symptoms and lower the risk of serious problems. People with systemic scleroderma often need ongoing care from specialists to keep track of organ health and adjust treatment as needed.

Scleroderma During Pregnancy

Many women with scleroderma can have a healthy pregnancy, but careful planning and regular check-ups are especially important for those with systemic sclerosis. Risks vary depending on the type of scleroderma, disease activity, and whether organs like the lungs, heart, or kidneys are involved. If you have serious organ involvement, it is best to talk to a specialist before trying to get pregnant.

Here are some tips for managing scleroderma while you are pregnant:

• Discuss pregnancy planning with your rheumatologist and obstetrician before conception.

• Review all medicines with your doctor because some scleroderma treatments may not be suitable during pregnancy.

• Make sure to go to all your regular prenatal and specialist appointments.

• Keep track of your blood pressure and kidney function as your healthcare team recommends.

• Let your healthcare team know right away if you notice new or worsening breathlessness, severe headaches, swelling, or any other symptoms that worry you.

• Follow a balanced and nutritious diet to support maternal and foetal health.

• Keep your hands and feet warm to reduce Raynaud's symptoms.

• Do not stop prescribed medicines without speaking to your doctor.

Can Scleroderma Be Prevented?

There is no guaranteed way to prevent scleroderma because its exact cause is not fully understood. However, certain measures can help reduce complications and protect overall health, such as:

• Protect Against Cold: Keep your hands and feet warm to help lower the chances of Raynaud's attacks and protect against cold-related tissue damage.

• Avoid Smoking: Try not to smoke, as it can make blood-vessel problems worse and raise the risk of complications.

• Maintain Skin Care: Regular moisturising and protecting the skin from injury can help reduce dryness, cracking, and infections.

• Stay Physically Active: Appropriate stretching, exercise, and physiotherapy can help maintain flexibility and mobility.

• Attend Regular Check-ups: Routine monitoring can help detect lung, heart, kidney, and digestive complications at an earlier stage.

• Manage Blood Pressure: Regular blood pressure monitoring is particularly important in systemic sclerosis because kidney complications can sometimes develop rapidly.

• Follow a Balanced Diet: A nutritious diet can support overall health and help manage digestive or nutritional problems associated with scleroderma.

Scleroderma Treatment Cost in India

The following table includes the estimated treatment costs for scleroderma in India:

Disclaimer: The prices listed here are estimates that may change depending on the hospital's location, amenities, and other factors.

Does Health Insurance Cover Scleroderma?

Yes, scleroderma may be covered under health insurance plans in India, depending on the policy terms. Coverage may include hospitalisation, outpatient treatment, day-care procedures, diagnostic tests and ambulance charges.

If scleroderma is covered under a critical illness policy, a lump-sum benefit may be paid upon diagnosis of a covered condition. An initial waiting period of around 30 days may apply to regular health plans, while critical illness policies may have a 90-day waiting period and a 30-day survival period.

However, if scleroderma is already present when the policy is purchased, it may be treated as a pre-existing disease and could be subject to a 2–4 year waiting period, depending on the policy terms.

How Much Health Insurance Coverage Is Needed for Scleroderma Treatment?

Scleroderma can affect several organs and often needs ongoing specialist care, tests, medicines, hospital stays, and treatment for complications. Because of this, healthcare costs can differ a lot based on how severe the condition is and which organs are involved. Choosing a health insurance policy with a sum insured of ₹10–25 lakh can help cover hospital stays and major treatment costs. People with more widespread disease or extra health needs might want to choose a higher sum insured for better protection.

Living with Scleroderma in India: Daily Challenges & Care

Living with scleroderma can involve more than managing skin changes. Depending on the type and severity of the condition, people may experience circulation problems, joint stiffness, digestive symptoms, fatigue or complications affecting internal organs. With regular medical care and some daily adjustments, many people can manage their symptoms and maintain their quality of life.