Scleroderma leads to excessive collagen production, resulting in skin thickening and potential internal organ damage, including to the heart, lungs, and kidneys.
Scleroderma, or systemic sclerosis, is an autoimmune disorder where the immune system mistakenly attacks the body’s tissues
Scleroderma, a rare autoimmune disease, causes the skin and connective tissues to thicken and harden. Affecting about 400,000 people in the U.S., it involves a range of symptoms and complications with varying severity. Dr Dipti Patel, Consultant Rheumatologist, Wockhardt hospital, Mumbai Central shares a concise overview of scleroderma, including its types, symptoms, causes, and treatment options.
What Is Scleroderma?
Scleroderma, or systemic sclerosis, is an autoimmune disorder where the immune system mistakenly attacks the body’s tissues. This leads to excessive collagen production, resulting in skin thickening and potential internal organ damage, including to the heart, lungs, and kidneys.
Types of Scleroderma
There are two main forms of scleroderma:
Localized Scleroderma: Primarily affects the skin and is generally less severe. It includes:
- Morphea: Characterized by patches of hardened skin.
- Linear Scleroderma: Causes thickened skin in lines or bands, often on limbs or the forehead.Systemic Scleroderma: Impacts both the skin and internal organs. It includes:
- Limited Scleroderma (CREST Syndrome): Progresses slowly with symptoms such as calcium deposits, Raynaud’s phenomenon, esophageal issues, skin thickening on fingers, and small red spots.
- Diffuse Scleroderma: Rapid progression with widespread skin thickening and early organ involvement.
Symptoms of Scleroderma
Symptoms vary by type and severity but commonly include:
- Skin Changes: Thickening, tightening, and hardening of the skin, potentially leading to joint contractures.
- Raynaud’s Phenomenon: Fingers and toes turning white or blue in response to cold or stress.
- Digestive Issues: Heartburn, difficulty swallowing, and bowel problems.
- Pulmonary Complications: Lung fibrosis or pulmonary hypertension, causing breathlessness.
- Kidney Issues: Potential for high blood pressure and kidney failure.
- Joint Pain and Muscle Weakness: Common in affected individuals.
Causes and Risk Factors
The exact cause of scleroderma is unclear but is believed to involve a mix of genetic, environmental, and immune system factors. Genetics, exposure to certain chemicals or infections, and abnormal immune responses are potential triggers. Women are more frequently affected, with onset typically between ages 30 and 50.
Diagnosis and Treatment
Diagnosing scleroderma involves clinical evaluations, lab tests for autoantibodies, and imaging studies to assess organ involvement. While there is no cure, treatments focus on symptom management:
- Medications: Corticosteroids and immunosuppressants to control inflammation and immune response.
- Physical Therapy: To improve mobility and manage stiffness.
- Lifestyle Changes: Stress management, healthy diet, and avoiding extreme temperatures.
- Surgical Interventions: For severe complications like gastrointestinal issues or lung fibrosis.
Living with Scleroderma
Managing scleroderma requires ongoing medical care, support groups, and patient education to handle symptoms and enhance quality of life effectively.
