Scleroderma (Systemic Sclerosis) Treatment in Indore

Scleroderma (Systemic Sclerosis) – Detailed Description

Scleroderma, also known as Systemic Sclerosis (SSc), is a rare autoimmune disease characterized by hardening and thickening of the skin and connective tissues. It can also affect internal organs such as the lungs, heart, kidneys, and digestive tract.

The disease occurs due to overproduction of collagen, leading to tissue fibrosis, vascular abnormalities, and immune system dysfunction. Scleroderma is classified into two main types:

Localised Scleroderma – Limited to skin; does not usually involve internal organs.

Systemic Sclerosis – Affects skin and internal organs; can be life-threatening in severe cases.

Causes & Risk Factors

1. Autoimmune Dysfunction

Immune system attacks healthy connective tissue, leading to inflammation and collagen overproduction.

2. Genetic Predisposition

Family history of autoimmune diseases increases the risk.

3. Environmental Triggers

Exposure to silica dust, certain chemicals, or viral infections may trigger disease.

4. Gender & Age

More common in women-3:1 ratio

Usually develops between 30–50 years

How Scleroderma Affects the Body

Skin fibrosis: too much collagen → thick, tight skin

Vascular Changes: Blood vessel dysfunction → Raynaud’s phenomenon, digital ulcers

Internal Organ Involvement: Fibrosis in lungs, kidneys, heart, GI tract

Immune Dysregulation: Chronic inflammation worsens tissue damage.

Symptoms

Skin Symptoms

Thickening, tight skin on the fingers, hands, face, or other areas

Shiny, hardened patches

Loss of flexibility in the fingers and joints

Vascular Symptoms

Raynaud's phenomenon- fingers and toes turn white or blue in cold

Painful digital ulcers or sores

Musculoskeletal Symptoms

Stiffness and joint pains

Muscle weakness

Gastrointestinal Symptoms

Difficulty swallowing-esophageal dysmotility

Acid reflux

Bloating, diarrhea, or constipation

Pulmonary Symptoms

Shortness of breath

Pulmonary fibrosis in extreme measures

Renal and Cardiac Symptoms

High blood pressure (renal crisis)

Heart rhythm disorders

Heart failure in advanced disease

Complications

Pulmonary arterial hypertension

Interstitial lung disease

Renal crisis (life-threatening hypertension and kidney failure)

Severe joint contractures

Skin ulcerations and infections

Diagnosis

Clinical Examination - Skin thickening, Raynaud's phenomenon, joint involvement

Blood Test

ANA-Anti nuclear antibodies

Anticentromere antibodies, limited SSc

Anti-Scl-70 / anti-topoisomerase I antibodies (diffuse SSc)

Imaging

High-resolution computed tomography for lungs

Echocardiography for cardiac function

Pulmonary Function Tests (PFTs): Assess lung involvement

Skin Biopsy – Confirm of collagen deposition (rarely needed)

Conventional Medical Treatment

1. Immunosuppressive Therapy

Methotrexate, Mycophenolate, Cyclophosphamide

Reduce inflammation and slow the fibrosis

2. Vasodilators

Nifedipine, Sildenafil for Raynaud’s phenomenon

Improved blood flow to the extremities

3. Corticosteroids

Short-term use in flares

Used cautiously because of the risk of renal crisis

4. Organ-Specific Treatments

Pulmonary fibrosis-antifibrotic agents, oxygen therapy

Hypertension: ACE inhibitors for renal protection

Gastroesophageal reflux: proton pump inhibitors

5. Physical and Occupational Therapy

Maintain joint flexibility

Preventing contracture

Improve hand function

6. Surgery

In severe cases: digital ulcers, joint contractures, or lung transplantation

Lifestyle & Supportive Care

1. Skin Care

lotions and moisturizers

Gentle massage to maintain mobility

Avoid cold exposure to prevent Raynaud's attacks.

2. Nutrition and Diet

Anti-inflammatory diet: fruits, vegetables, omega-3 fatty acids

Avoid spicy foods; it reduces acid reflux.

3. Exercise

Low-impact exercises to enhance joint flexibility and circulation.

Yoga and stretching

4. Stress Management

Meditation, breathing exercises, and counseling help cope with chronic illness

5. Complementary & Integrative Approaches

Homeopathy, Ayurveda, and herbal medicine may help with symptom relief, fatigue, and skin care as supportive therapy, but they do not replace medical treatment

Prognosis

Scleroderma is chronic and progressive, but the course varies by type: Localized Scleroderma: Usually mild, limited to skin, excellent prognosis Systemic Sclerosis: Can be severe; prognosis depends on organ involvement Early diagnosis and treatment improve quality of life and reduce complications Regular lungs, heart, and kidney monitoring is recommended.