Systemic Sclerosis (Scleroderma)
Autoimmune Disease Causing Lung Fibrosis and Pulmonary Hypertension
Systemic Sclerosis is an autoimmune condition where the body produces excess collagen, leading to thickening and scarring of tissues. When it affects the lungs, it can cause interstitial lung disease (ILD) and pulmonary hypertension, resulting in progressive breathing difficulty and serious complications.
Skin Tightening + Breathlessness?
Early lung screening is critical in systemic sclerosis
What is Systemic Sclerosis?
Autoimmune
Body attacks itselfExcess Collagen
Thickening & scarringLung Involvement
ILD & Pulmonary HypertensionAutoimmune symptoms + lung involvement → Key clinical clue
Systemic Sclerosis is an autoimmune disease where excess collagen deposition occurs, blood vessels become abnormal, and organs develop fibrosis.
What is Systemic Sclerosis?
Systemic Sclerosis is an autoimmune disease where:
- Excess collagen deposition occurs
- Blood vessels become abnormal
- Organs develop fibrosis
When lungs are involved:
- Interstitial lung disease (ILD) develops
- Pulmonary hypertension may occur
Early Signs & Symptoms
General Symptoms
Skin tightening
Early signRaynaud's phenomenon
Color change in fingersJoint stiffness
ArthralgiaLung Symptoms
Dry cough
Early lung signBreathlessness
ProgressiveReduced exercise tolerance
Functional limitationAdvanced Symptoms
Severe breathlessness
Oxygen dependency
Signs of pulmonary hypertension
Causes & Risk Factors
Cause
Autoimmune disorder
Risk Factors
- Female gender
- Family history of autoimmune diseases
- Environmental triggers
Disease Progression
Early Stage
Mild lung involvement
May be asymptomatic
Moderate Stage
ILD develops
Reduced lung function
Severe Stage
Fibrosis progresses
Pulmonary hypertension
End Stage Lung Disease
Respiratory failure
Lung transplant required
Autoimmune patients should monitor lung function regularly
Diagnosis & Tests
HRCT chest
High-resolution CT for fibrosisPulmonary function tests
Restriction and diffusion capacityAutoimmune blood tests
ANA, Scl-70, Anti-centromereEchocardiography
Pulmonary hypertension screeningAssess your autoimmune lung risk
Early detection improves outcomes
Treatment Options (Before Transplant)
1. Immunosuppressive Therapy
- Steroids
- Disease-modifying agents
2. Anti-fibrotic Therapy
- Slows lung fibrosis
3. Pulmonary Hypertension Treatment
- Targeted medications
4. Pulmonary Rehabilitation
- Breathing exercises
When is Lung Transplant Needed?
Advanced ILD
Severe pulmonary hypertension
Oxygen dependency
Poor quality of life
Early Monitoring Prevents Severe Complications
Multidisciplinary care is essential
Important Consideration: Multi-System Autoimmune Disease
• Affects multiple organs • Requires long-term follow-up • Early lung screening improves outcomes
Medical Management vs Transplant
Medical Management
Stage: Early/Moderate • Goal: Control immune response • Outcome: Slows progression
Lung/Heart-Lung Transplant
Stage: Advanced • Goal: Replace organs • Outcome: Life-saving
Transplant Options
Double Lung Transplant
Preferred in ILD
Heart-Lung Transplant
If severe pulmonary hypertension
Cost & Planning
Costs depend on:
Disease severity
Multi-organ involvement
Hospital & city
Download Guide
Lung Transplant Cost & Planning Guide
Free & confidentialGet Expert Help
Find Pulmonologists & Rheumatologists
Find SpecialistExplore Verified Lung Transplant Centers
Patient Resources
Autoimmune disease management guide
Lung monitoring checklist
Medication adherence plan
Pulmonary rehab exercises
Pre-transplant workup
Donor evaluation and legal documentation
Frequently Asked Questions
Systemic sclerosis can significantly affect lung function if not monitored.
Early diagnosis and treatment can slow disease progression. Stay informed. Act early.