Systemic Sclerosis-related ILD
OVERVIEW
Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by:1,2
- Immune activation and autoimmunity
- Endothelial dysfunction resulting in small-vessel vasculopathy
- Fibroblast dysfunction with resultant excessive collagen production and fibrosis
- Immunologic abnormalities
The extent of skin involvement determines the specific sub-classification.1
LIMITED CUTANEOUS SCLEROSIS (lcSSc)
Skin involvement, commonly affects:3
- Face
- Distal to knees and elbows
DIFFUSE CUTANEOUS SCLEROSIS (dcSSc)
Skin involvement:3
- Diffuse
SSC SINE SCLERODERMA (RARE)
Skin involvement:3
- Absent
EPIDEMIOLOGY
Prevalence estimated to be 135-300 cases per million adults and annual incidence of 21-56 cases per million adults per year.4-6
Onset of disease typically occurs between the ages of 30-60 years.5
RISK FACTORS AND SURVIVAL
RISK FACTORS:7
- Women are 4 times more likely to develop the disease than men
- Race (African American at higher risk)
SURVIVAL RATE:8
Reported 5-year and 10-year survival of SSc patients were 75% and 63%, respectively.
SYMPTOMS
SSc CAN AFFECT MULTIPLE ORGANS9,10
SSc extending beyond the skin:
- Heart
- Kidneys
- Lungs
- Gastrointestinal
- Musculoskeletal
SSc EPIDEMIOLOGY AND MORTALITY
ILD IS THE MOST COMMON LUNG MANIFESTATION AMONG PATIENTS WITH SSc11
- Up to 90% of patients with SSc show changes consistent with ILD on high-resolution computed tomography (HRCT)12
- ILD manifestations of SSc most commonly present as a pattern of nonspecific interstitial pneumonitis (NSIP)12
- Usual interstitial pneumonia (UlP) pattern is also seen but is less common13
- In an analysis of the EUSTAR cohort, 53% of patients with diffuse cutaneous systemic sclerosis (dcSSc) and 35% of patients with lcSSc had clinically significant ILD13
- ILD accounts for approximately 33% of SSc-associated mortality11
