Why does classification matter in Systemic Sclerosis?
Knowledge of skin type (limited versus diffuse) and the autoantibody profile of a scleroderma patient is often helpful in their care.
As demonstrated earlier, the limited and diffuse scleroderma phenotypes “behave” differently, and specific autoantibodies are associated with certain internal organ manifestations.
• Patients with lcSystemic Sclerosis and a positive ACA are at highest risk of developing PAH, yet rarely develop progressive ILD or renal crisis.
• Patients with lcSSc and a positive anti-Scl-70 antibody are at a high risk for developing progressive ILD, yet rarely develop renal crisis.
• Patients with dcSSc and a positive RNA-polymerase III antibody are at highest risk of developing renal crisis.
• Data suggests that patients with a positive RNA polymerase III antibody are at higher risk for malignancy, highlighting the importance of age-appropriate cancer screening in Systemic Sclerosis patients with this autoantibody.