Why does classification matter in Systemic Sclerosis

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.

For example:

• 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.


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