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Autoantibodies to RNA Polymerase III antigen are found in approximately 11 % to 23 % of patients with systemic sclerosis (SSc). These patients typically lack other common SSc-associated antibodies such as anti‑centromere and anti‑Scl‑70, forming a distinct serologic group. Presence of anti‑RNA Pol III is associated with increased risk for diffuse cutaneous SSc, skin involvement, and hypertensive renal disease.