Skip to main content
. Author manuscript; available in PMC: 2023 Feb 2.
Published in final edited form as: Lancet. 2022 Nov 25;401(10373):304–318. doi: 10.1016/S0140-6736(22)01692-0

Table 1:

Autoantibodies associated with systemic sclerosis and their clinical correlates

Cutaneous subtype Clinical correlates
Anti-centromere Limited cutaneous systemic sclerosis Pulmonary arterial hypertension
Anti-topoisomerase 1 (anti-ScI 70)* Diffuse cutaneous systemic sclerosis Progressive interstitial lung disease
Anti-RNA polymerase 3 Diffuse cutaneous systemic sclerosis Renal crisis and malignancy
Anti-U1 ribonucleoprotein (anti-ribonucleoprotein) Limited cutaneous systemic sclerosis Mixed connective tissue disease
Anti-U3 ribonucleoprotein (anti-fibrillarin) Diffuse cutaneous systemic sclerosis Pulmonary arterial hypertension and myositis
Anti-Pm-Scl Limited cutaneous systemic sclerosis Myositis
Anti-Th/To Limited cutaneous systemic sclerosis Interstitial lung disease and pulmonary arterial hypertension

Testing for systemic sclerosis-associated auto-antibodies is recommended early in the evaluation for systemic sclerosis because it can help establish a systemic sclerosis diagnosis and inform risk-stratification strategies. Patients will seldomly produce more than one systemic sclerosis-related auto-antibody concurrently.

*

Immunodiffusion is the preferred method for detecting anti-topoisomerase 1 antibodies, given that multibead assays are associated with high false-positive rates.