Predominant Features Associated with Systemic Sclerosis specific Autoantibodies
From Steen, V. D. (2005). “Autoantibodies in systemic sclerosis.” Semin Arthritis Rheum 35(1): 35-42.
| ACA a | Th/To | U1-RNP (MCTD) | PmScl | U3-RNP (fibrillarin) | Scl-70 (topoisomerase I) | RNA Pol III b | |
|---|---|---|---|---|---|---|---|
| SSc subset (% pts) | Limited (50%–90%) | Limited (4%–16%) | Limited (100% MCTD) | Limited (3%) | Diffuse (7%) | Diffuse (20%–30%) | Diffuse (25%) |
| Lungs | PAH | ILD + PAH | PAH ILD | ILD Myositis | ILD + PAH | ILD | – |
| Kidneys | – | – | – | – | SRC | SRC | SRC |
ACA, anticentromere antibody; ILD, interstitial lung disease; MCTD, mixed connective tissue disease; PAH, pulmonary arterial hypertension; SRC, scleroderma renal crisis.
a ACA antibody positive patients should be screened for primary biliary cirrhosis with antimitochondrial antibody test and LFTs
b RNA polymerase III antibody positive patients are at higher risk for malignancy
