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Table 5 Autoantibodies in systemic sclerosis

From: The clinical relevance of autoantibodies in scleroderma

Autoantibody Methods of testing Prevalence in SSc Clinical and serologic associations Prognosis
Anti-centromere IIF 20–30% CREST Better prognosis than anti-Scl-70
  IB   lcSSc ↑ Survival compared with anti-Scl-70 or anti-nucleolar antibodies
  ELISA   ↓ Pulmonary fibrosis No benefit in following levels over time
    Pulmonary hypertension  
Anti-Scl-70 ID ~15–20% Mutually exclusive with ACA Worse prognosis
  CIE   dcSSc ? Levels by ELISA fluctuate with extent of disease involvment
  IB   Pulmonary fibrosis and secondary cor pulmonale  
Anti-PM-Scl ID ~3% lcSSc Benign/chronic course with better response to steroids
  IP (Rare in Japanese) PM/SSc overlap  
Anti-Th/To IP ~2–5% Mutually exclusive with ACA Worse prognosis with reduced 10-year survival
   (More common in Japanese) lcSSc  
    ↓ Joint involvement  
    ↑ puffy fingers, small bowel involvement, hypothyroidism  
AFA IP ~4% Mutually exclusive with ACA, anti-Scl-70, anti-RNAP Seen in younger patients with greater internal organ involvement
   16–22% in patients of African descent dcSSc  
   4% in Caucasians Myositis, pulmonary hypertension, renal disease  
Anti-RNAP IP ~20% dcSSc Increased mortality
    Anti-RNAP II with ↓ lung function  
    Cor pulmonale unrelated to pulmonary fibrosis  
Anti-Ku IB Infrequent Overlap syndrome with scleroderma features  
Anti-Ro ID Infrequent Seen in one-third to one-half of SSc patients with sicca complex  
Anti-Sm IIF Rare SLE overlap Poor prognosis
  IP/CIE/ID Lupus nephritis, renal crisis   
  ELISA Pulmonary hypertension   
Anti-ribonucleoprotein IIF ~8% MCTD  
  IP/CIE/ID   Less CNS and renal diseases  
  ELISA   Raynaud's, puffy hands, sicca, myositis, esophageal disease  
  HA   lcSSc  
    Septal hypertrophy  
    Cor pulmonale secondary to pulmonary hypertension  
    Negatively correlates with dsDNA and low complement glomerulonephritis in those with SLE overlap More benign prognosis with favorable response to steroids
Anti-phospholipid antibodies ELISA ~20–25% with <1% SSc with APS Mutually exclusive with anti-centromere antibodies Associations inconsistent – needs further study
    aCL with ↑ disease severity and ↓ proximal skin involvement, scarring, esophageal hypomotility in one study  
    β2gp/aCL with pulmonary hypertension  
    Associations inconsistent – needs further study  
  1. ACA, anti-centromere antibodies; aCL, anticardiolipin antibodies; AFA, antifibrillarin/anti-U3-ribonucleoprotein; β2gp, β2 glycoprotein antibodies; CIE, counterimmunoelectrophoresis; CNS, central nervous system; dcSSc, diffuse cutaneous systemic sclerosis; dsDNA, double-stranded DNA; ELISA, enzyme-linked immunosorbent assay; HA, hemagglutination; IB, immunoblotting; ID, immunodiffusion; IIF, indirect immunofluorescence; IP, immunoprecipitation; lcSSc, limited cutaneous systemic sclerosis; MCTD, mixed connective tissue disease; PM/SSc, myositis/scleroderma overlap; SLE, systemic lupus erythematosus.