Table 5

Autoantibodies in systemic sclerosis

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

ELISA

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

IP

ELISA

Anti-Ro

ID

Infrequent

Seen in one-third to one-half of SSc patients with sicca complex

ELISA

Anti-Sm

IIF

Rare

SLE overlap

Poor prognosis

IP/CIE/ID

Lupus nephritis, renal crisis

ELISA

Pulmonary hypertension

HA

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


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.

Ho and Reveille Arthritis Res Ther 2003 5:80-93   doi:10.1186/ar628