From the Guidelines
Systemic Sclerosis (Scleroderma) is associated with specific antibodies, including anti-centromere, anti-topoisomerase (Scl-70), and anti-RNA polymerase antibodies.
Specific Antibodies
- Anti-centromere antibodies are often found in patients with limited cutaneous systemic sclerosis (lcSSc) 1
- Anti-topoisomerase (Scl-70) antibodies are associated with a higher frequency of interstitial lung disease (ILD) and are often found in patients with diffuse cutaneous systemic sclerosis (dcSSc) 2
- Anti-RNA polymerase antibodies are associated with an increased risk of scleroderma renal crisis and malignancy, and are often found in patients with dcSSc 2, 3 ### Clinical Significance These antibodies can be used to diagnose and monitor Systemic Sclerosis, and can also help predict the risk of certain complications, such as ILD and scleroderma renal crisis.
Screening and Diagnosis
Screening for these antibodies can be performed using various methods, including enzyme-linked immunosorbent assay (ELISA) and immunoprecipitation 1, 2.
Treatment and Management
While there is no cure for Systemic Sclerosis, treatment and management of the disease can help alleviate symptoms and slow disease progression. This may include immunosuppressive therapy, such as mycophenolate mofetil (MMF) and cyclophosphamide, as well as autologous haematopoietic stem cell transplantation (AHSCT) 2, 3.
From the Research
Specific Antibodies Associated with Systemic Sclerosis (Scleroderma)
The following antibodies are associated with Systemic Sclerosis (Scleroderma):
- Anticentromere antibodies (ACA) 4, 5, 6, 7
- Anti-Scl-70 antibodies (ATA) 4, 5, 8, 6, 7
- Anti-RNA polymerase III antibodies (ARA) 5, 8, 6, 7
- Anti-U3RNP antibodies 6, 7
- Anti-U1RNP antibodies 6
- Anti-PmScl antibodies 5, 8, 6, 7
- Anti-Ku antibodies 4, 5, 8, 6, 7
- Anti-Th/To antibodies 4, 5, 8, 6, 7
- Anti-fibrillarin antibodies 4, 5, 8, 7
- Anti-NOR90 antibodies 4, 7
- Anti-PDGFR antibodies 4, 7
- Anti-RP11 and anti-RP155 antibodies 8, 7
Clinical Associations of Specific Antibodies
These antibodies have been associated with specific clinical features and prognosis in Systemic Sclerosis, including:
- Anticentromere antibodies: limited cutaneous involvement, lower frequency of pulmonary fibrosis, and lower mortality 5, 7
- Anti-Scl-70 antibodies: diffuse cutaneous involvement, increased frequency of pulmonary fibrosis, and higher mortality 5, 7
- Anti-RNA polymerase III antibodies: diffuse cutaneous and systemic involvement, and greater mortality 5, 7
- Anti-U3RNP and anti-U1RNP antibodies: specific clinical features and prognosis, including overlap features and diffuse cutaneous SSc form 6
Prevalence of Specific Antibodies
The prevalence of these antibodies varies among patients with Systemic Sclerosis, with some studies reporting:
- 41.7% of patients testing positive for SSc-specific autoantibodies 4
- 79% of patients testing positive for one or more SSc autoantibodies 8
- 94% of patients having positive antinuclear antibodies 7
- Significant differences in prevalence of specific antibodies between diffuse cutaneous and limited cutaneous SSc patients 7