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Anti-fibrillarin antibodies in systemic sclerosis.
V J Tormey1, C C Bunn, C P Denton
1Department of Immunology, Royal Free Hospital, Pond Street, London NW3 2QG, UK.
Rheumatology (Oxford, England)
|October 16, 2001
Summary
Anti-fibrillarin antibody (AFA) positive patients often have early-onset systemic sclerosis (SSc) with significant internal organ involvement, including myositis and pulmonary hypertension. This finding is crucial for understanding disease progression in SSc.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Systemic sclerosis (SSc) is a complex autoimmune disease.
- Organ involvement significantly impacts SSc patient prognosis.
- Anti-fibrillarin antibody (AFA) is a known SSc-associated antibody.
Purpose of the Study:
- To determine the prevalence and clinical characteristics of AFA-positive patients in a UK systemic sclerosis cohort.
- To investigate the specific organ systems affected in AFA-positive SSc patients.
Main Methods:
- Indirect immunofluorescence and immunoprecipitation were used to identify AFA in 1026 SSc patients.
- Clinical data and organ involvement were assessed in AFA-positive individuals.
Main Results:
- AFA was found in 4.1% of patients, predominantly with early disease onset (mean age 36).
- AFA-positive patients showed high rates of myositis (54%), pulmonary hypertension (35%), and renal disease (23%), particularly within the diffuse cutaneous SSc (dcSSc) subgroup.
- In Caucasian patients, AFA was not exclusively associated with dcSSc, differing from prior reports.
Conclusions:
- AFA identifies a subset of young SSc patients experiencing frequent and severe internal organ involvement.
- Pulmonary hypertension, myositis, and renal disease are key manifestations in AFA-positive SSc.
- The association of AFA with limited cutaneous SSc (lcSSc) in Caucasians broadens its clinical relevance beyond dcSSc.