Rheumatology Advance Access published online on February 28, 2003
Rheumatology, doi:10.1093/rheumatology/keg170
Rheumatology © British Society for Rheumatology 2003; all rights reserved
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Original Papers
1 Department of Rheumatology, Lund University Hospital, Lund, Sweden
* Corresponding author. E-mail: r.hesselstrand{at}telia.com.
Received 28 November 2001
; accepted 6 November 2002
Objectives. To study the frequency and specificity of antinuclear antibodies (ANA) and their association with internal organ involvement and survival in systemic sclerosis (SSc). Methods. Sera from 276 SSc patients were analysed by an indirect immunofluorescence (IIF) technique with HEp-2 cells as a substrate to categorize centromeric (ACA), nucleolar, speckled and homogeneous nuclear IIF patterns. Specific ANA were determined as follows: anti-DNA topoisomerase I (anti-topo I) by double immunodiffusion, anti-U1 RNP by passive haemagglutination, anti-RNA polymerase I, II and III (anti-RNAP) and anti-histone (AHA) antibodies by enzyme immunoassays. During the follow-up of 7.0±4.5 (mean±S.D.) yr the occurrence of clinical manifestations and internal organ involvement was registered. Results. ANA were present in 84% of the patients. The most common patterns of the IIF were speckled (41%), homogeneous (25%), nucleolar (24%) and centromeric (18%). A nucleolar pattern was associated with pulmonary fibrosis (P < 0.01) and cardiomegaly (P < 0.05). ACA were related to organic vasculopathy (P < 0.05) and renal involvement (P < 0.01), but not to pulmonary fibrosis (P < 0.01). Anti-topo I were present in 9.4%, anti-U1 RNP in 21%, anti-RNAP in 22% and AHA in 16% of the patients. Pulmonary involvement was more common in patients with anti-topo I (P < 0.05), whereas AHA-positive patients were characterized by cardiac (P < 0.05), pulmonary (P < 0.05) and renal (P < 0.05) involvement. A nucleolar IIF pattern and AHA were both associated with a decreased survival [relative risk of death 1.71 (P < 0.05) and 2.36 (P < 0.01), respectively]. Conclusions. AHA and a nucleolar HEp-2 cell pattern may indicate critical organ involvement and predict a reduced survival in SSc patients.
Key words: Systemic sclerosis, Survival, Antinuclear antibodies.
The association of antinuclear antibodies with organ involvement and survival in systemic sclerosis
2 Specialty Laboratories Inc., Santa Monica, CA, USA
3 Department of Autoimmunology, Statens Serum Institut, Copenhagen, Denmark
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