| Abstract | OBJECTIVE: To characterize a new serum autoantibody in patients with systemic sclerosis (SSc) directed against U11/U12 RNP and to identify the clinical features associated with this autoantibody. METHODS: We identified autoantibodies directed against the U11/U12 RNP complex in sera of patients with SSc and confirmed antibody specificity by immunoprecipitation, reverse transcriptase-polymerase chain reaction, and Southern blotting. We determined the prevalence of these antibodies in SSc and their specificity for SSc. We compared anti-U11/U12 RNP autoantibody-positive and negative SSc patients on demographic, disease classification, clinical variables, and survival. RESULTS: We identified 33 patients with anti-U11/U12 RNP antibodies. In 2 consecutive series of SSc patients first seen at 10-year intervals (1994-1995 and 2004-2005), the prevalence of anti-U11/U12 RNP antibody-positive patients was 15 of 462 (3.2%). Seventeen (52%) of these 33 patients had limited cutaneous involvement. All patients had Raynaud's phenomenon and 82% had gastrointestinal (GI) involvement. None had "intrinsic" pulmonary arterial hypertension. The most significant clinical difference between anti-U11/U12 antibody-positive and negative cohorts was the prevalence of lung fibrosis, which occurred in 79% of the anti-U11/U12 RNP antibody-positive patients versus 37% of the anti-U11/U12 RNP antibody-negative patients (P < 0.0001). GI involvement was also significantly increased in the anti-U11/U12 RNP antibody-positive group. Patients with anti-U11/U12 RNP antibodies and pulmonary fibrosis had a 2.25-fold greater risk of death than anti-U11/U12 RNP negative patients with pulmonary fibrosis. CONCLUSION: Anti-U11/U12 RNP antibodies are present in the sera of approximately 3% of patients with SSc and are a marker for lung fibrosis, which is often severe. |
| Authors | Noreen Fertig, Robyn T Domsic, Tatiana Rodriguez-Reyna, Masataka Kuwana, Mary Lucas, Thomas A Medsger Jr, Carol A Feghali-Bostwick
(Affiliation: University of Pittsburgh, Pittsburgh, PA 15213, USA.)
|
| Journal | Arthritis and rheumatism
(Arthritis Rheum)
Vol. 61
Issue 7
Pg. 958-65
(Jul 15 2009)
ISSN: 0004-3591 [Print] United States |
| PMID | 19565553
(Publication Type: Comparative Study, Journal Article, Research Support, N.I.H., Extramural, Research Support, Non-U.S. Gov't)
|
| Chemical References |
- Autoantibodies
- Biological Markers
- Ribonucleoproteins, Small Nuclear
- U11/U12 small nuclear ribonucleoprotein
|
| Topics |
- Adult
- Antibody Specificity
(immunology)
- Autoantibodies
(blood)
- Biological Markers
(blood)
- Cohort Studies
- Disease Progression
- Female
- Humans
- Immunoprecipitation
- Male
- Middle Aged
- Pulmonary Fibrosis
(blood, diagnosis, mortality)
- Ribonucleoproteins, Small Nuclear
(immunology)
- Scleroderma, Systemic
(blood, complications, immunology)
- Survival Rate
|
