RT Journal Article
SR Electronic
T1 Clinical and Serological Hallmarks of Systemic Sclerosis Overlap Syndromes
JF The Journal of Rheumatology
JO J Rheumatol
FD The Journal of Rheumatology
SP jrheum.101248
DO 10.3899/jrheum.101248
A1 Angela Pakozdi
A1 Svetlana Nihtyanova
A1 Pia Moinzadeh
A1 Voon H. Ong
A1 Carol M. Black
A1 Christopher P. Denton
YR 2011
UL http://www.jrheum.org/content/early/2011/08/10/jrheum.101248.abstract
AB Objective To determine the prevalence of systemic sclerosis (SSc) overlap syndrome and autoantibody profile in a large single-center cohort. Methods SSc diagnoses, subsets, and autoantibody profiles were obtained from clinical records of patients attending the Centre for Rheumatology, Royal Free Hospital, between September 1999 and February 2007. Results In total, 332 (20%) of 1700 patients with SSc had overlap syndrome. This comprised myositis (42.8%), rheumatoid arthritis (RA; 32%), Sjögren’s syndrome (SS; 16.8%), and systemic lupus erythematosus (SLE; 8.4%). Antinuclear antibody was positive in 96.6% of patients. Anticentromere antibody (ACA) was exclusively present in limited cutaneous SSc (lcSSc) overlap cases (22%), and more common in SSc/SS overlap (44.7%), whereas no difference was found in the prevalence of Scl-70 autoantibody between lcSSc and diffuse cutaneous SSc overlap groups. U1RNP was more frequent in SSc/SLE (44%), while Ro antibody was more likely to be found in SSc/SS (29.8%). ACA was absent and anti-Scl-70 was infrequent in SSc/myositis; polymyositis-scleroderma antibody was more frequent in this group (33.1%). About 50% of patients had raised rheumatoid factor (RF), with no difference between overlap groups irrespective of RF titer. In contrast, anticyclic citrullinated peptide antibody was more frequent in patients with RA features. Conclusion About one-fifth of SSc cases had overlap features. There were distinct serological features that may predict specific clinical presentation and disease course.