Clinical features and serum antinuclear antibodies in 230 Danish patients with systemic sclerosis

Abstract

The objective was to investigate the relationship between the presence of different types of antinuclear antibodies (ANA) in patients with systemic sclerosis (SSc) and the presence of clinical features. Sera from 230 patients with SSc were tested for the presence of ANA, including anticentromere antibodies (ab), antitopoisomerase I ab, anti-U1 RNP ab and antinucleolar ab, including anti-Th RNP, anti-U3 RNP and anti-U17 RNP. Clinical features were registered prospectively in a clinical database. Eighty-two per cent of the patients were women. The median age was 58 yr (45-67, quartiles) and median age at disease onset was 44 (30-55) yr. ANA were found in 86% of the patients (anticentromere: 34%; antitopoisomerase I: 14%; anti-U1 RNP: 6.5%; antinucleolar total: 16%; anti-Th RNP: 2.2%; anti-U3 RNP: 3.5%; anti-U17 RNP: 0%). Anticentromere ab were found to be related to a high prevalence of calcinosis, telangiectasia, digital ulcers, acrosclerosis, primary biliary cirrhosis, isolated reduction of pulmonary diffusing capacity, and a low prevalence of radiological evidence of pulmonary fibrosis. Antitopoisomerase I ab were associated with a high prevalence of digital joint deformity, distal osteolysis, radiological signs of pulmonary fibrosis, a low prevalence of calcinosis and late onset of disease. Anti-U1 RNP ab were related to a high prevalence of arthritis and myositis, a low prevalence of calcinosis, and early disease onset. The presence of antinucleolar ab, including anti-U3 RNP and anti-Th RNP, was not significantly related to any particular clinical features in this study; possibly due to the small number of patients with these ab. The presence of anticentromere, antitopoisomerase I and anti-U1 RNP ab in the serum was also found to have previously described clinical correlations in a group of Danish SSc patients.

High Prevalence of Acute Exacerbation of Interstitial Lung Disease in Japanese Patients with Systemic Sclerosis

“…For example, the ratio was 1.7 (252/146) at the Royal Free Hospital in the UK (Nihtyanova et al 2014), 2.1 (273/132) at Kitasato University in Japan (Hashimoto et al 2011), and 2.9 (127/44) at Keio University in Japan (Shirai et al 2015). In addition, the 10-year survival rate in our study (92.0%) was greater than that found in previous studies from Japan and Western countries (72.6%-88.0%) (Jacobsen et al 2001;Czirjak et al 2008;Hashimoto et al 2011). The reason for these discrepancies remains unclear; however, the prevalence of limited type without internal organ involvement has been increasing in recent years (Ferri et al 2014), which may have attributed to these differences.…”

Prevalence of Anti–RNA Polymerase III Antibodies in Systemic Sclerosis: New Data From a French Cohort and a Systematic Review and Meta‐Analysis

“…In Japan, prevalence was estimated at 5% in one study, and 11% in a later study (2,15). In Europe, important variations exist between countries: a prevalence of 3-9% was seen in France and Italy, while a prevalence of 20-22% was seen in Denmark and the UK (12,14,16,21,22). The aims of the present study were to assess the prevalence of anti-RNAP III in a new French cohort and to perform a systematic review and meta-analysis of published reports to assess the prevalence of anti-RNAP III in patients worldwide and the potential factors explaining the observed heterogeneity.…”

Association of C‐reactive protein with high disease activity in systemic sclerosis: Results from the Canadian Scleroderma Research Group

“…There are 2 subtypes of SSc, i.e., diffuse cutaneous SSc (dcSSc) and limited cutaneous SSc (lcSSc), according to the extent of cutaneous involvement (2); dcSSc has more extensive skin involvement, including the trunk or proximal extremities, and is associated with more internal organ involvement such as significant interstitial lung disease (ILD), cardiomyopathy, and scleroderma renal crisis (SRC) and with increased mortality (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18).…”