OBJECTIVE:To assess the prevalence of abnormal cervical cancer screening (Pap tests) reported by women with SSc onset before the age of 50 yrs.METHODS:Female members of a Canadian multi-centre SSc cohort completed standardized assessments and were questioned regarding a history of an abnormal Pap test. Potential correlates examined included demographics, reproductive history, smoking, diffuse vs limited SSc type, immunosuppressant exposure and SSc duration.RESULTS:In the 320 women with SSc onset before the age of 50 yrs, the life-time prevalence of an abnormal Pap test (according to self-report) was 25.4% (95% CI CI 20.9, 30.4%). By comparison, self-reported prevalence of abnormal Pap tests among general population Canadian females was recently reported at 13.8% (95% CI 11.6, 16.4%). Women with diffuse SSc (n = 142), tended to have a higher prevalence of self-reported cervical dysplasia (31.7%) compared with those with limited disease (20.7%), but the CIs overlapped. A multivariate logistic regression found a significant positive association between self-reported abnormal Pap test and diffuse disease [odds ratio (OR) 1.87; 95% CI 1.01, 3.47]. An independent association of an abnormal Pap test with smoking (OR 2.43; 95% CI 1.23, 4.78) and with younger age at disease onset was also noted.CONCLUSIONS:We noted a high prevalence of abnormal Pap tests self-reported in our sample. Increased risk was seen among those with diffuse SSc, and also among smokers and those with a younger age at disease onset. Thus, it seems prudent to ensure that adequate attention is paid to cervical cancer screening for women with SSc.

OBJECTIVEA large proportion of patients with limited systemic sclerosis (SSc) do not meet the current American College of Rheumatology (ACR) classification criteria for SSc. We undertook this study to determine whether the addition of easily available clinical variables, namely nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias, could improve the sensitivity of the current ACR classification criteria for patients with limited SSc.METHODSPatients in the Canadian Scleroderma Research Group Registry with skin involvement distal to the metacarpophalangeal joints were identified and divided into two groups according to whether they fulfilled the current ACR classification criteria for SSc or not. Sensitivity of the criteria was calculated. Regression tree analysis was performed to determine whether the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias could improve the sensitivity of the criteria.RESULTSOne hundred and one (101) patients were included, in majority women with a mean age of 59 (+/- 13). Of these, 68 (67%) met the ACR classification criteria. The sensitivity of the criteria increased from 67% to 99% with the addition of nailfold capillary abnormalities identified using a dermatoscope and visible telangiectasias.CONCLUSIONSThe SSc research community would benefit from having better classification criteria to identify patients with limited SSc. The current classification criteria for SSc may be significantly improved by the inclusion of easily identified clinical variables including nailfold capillary abnormalities using a dermatoscope.

Aim: Up to 50% of patients with systemic sclerosis (SSc) have complaints of dyspnoea. We evaluated the independent contributions of dyspnoea to function and health related quality of life (HRQoL) in SSc and also assessed the contributions of pulmonary hypertension, measured by the pulmonary artery systolic pressure (PASP), and interstitial lung disease, measured by the forced vital capacity (FVC), to dyspnoea. Methods: We assessed dyspnoea, PASP, FVC, function and HRQoL in a cohort of unselected patients with SSc. Multiple linear regression was used to assess the independent contributions of dyspnoea, PASP and FVC to function and HRQoL, after controlling for possible confounding variables. Results: A total of 194 patients with mean disease duration of 11.6 years were studied. Dyspnoea was a significant independent predictor of function and HRQoL. A model including age, gender, disease duration, disease severity and dyspnoea explained 33.3%, 10.6%, 39.2% and 29.4% of the variance of the Stanford Health Assessment Questionnaire, the Short-Form 36 (SF-36) mental component summary score, the SF-36 physical component summary score and the World Health Organization Disability Assessment Schedule II. PASP and FVC were significant independent predictors of dyspnoea but only 21.9% of the variance in dyspnoea was explained by age, gender, disease duration, FVC and PASP. The FVC was a significant independent predictor of function and HRQoL. Conclusion: In an unselected population of SSc patients, dyspnoea is a very important contributor to function and HRQoL. Interstitial lung disease, as measured by the FVC, contributes significantly to the sense of dyspnoea, function and HRQoL in SSc. Pulmonary hypertension, assessed echocardiographically by the PASP, predicts the degree of dyspnoea but not function and HRQoL in SSc.

The objective was to investigate the frequency of anti-cyclic citrullinated peptides (CCP) antibodies in systemic sclerosis (SSc) and primary biliary cirrhosis (PBC), utilizing a new "third generation" anti-CCP ELISA (anti-CCP3) kit and a conventional anti-CCP2 assay. Patients with PBC, SSc, RA, and normal controls were included in the study. Serum samples were screened for autoantibodies by indirect immunofluorescence (IIF), antibodies to CCP by a second- and third-generation ELISA, antibodies to "scleroderma" antigens (CENP B, Scl-70, PM/Scl and fibrillarin-Scl-34) by a line immunoassay (LIA), and IgM RF by ELISA. The frequency of anti-CCP2 antibodies in SSc and PBC samples was 14.8% (11/74) and 6.2% (5/80), respectively, and the frequency of anti-CCP3 antibodies in SSc was 13.5% (10/74) and in PBC was 3.7% (3/80). By comparison, in the RA group the frequency of anti-CCP3 and anti-CCP2 antibodies was 79.1% (38/48) and 77% (37/48), respectively. Anti-CCP3 ELISA had a sensitivity, specificity, and positive and negative likelihood ratios (LR) of 79% (95% confidence interval [CI] = 64-89%), 93% (95% CI = 88-96%), 11.8 (95% CI = 6.8-20.3), and 0.22 (95% CI = 0.12-0.38), respectively. By comparison, the anti-CCP2 assay had a sensitivity, specificity, and positive and negative LRs of 77% (95% CI = 62-87), 90% (95% CI = 85-94), 8.3 (95% CI = 5.2-13.2), and 0.25 (95% CI = 0.15-0.42), respectively. In patients with SSc, there was an association of anti-CCP2 antibodies with the presence of arthritis, but there was no association of anti-CCP2 or anti-CCP3 with anti-CENP B, anti-Scl 70, or RF. This study confirmed the high specificity and sensitivity of both anti-CCP assays for the diagnosis of RA. The presence of anti-CCP antibodies in SSc was only correlated with the presence of arthritis.