Objective
Large descriptive studies of pain in systemic sclerosis (SSc) are lacking. The present study estimated prevalence, severity, and associations between SSc clinical variables and pain in all ...patients with SSc and in limited cutaneous (lcSSc) and diffuse cutaneous (dcSSc) subsets.
Methods
Patients enrolled in a multicenter SSc registry (n = 585) completed a standardized clinical assessment and questionnaires about their physical and psychosocial health, including a pain severity numerical rating scale (NRS; range 0–10). Pain prevalence and severity were estimated with descriptive statistics. Crude and adjusted associations between specific SSc clinical variables and pain were estimated with linear regression for the entire group and by SSc subtype.
Results
Of the patients, 484 (83%) reported pain (268 46% mild pain NRS 1–4, 155 27% moderate pain NRS 5–7, and 61 10% severe pain NRS 8–10). More frequent episodes of Raynaud's phenomenon, active ulcers, worse synovitis, and gastrointestinal (GI) symptoms were associated with pain in multivariate analysis adjusting for demographic variables, depressive symptoms, and comorbid conditions. Patients with dcSSc reported only slightly higher mean ± SD pain than those with lcSSc (dcSSc 3.9 ± 2.8 versus lcSSc 3.4 ± 2.7; Hedges's g = 0.18, P = 0.05). Regression estimates did not differ significantly between SSc subsets.
Conclusion
Pain symptoms were common in the present study of patients with SSc and were independently associated with more frequent episodes of Raynaud's phenomenon, active ulcers, worse synovitis, and GI symptoms. Subsetting by extent of skin involvement was only minimally related to pain severity and did not affect associations with clinical variables. More attention to pain and how to best manage it is needed in SSc.
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.
Objective
To determine mortality and causes of death in a multinational inception cohort of subjects with systemic sclerosis (SSc).
Methods
We quantified mortality as standardized mortality ratio ...(SMR), years of life lost, and percentage mortality in the first decade of disease. The inception cohort comprised subjects recruited within 4 years of disease onset. For comparison, we used a prevalent cohort, which included all subjects irrespective of disease duration at recruitment. We determined a single primary cause of death (SSc related or non–SSc related) using a standardized case report form, and we evaluated predictors of mortality using multivariable Cox regression.
Results
In the inception cohort of 1,070 subjects, there were 140 deaths (13%) over a median follow‐up of 3.0 years (interquartile range 1.0–5.1 years), with a pooled SMR of 4.06 (95% confidence interval 95% CI 3.39–4.85), up to 22.4 years of life lost in women and up to 26.0 years of life lost in men, and mortality in the diffuse disease subtype of 24.2% at 8 years. In the prevalent cohort of 3,218 subjects, the pooled SMR was lower at 3.39 (95% CI 3.06–3.71). In the inception cohort, 62.1% of the primary causes of death were SSc related. Malignancy, sepsis, cerebrovascular disease, and ischemic heart disease were the most common non–SSc‐related causes of death. Predictors of early mortality included male sex, older age at disease onset, diffuse disease subtype, pulmonary arterial hypertension, and renal crisis.
Conclusion
Early mortality in SSc is substantial, and prevalent cohorts underestimate mortality in SSc by failing to capture early deaths, particularly in men and those with diffuse disease.
The prevalence of organ complications in scleroderma (systemic sclerosis; SSc) varies by definition used. This study was done to determine the frequency of several features of SSc.
A search of ...Medline-Ovid/Embase, PubMed, and Scopus databases from 1980 to November 30, 2011, was conducted to identify relevant articles with at least 50 patients with SSc extracting prevalence of each organ complication. Study quality was assessed using the STROBE (Strengthening The Reporting of OBservational studies in Epidemiology) checklist. Pooled prevalence was calculated using the random effects method. Heterogeneity was quantified using I(2).
A total of 5916 articles were identified (913 from Medline-Ovid/Embase, 1009 from PubMed, and 3994 from Scopus); 5665 were excluded, leaving 251 articles for full-text review, with 69 included. Where available, frequencies were also included from the Canadian Scleroderma Research Group. Many severe complications in SSc occur about 15% of the time, including cardiac involvement (15%, 95% CI 6-24), diastolic dysfunction (16%, 95% CI 14-17), estimated pulmonary artery pressure > 40 mm Hg (18%, 95% CI 14-21), pulmonary arterial hypertension by right heart catheterization (15%, 95% CI 12-17), forced vital capacity (FVC) < 70% predicted (15%, 95% CI 12-17), FVC < 80% predicted (17%, 95% CI 12-21), myositis (13%, 95% CI 10-17), inflammatory arthritis (12%, 95% CI 9-16), Sjögren overlap (13%, 95% CI 10-16), and digital ulcers (DU; 15%, 95% CI 10-20); and 15% of DU have complications (amputations 12%, 95% CI 8-16, and hospitalizations 13%, 95% CI 6-21). Scleroderma renal crisis is uncommon but occurs in almost 15% (12%, 95% CI 5-19) of cases of disseminated cutaneous SSc. There is no 15% rule within skin and gastrointestinal tract for SSc.
The "15%" rule for frequency of significant organ involvement in SSc is helpful.
Abstract Objective To determine whether exposure to angiotensin-converting enzyme (ACE) inhibitors prior to the onset of scleroderma renal crisis (SRC) leads to worse outcomes of SRC. Methods ...Prospective cohort study of incident SRC subjects. The exposure of interest was ACE inhibitors prior to the onset of SRC. The outcomes of interest were death or dialysis during the first year after the onset of SRC. Results A total of 87 subjects with incident SRC were identified and 1-year follow-up data were obtained in 75 (86%) subjects. Overall, 27 (36%) subjects died within the first year and an additional 19 (25%) remained on dialysis 1 year after the onset of SRC. In adjusted analyses, exposure to ACE inhibitors prior to the onset of SRC was associated with an increased risk of death (hazard ratio 2.42, 95% CI 1.02, 5.75, p < 0.05 in the primary analysis and 2.17, 95% CI 0.88, 5.33, p = 0.09 after post-hoc adjustment for pre-existing hypertension). Conclusion Overall, the 1-year outcomes of SRC were poor. Prior exposure to ACE inhibitors was associated with an increased risk of death after the onset of SRC, although there was uncertainty around the magnitude of the risk and the possibility of residual confounding could not be ruled out. Further studies will be needed to confirm these findings.
ObjectiveWe sought to develop the first Damage Index (DI) in systemic sclerosis (SSc).MethodsThe conceptual definition of ‘damage’ in SSc was determined through consensus by a working group of the ...Scleroderma Clinical Trials Consortium (SCTC). Systematic literature review and consultation with patient partners and non-rheumatologist experts produced a list of potential items for inclusion in the DI. These steps were used to reduce the items: (1) Expert members of the SCTC (n=331) were invited to rate the appropriateness of each item for inclusion, using a web-based survey. Items with >60% consensus were retained; (2) Using a prospectively acquired Australian cohort data set of 1568 patients, the univariable relationships between the remaining items and the endpoints of mortality and morbidity (Physical Component Summary score of the Short Form 36) were analysed, and items with p<0.10 were retained; (3) using multivariable regression analysis, coefficients were used to determine a weighted score for each item. The DI was externally validated in a Canadian cohort.ResultsNinety-three (28.1%) complete survey responses were analysed; 58 of 83 items were retained. The univariable relationships with death and/or morbidity endpoints were statistically significant for 22 items, with one additional item forced into the multivariable model by experts due to clinical importance, to create a 23-item weighted SCTC DI (SCTC-DI). The SCTC-DI was predictive of morbidity and mortality in the external cohort.ConclusionsThrough the combined use of consensus and data-driven methods, a 23-item SCTC-DI was developed and retrospectively validated.
Abstract
Objectives
Damage accrual in SSc can be tracked using the Scleroderma Clinical Trials Consortium Damage Index (DI). Our goal was to develop a prediction model for damage accrual in SSc ...patients with early disease.
Methods
Using patients with <2 years disease duration from Canada and Australia as a derivation cohort, and from the Netherlands as a validation cohort, we used group-based trajectory modelling (GBTM) to determine ‘good’ and ‘bad’ latent damage trajectories. We developed a prediction model from this analysis and applied it to patients from derivation and validation cohorts. We plotted the actual DI trajectories of the patients predicted to be in ‘good’ or ‘bad’ groups.
Results
We found that the actual trajectories of damage accumulation for lcSSc and dcSSc were very different, so we studied each subset separately. GBTM found two distinct trajectories in lcSSc and three in dcSSc. We collapsed the two worse trajectories in the dcSSc into one group and developed a prediction model for inclusion in either ‘good’ or ‘bad’ trajectories. The performance of models using only baseline DI and sex was excellent with ROC AUC of 0.9313 for lcSSc and 0.9027 for dcSSc. Using this model, we determined whether patients would fall into ‘good’ or ‘bad’ trajectory groups and then plotted their actual trajectories which showed clear differences between the predicted ‘good’ and ‘bad’ cases in both derivation and validation cohorts.
Conclusions
A simple model using only cutaneous subset, baseline DI and sex can predict damage accumulation in early SSc.
The aim was to establish the prevalence and severity of faecal incontinence (FI) in SSc, its association with other intestinal manifestations and potential predictors of FI, and its impact on quality ...of life.
A multicentre, cross-sectional study of 298 SSc subjects followed in the Canadian Scleroderma Research Group cohort was performed using validated questionnaires: Jorge-Wexner score (an FI severity scale), Bristol stool scale (a visual scale of stool consistency) and FI Quality-of-Life scale. Constipation was defined by the Rome III criteria. Associations between the Jorge-Wexner score and other clinical variables were determined using multivariate regression analyses.
Eighty-one (27.2%) subjects had FI, which was mild in 37 (12.4%) and moderate to severe in 44 (14.8%). Most patients had well-formed stools, 111 (38.8%) reported constipation and 38 (13.4%) had been previously treated for small intestinal bacterial overgrowth (SIBO). Variables independently associated with FI were: loose vs well-formed stools odds ratio (OR) = 7.01, 95% CI: 2.09, 23.51), constipation (OR = 3.64, 95% CI: 1.61, 8.27, P = 0.002), history of SIBO (OR = 2.97, 95% CI: 1.06, 8.27) and urinary incontinence (OR = 2.45, 95% CI: 1.14, 5.27). Quality of life measured with the FI Quality-of-Life scale was inversely correlated with FI severity (correlation coefficients between -0.602 and -0.702, P < 0.001).
FI was common and often severe in SSc. Loose stools, SIBO, constipation and urinary incontinence were strongly associated with FI. Other than targeting anorectal dysfunction, concomitant treatment of clinical correlates could lead to improvement in FI and quality of life in SSc.
Abstract Context Pain and itch are common symptoms reported by patients with scleroderma (SSc), which can markedly diminish function and health-related quality of life (HRQL). Objectives The aim of ...this exploratory study was to examine the impact that pain, itch, and the interaction of both, have on function (depressive symptoms, overall disability, fatigue, sleep disturbance) and HRQL in patients with SSc. Methods A total of 964 patients from the Canadian Scleroderma Research Group Registry completed questionnaires measuring itch and pain severity, function, and HRQL. Multiple regression analyses were performed to examine the impact that pain, itch and pain*itch interaction have on each outcome variable while controlling for demographic measures. A P -value of ≤ 0.01 was required for a difference to be deemed statistically significant. Results Our results revealed that patients with SSc who reported higher pain and itch severity were also more likely to have greater depressive symptoms, overall disability, sleep and fatigue problems, even when demographic measures were controlled for ( P -values ≤ 0.001). Similar results were obtained for HRQL, regardless of the domains ( P -values ≤ 0.001). A significant association between pain*itch interaction and sleep ( P = 0.002), physical functioning ( P = 0.003) and general health ( P ≤ 0.001) variables also was found. Further investigation of the nature of the pain*itch interaction showed that the effect of pain severity on outcome variables diminishes as itch severity increases. Conclusion Both pain and itch appear to have a detrimental impact on functioning and HRQL in patients with SSc, suggesting that more targeted approaches to symptom management are warranted.
Systemic sclerosis (SSc) is a multisystem disease associated with significant morbidity and increased mortality. Little is known about nutritional status in SSc. We investigated the prevalence and ...demographic and clinical correlates of nutritional status in a large cohort of patients with SSc.
This was a cross-sectional multicenter study of patients (n = 586) from the Canadian Scleroderma Research Group Registry. Patients were assessed with detailed clinical histories, medical examinations, and self-administered questionnaires. The primary outcome was risk for malnutrition using the "malnutrition universal screening tool" (MUST). Multiple logistic regression was used to assess the relationship between selected demographic and clinical variables and MUST categories.
Of the 586 patients in the study, MUST scores revealed that almost 18% were at high risk for malnutrition. The significant correlates of high malnutrition risk included the number of gastrointestinal (GI) complaints, disease duration, diffuse disease, physician global assessment of disease severity, hemoglobin, oral aperture, abdominal distension on physical examination, and physician-assessed possible malabsorption. Among 14 GI symptoms, only poor appetite and lack of a history of abdominal swelling and bloating predict MUST. These factors accounted for 24% of the variance in MUST scores.
The risk for malnutrition in SSc is moderate and is associated with shorter disease duration, markers of GI involvement, and disease severity. Patients with SSc should be screened for malnutrition, and potential underlying causes assessed and treated when possible.
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