The Effect of Age and Comorbidities: Children vs. Adults in Their Response to SARS-CoV-2 Infection.

While children have experienced less severe coronavirus disease (COVID-19) after SARS-CoV-2 infection than adults, the cause of this remains unclear. The objective of this study was to describe the humoral immune response to COVID-19 in child vs. adult household contacts, and to identify predictors of the response over time. In this prospective cohort study, children with a positive SARS-CoV-2 polymerase chain reaction (PCR) test (index case) were recruited along with their adult household contacts. Serum IgG antibodies against SARS-CoV-2 S1/S2 spike proteins were compared between children and adults at 6 and 12 months after infection. A total of 91 participants (37 adults and 54 children) from 36 families were enrolled. Overall, 78 (85.7%) participants were seropositive for anti-S1/S2 IgG antibody at 6 months following infection; this was higher in children than in adults (92.6% vs. 75.7%) (p = 0.05). Significant predictors of a lack of SARS-CoV-2 seropositivity were age ≥ 25 vs. < 12 years (odds ratio [OR] = 0.23, p = 0.04), presence of comorbidities (vs. none, adjusted OR = 0.23, p = 0.03), and immunosuppression (vs. immunocompetent, adjusted OR = 0.17, p = 0.02).

Antinuclear antibody-negative systemic sclerosis.

OBJECTIVE: To examine the demographic and clinical characteristics of systemic sclerosis (SSc) patients without antinuclear antibodies (ANA) compared to ANA-positive patients. METHODS: SSc patients enrolled in the Scleroderma Family Registry and DNA Repository were included. Relevant demographic and clinical data were entered by participating sites or obtained by chart review. ANA and SSc-related antibodies were determined in all investigated patients using commercially available kits at our laboratories. RESULTS: This study included 3249 patients, of whom 208 (6.4%) were ANA negative. The proportion of male patients was higher in the ANA-negative group (OR = 1.65; p = 0.008). ANA-negative patients experienced less vasculopathic manifestations of SSc. The percent predicted diffusing capacity of carbon monoxide (DLCO) was higher in ANA-negative patients (p = 0.03). Pulmonary arterial hypertension (PAH) per right heart catheterization was less common in the ANA-negative group (OR = 0.28; p = 0.03). Furthermore, patients with negative ANA had a lower prevalence of telangiectasias and digital ulcers/pits (OR = 0.59, p = 0.03 and OR = 0.38, p = 0.01, respectively). Although diffuse cutaneous involvement was more common, the modified Rodnan Skin Score (mRSS) was lower in the ANA-negative group (2.4 points lower, p = 0.05). Furthermore, they experienced more malabsorption (p = 0.05). There was no difference in the frequency of pulmonary fibrosis or scleroderma renal crisis. All-cause mortality was not different between the 2 groups (p = 0.28). CONCLUSIONS: In conclusion, the results of this study suggest that SSc patients who are ANA negative constitute a distinct subset of SSc with less vasculopathy (less PAH, digital ulcers, and fewer telangiectasias), a greater proportion of males, and possibly, more frequent lower gastrointestinal involvement.

Physical and occupational therapy referral and use among systemic sclerosis patients with impaired hand function: results from a Canadian national survey.

OBJECTIVES: Contractures and deformities of the hand are major factors in disability and reduced health-related quality of life in systemic sclerosis (SSc). Physical (PT) and occupational therapy (OT) have been emphasised to address impaired hand function, but little is known about the extent they are employed. The objective of this study was to determine the proportion of Canadian SSc patients with hand involvement who are referred to and use PT or OT services and factors associated with referral. METHODS: Participants were respondents to the Canadian Scleroderma Patient Survey of Health Concerns and Research Priorities who rated ≥1 of 5 hand problems (hand stiffness, difficulty making fist, difficulty holding objects, difficulty opening hand, difficulty with faucet) as occurring at least sometimes with moderate or higher impact. Patients indicated if their physicians recommended PT or OT and if they used these services. Multivariate logistic regression assessed independent predictors of PT or OT referral. RESULTS: Of 317 patients with hand involvement, 90 (28%) reported PT or OT referral, but only 39 (12%) reported using these services. PT or OT referral was associated with more hand problems (odds ratio [OR]=1.24, 95% confidence interval [CI] 1.02-1.51, p=0.031) younger age (OR=0.96, 95% CI 0.94-0.99, p=0.004) and not being employed (OR=0.50, 95% CI 0.26-0.97, p=.0041). CONCLUSIONS: Few SSc patients with hand involvement are referred to PT or OT, and even fewer use these services. High-quality randomised controlled trials of PT and OT interventions to improve hand function in SSc are needed.

New directions for patient-centred care in scleroderma: the Scleroderma Patient-centred Intervention Network (SPIN).

Systemic sclerosis (SSc), or scleroderma, is a chronic multisystem autoimmune disorder characterised by thickening and fibrosis of the skin and by the involvement of internal organs such as the lungs, kidneys, gastrointestinal tract, and heart. Because there is no cure, feasibly-implemented and easily accessible evidence-based interventions to improve health-related quality of life (HRQoL) are needed. Due to a lack of evidence, however, specific recommendations have not been made regarding non-pharmacological interventions (e.g. behavioural/psychological, educational, physical/occupational therapy) to improve HRQoL in SSc. The Scleroderma Patient-centred Intervention Network (SPIN) was recently organised to address this gap. SPIN is comprised of patient representatives, clinicians, and researchers from Canada, the USA, and Europe. The goal of SPIN, as described in this article, is to develop, test, and disseminate a set of accessible interventions designed to complement standard care in order to improve HRQoL outcomes in SSc.

Anti-centromere antibodies in a large cohort of systemic sclerosis patients: comparison between immunofluorescence, CENP-A and CENP-B ELISA.

INTRODUCTION: Anti-centromere antibodies (ACA) are useful biomarkers in the diagnosis of systemic sclerosis (SSc) where they are found in 20-40% of patients and, albeit with lower prevalence, in patients with other systemic autoimmune rheumatic diseases. Historically, ACA were detected by indirect immunofluorescence (IIF) on HEp-2 cells and confirmed by immunoassays using recombinant CENP-B. During the last few years, to accommodate high throughput diagnostics, a number of laboratories changed from IIF to ELISA assays. The objective of this study was to compare the detection of ACA by IIF to CENP-A and a CENP-B ELISA in a large cohort of SSc patients. METHODS: Sera collected from SSc patients (n=834) were tested for ACA by IIF on HEp-2 cells (ImmunoConcepts, Sacramento, CA) and CENP-A and CENP-B ELISA (both Dr. Fooke Laboratorien GmbH, Neuss, Germany). Furthermore, other autoantibodies were determined by QUANTA-Plex(TM) SLE 8 profile (INOVA, San Diego, CA), QUANTA Lite RNA Pol III (INOVA) and PM1-Alpha ELISA (Dr. Fooke). RESULTS: The prevalence of ACA was 35.0% by IIF, 41.6% by CENP-A and 57.8% by CENP-B ELISA. When the CENP-A and the CENP-B ELISA results were compared to the IIF, the area under the curve value derived from receiver operating characteristic analysis was 0.98 for both assays. ACA and anti-topoisomerase I antibodies co-occurred in 1.2% (ACA by IIF), in 3.5% (by CENP-A ELISA) and in 7.4% (by CENP-B ELISA). Anti-CENP-A antibodies were negatively associated with anti-Scl-70, anti-RNA Pol III, (both p<0.0001), anti-U1-RNP (p=0.008) and anti-PM1-Alpha antibodies (p=0.0337). The degree of association was dependent on the cut-off value used. CONCLUSION: Although we found good agreement between IIF and ELISA for the detection of ACA in SSc, a significant portion of CENP ELISA positive sera did not show the typical ACA staining pattern. Based on these findings, we conclude that an IIF ACA negative result might not rule out the presence of ACA. In addition, new CENP ELISA kits are reliable for the detection of anti-CENP in SSc sera.

Frequency and impact of symptoms experienced by patients with systemic sclerosis: results from a Canadian National Survey.

OBJECTIVE: Knowledge about the range of symptoms experienced by patients with SSc, and their impact on daily functioning is limited. The objective of the present study was to identify symptoms of SSc that patients rated as frequent and that highly impacted their ability to carry out daily activities. METHODS: A total of 464 persons with SSc responded to the Canadian Scleroderma Patient Survey of Health Concerns and Research Priorities, including questions regarding the frequency and impact of 69 SSc symptoms. Descriptive analyses were performed dichotomizing symptom frequencies as never or rarely vs sometimes, most of the time or always and symptom impact on daily activities as no or minimal impact vs moderate to severe impact. RESULTS: The five highest rated symptoms in terms of frequency and moderate to severe impact on daily activities, respectively, were: fatigue (89 and 72%), RP (86 and 67%), hand stiffness (81 and 59%), joint pain (81 and 64%) and difficulty sleeping (76 and 59%). In addition to these symptoms, items related to decreased hand function (difficulty making a fist and difficulty holding objects) and pain (muscle pain and joint tenderness) were frequently endorsed and commonly associated with moderate to severe impact on daily activities. CONCLUSION: This study confirmed the importance for quality of life of core symptoms of SSc, such as pain, fatigue and limitations in hand function. It also identified areas with very little research, such as sleep problems, that appear to play important roles in daily functioning, and that merit more focused study.

The association between disease activity and duration in systemic sclerosis.

OBJECTIVE: The absence of a standardized disease activity index has been an important barrier in systemic sclerosis (SSc) research. We applied the newly derived Valentini Scleroderma Disease Activity Index (SDAI) among our cohort of patients with SSc to document changes in disease activity over time and to assess possible differences in activity between limited and diffuse disease. METHODS: Cross-sectional study of a national cohort of patients enrolled in the Canadian Scleroderma Research Group Registry. Disease activity was measured using the SDAI. Depression scores were measured using the Centre for Epidemiologic Studies Depression Scale (CES-D). RESULTS: A total of 326 out of 639 patients had complete datasets at the time of this analysis; 87% were female, of mean age 55.6 years, with mean disease duration 14.1 years. SDAI declined steeply in the first 5 years after disease onset and patients with diffuse disease had 42% higher SDAI scores than patients with limited disease with the same disease duration and depression scores (standardized relative risk 1.42, 95% CI 1.21, 1.65). Patients with higher CES-D scores had higher SDAI scores relative to patients with the same disease duration and disease subset (standardized RR 1.22, 95% CI 1.14, 1.31). Among the 10 components that make up the SDAI, only skin score (standardized OR 0.59, 95% CI 0.43, 0.82) and patient-reported change in skin (standardized OR 0.64, 95% CI 0.45, 0.92) decreased with increasing disease duration. High skin scores (standardized OR 32.2, 95% CI 15.8, 72.0) were more likely and scleredema (standardized OR 0.58, 95% CI 0.37, 0.92) was less likely to be present in patients with diffuse disease. High depression scores were associated with positive responses for patient-reported changes in skin and cardiopulmonary function. CONCLUSION: Disease activity declined with time and patients with diffuse disease had consistently higher SDAI scores. Depression was found to be associated with higher patient activity scores and strongly associated with patient self-response questions. The role of depression should be carefully considered in future applications of the SDAI, particularly as several components of the score rely upon patient recall.

Sociodemographic, disease, and symptom correlates of fatigue in systemic sclerosis: evidence from a sample of 659 Canadian Scleroderma Research Group Registry patients.

OBJECTIVE: To assess fatigue levels and demographic, socioeconomic, disease, and psychosocial correlates of fatigue in patients with systemic sclerosis (SSc). METHODS: We conducted a cross-sectional, multicenter study of 659 patients with SSc from the Canadian Scleroderma Research Group Registry. Fatigue was assessed during annual Registry visits with the Short Form 36 (SF-36) health survey vitality subscale. Patients completed measures of depressive symptoms and pain and underwent clinical histories and medical examinations. Kendall's tau was used to assess bivariate association of sociodemographic, medical, and psychosocial variables with fatigue. Multivariable associations of demographic (step 1), socioeconomic (step 2), global disease (step 3), specific disease and lifestyle (step 4), and psychosocial (step 5) factors with fatigue were assessed using hierarchical multiple linear regression. RESULTS: The mean +/- SD score of the patients on the SF-36 vitality subscale was 45.6 +/- 10.8, substantially lower (indicating more fatigue) than the mean +/- SD score for the Canadian general population (65.8 +/- 18.0). In multivariate analysis, higher fatigue was significantly associated with the number of medical comorbidities (standardized beta = -0.11, P = 0.004), breathing problems (standardized beta = -0.23, P < 0.001), the number of gastrointestinal (GI) symptoms (standardized beta = -0.27, P < 0.001), and current smoking (standardized beta = -0.08, P = 0.018). As a group, specific symptom and lifestyle variables predicted the most incremental variance in fatigue (R(2) = 21.6%, P < 0.001), despite being added to the model after demographic, socioeconomic, and global disease duration/severity indicators. Symptoms of depression (beta = -0.42) and pain (beta = -0.21) were also independently associated with fatigue (P < 0.001). CONCLUSION: High levels of fatigue are common in patients with SSc and are independently associated with clinical variables, including number of comorbidities, breathing problems, GI symptoms, and smoking.

Symptoms of depression predict the trajectory of pain among patients with early inflammatory arthritis: a path analysis approach to assessing change.

OBJECTIVE: To assess the longitudinal relationships, including directionality, among chronic pain, symptoms of depression, and disease activity in patients with early inflammatory arthritis (EIA). METHODS: One hundred eighty patients with EIA completed an examination, including swollen joint count, and were administered the Center for Epidemiological Studies Depression Scale (CES-D) and the McGill Pain Questionnaire (MPQ) at 2 timepoints 6 months apart. Cross-lagged panel path analysis was used to simultaneously assess concurrent and longitudinal relationships among pain, symptoms of depression, and number of swollen joints. RESULTS: Pain, symptoms of depression, and number of swollen joints decreased over time (p < 0.001) and were prospectively linked to pain, symptoms of depression, and number of swollen joints, respectively, at 6 months. Symptoms of depression and pain were correlated with each other at baseline (0.47) and at 6-month followup assessments (0.28). Baseline symptoms of depression significantly predicted pain symptoms at 6 months (standardized regression coefficient = 0.28, p = 0.001), whereas pain and disease activity did not predict the course of any other variable after controlling for baseline values. CONCLUSION: Symptoms of depression predicted the trajectory of pain from baseline to 6 months. In addition, there were reciprocal/bidirectional associations between pain and symptoms of depression over time. More research is needed to better understand the relationship between pain and depressive symptoms and how to best manage patients with EIA who have high levels of both.

Prevalence and clinical correlates of symptoms of depression in patients with systemic sclerosis.

OBJECTIVE: To assess the prevalence and predictors of symptoms of depression in a large sample of patients with systemic sclerosis (SSc). METHODS: We conducted a cross-sectional, multicenter study of 376 patients with SSc from the Canadian Scleroderma Research Group Registry. Patients were assessed with the Center for Epidemiologic Studies Depression Scale (CES-D) and through extensive clinical histories and medical examinations. Hierarchical multiple linear regression was used to assess the relationship of sociodemographic and clinical variables with symptoms of depression. RESULTS: The percentages of patients who scored > or =16 and > or =23 on the CES-D were 35.1% and 18.1%, respectively. Patients with less education; patients who were not married; patients with higher physician-rated overall disease severity; and patients with more tender joints, more gastrointestinal symptoms, and more difficulty breathing had significantly higher total CES-D scores. As a group, specific symptom indicators (tender joints, gastrointestinal symptoms, breathing) predicted the most incremental variance in depressive symptoms (DeltaR(2) = 14.2%, P < 0.001) despite being added to the model after demographic, socioeconomic, and global disease duration/severity indicators. CONCLUSION: High levels of depressive symptoms are common in patients with SSc and are related to overall SSc disease severity, as well as specific medical symptoms. Screening for depression among patients with SSc is recommended, although more research is needed to determine the best method for doing this. Successfully treating dyspnea, gastrointestinal symptoms, and joint pain may improve mood, although this has not yet been demonstrated.

Patients with systemic autoimmune diseases could not distinguish comorbidities from their index disease.

OBJECTIVE: To assess the construct validity of the Self-Administered Comorbidity Questionnaire (SCQ) in patients with systemic sclerosis (SSc) and systemic lupus erythematosus (SLE). METHODS: The SCQ was modified for the SSc cohort to emphasize the objective of recording problems other than the patients' scleroderma. It was administered to 406 SSc and 147 SLE patients. Construct validity of the SCQ was evaluated separately in the SSc and SLE cohorts by testing the hypotheses that a valid comorbidity index should correlate with age and health-related quality of life (Medical Outcomes Trust Short Form 36 [SF 36]) but not with disease-specific variables. RESULTS: The SCQ score correlated with age in the SSc patients only (Tau B=0.37, P<0.001) and not in the SLE patients. It correlated with the SF 36 in both SSc and SLE. However, it also correlated with several disease-related variables. There was significant overlap between reports of comorbidities and disease-related problems in the SSc cohort. CONCLUSION: Patients with systemic autoimmune diseases cannot distinguish true comorbidities from conditions related to their index disease and, as such, a self-administered comorbidity questionnaire does not appear useful in these diseases.

Reliability and validity of the center for epidemiologic studies depression scale in patients with systemic sclerosis.

OBJECTIVE: Reported rates of depressive symptoms in patients with systemic sclerosis (SSc) are high. No depression assessment tools, however, have been validated for patients with SSc. Our objective was to assess the internal consistency reliability, convergent validity, and structural/construct validity of the Center for Epidemiologic Studies Depression Scale (CES-D) in patients with SSc. METHODS: We conducted a cross-sectional, multicenter study of 470 SSc patients. Internal consistency reliability was assessed with Cronbach's alpha and structural/construct validity with confirmatory factor analysis. RESULTS: Internal consistency reliability was good for the overall CES-D scale (alpha = 0.88) and for its 4 factors (alpha = 0.67-0.88). Correlations of the CES-D total score were -0.73 with mental health, -0.36 with physical health, 0.41 with disability, and 0.44 with pain. The 4-factor model originally found in the general population and validated for patients with rheumatoid arthritis (depressed affect, somatic/vegetative, [lack of] positive affect, and interpersonal factors) fit the data well, as did a second-order version of the same model with an overarching depression factor that loaded onto each of the 4 first-order factors. The 4-factor model fit the SSc data better than alternative models. CONCLUSION: Internal consistency reliability and convergent validity were good, the 4-factor structure reported in the general population was replicated, and a second-order model with an overarching depression factor fit well. These findings indicate that the CES-D is a valid and reliable measure of depressive symptoms for patients with SSc.

High rates of depressive symptoms among patients with systemic sclerosis are not explained by differential reporting of somatic symptoms.

OBJECTIVE: Between 36% and 65% of patients with systemic sclerosis (SSc) report symptoms of depression above cutoff thresholds on self-report questionnaires. The objective of this study was to assess whether these high rates result from differential reporting of somatic symptoms related to the high physical burden of SSc. METHODS: Symptom profiles reported on the Center for Epidemiologic Studies Depression Scale (CES-D) were compared between a multicenter sample of 403 patients with SSc and a sample of respondents to an Internet depression survey, matched on total CES-D score, age, race/ethnicity, and sex. An exact nonparametric generalized Mantel-Haenszel procedure was used to identify differential item functioning between groups. RESULTS: Patients with SSc reported significantly higher frequencies (moderate to large effect size; P < 0.01) on 4 CES-D somatic symptom items: bothered, appetite, effort, and sleep. Internet respondents had higher item scores on 2 items that assessed interpersonal difficulties (unfriendly, large effect size; P < 0.01; disliked, large effect size; P < 0.01) and on 2 items that assessed lack of positive effect (happy, moderate effect size; P = 0.01; enjoy, large effect size; P < 0.01). Adjustment of standard CES-D cutoff criteria for potential bias due to somatic symptom reporting resulted in a reduction of only 3.6% in the number of SSc patients with significant symptoms of depression. CONCLUSION: High rates of depressive symptoms in SSc are not due to bias related to the report of somatic symptoms. The pattern of differential item functioning between the SSc and Internet groups, however, suggests some qualitative differences in depressive symptom presentation.

Quality of life in systemic sclerosis: psychometric properties of the World Health Organization Disability Assessment Schedule II.

OBJECTIVE: To determine the validity of the World Health Organization Disability Assessment Schedule II (WHODAS II) in systemic sclerosis (SSc). METHODS: Patients enrolled in the Canadian Scleroderma Research Group registry participated in a standardized evaluation and completed the WHODAS II. Criterion validity was assessed by comparing the WHODAS II with the Medical Outcomes Study Short Form 36 (SF-36), construct validity was assessed by examining how it relates to common measures of outcome in SSc, and discriminative validity was assessed by examining how it distinguishes patients with more severe disease from those with less severe disease. RESULTS: A total of 402 patients with SSc were included (mean +/- SD age 55 +/- 13 years, 87% women, mean +/- SD disease duration 11 +/- 9 years). The mean +/- SD WHODAS II score was 24.6 +/- 17.4, and the greatest impairments were in life activities and mobility. There were moderate to good correlations between the WHODAS II and the SF-36 Physical Component Summary score (r = -0.44), the SF-36 Mental Component Summary score (r = -0.41), and measures of function (r = 0.54), depression (r = 0.44), pain (r = 0.40), and fatigue (r = -0.49, P < 0.0001 for all). The WHODAS II was able to consistently distinguish patients with milder disease from those with more severe disease. CONCLUSION: The WHODAS II had good psychometric properties in patients with SSc and should be considered a valid measure of health-related quality of life in SSc.

Clinical correlates of quality of life in systemic sclerosis measured with the World Health Organization Disability Assessment Schedule II.

OBJECTIVE: To identify the clinical characteristics of systemic sclerosis (SSc) that best correlate with the health-related quality of life (HRQOL) of patients with SSc, using the World Health Organization Disability Assessment Schedule II (WHODAS II) as the measure of HRQOL. METHODS: A cross-sectional, multicenter study of 337 patients from the Canadian Scleroderma Research Group Registry was conducted. Patients were assessed through detailed clinical histories, medical examination, and the WHODAS II. Hierarchical multiple linear regression was used to assess the relationship between selected clinical variables and HRQOL. RESULTS: The mean WHODAS II score was 23.7 (range 0-100), with the greatest impairments seen in the subscales measuring life activities, mobility, and participation in society. In multivariate analysis, significant predictors of the WHODAS II were skin scores, shortness of breath, number of gastrointestinal problems, fatigue, pain, and depression. The final model explained 61% of the variance in the WHODAS II scores. CONCLUSION: The clinical characteristics identified in this study as significant correlates of HRQOL in SSc should each be targets of intervention in order to improve the HRQOL of patients with this disease.

Hypocomplementemia in systemic sclerosis--clinical and serological correlations.

OBJECTIVE: Although complement fixation is not commonly thought to be part of the pathogenesis of systemic sclerosis (SSc), hypocomplementemia has been associated with SSc. We hypothesized that hypocomplementemia in SSc might indicate the presence of overlap disease. We investigated if SSc patients with hypocomplementemia had more features of overlap disease than those with normal complement levels. METHODS: Study subjects consisted of those enrolled in the Canadian Scleroderma Research Group Registry. Patients were divided into 2 groups: those with normal complement levels (normal C3 and C4) and those with hypocomplementemia (low C3 or C4). Evidence of overlap disease was defined as physician reports of other specific rheumatic conditions. Autoantibodies were assayed. Differences in rates of concomitant diseases and in antibody profiles were compared between groups. RESULTS: Our study included 321 patients (88% women, mean age 56 +/- 13 yrs, mean disease duration 11 +/- 9 yrs). Of these, 276 (86%) had normal complements and 45 (14%) had hypocomplementemia. Patients with hypocomplementemia were significantly more likely to have physician-reported inflammatory myositis (27% vs 12%; p < 0.008) and vasculitis (11% vs 2%; p < 0.011) than those with normal complement. There was also a trend toward more antichromatin antibodies (18% vs 9%; p = 0.051) in patients with hypocomplementemia compared to normals. CONCLUSION: Hypocomplementemia may identify a particular subgroup of SSc patients who have overlap disease.

Depression in patients with systemic sclerosis: a systematic review of the evidence.

OBJECTIVE: To assess the prevalence, course, and predictors of depression in patients with systemic sclerosis (SSc). METHODS: We conducted a comprehensive search in November 2006 of MEDLINE, PsycINFO, and CINAHL databases to identify original research studies published in any language that used a structured interview or validated questionnaire to assess major depressive disorder or clinically significant symptoms of depression in patients with SSc. The search was augmented by hand searching 26 selected journals through December 2006 and references from identified articles and reviews. Studies were excluded if only an abstract was provided or if depression was not measured by a validated method. RESULTS: No studies used a structured clinical interview to assess the prevalence of major depressive disorder. The prevalence of clinically significant depressive symptoms was 51-65% based on 2 studies that used a Beck Depression Inventory (BDI) score >or=10 and 46-56% based on 2 studies that used a BDI score >or=11. These rates and those reported in 4 other studies that used different assessment tools (36-43%) were consistently high compared with other medical patient groups assessed with the same instruments and cutoffs. Methodologic issues limited the ability to draw strong conclusions from studies of predictors. CONCLUSION: Symptoms of depression are common among patients with SSc. The high rates reported across studies suggest that routine screening is recommended. There is a need for studies that examine depression at different time points from the diagnosis of SSc and that systematically investigate factors associated with high levels of depressive symptoms.

The development of systemic sclerosis classification criteria.

Systemic sclerosis (SSc) is a rare connective tissue disorder whose aetiology remains obscure, although environmental and genetic influences are likely to play a role. Disease registries have contributed to enhancing our understanding of this debilitating illness, but without sensitive, specific, and extensively validated classification criteria, accurate comparison between registries and the identification of patients suitable for clinical trials can be problematic. The American College of Rheumatology (ACR) criteria, published in 1980, have become outdated as our understanding of disease specific autoantibodies and nailfold capillaroscopy has improved. In addition, the sensitivity of the ACR criteria is low with respect to limited SSc. Although subsequent classification systems have been proposed, none has gained universal approval. The two- versus three-subset disease model remains a point of debate. Newly derived criteria are likely to draw upon the older classification systems as well as incorporating up-to-date diagnostic techniques and biomarkers. Validation will be critical before their use becomes widespread.

Office capillaroscopy in systemic sclerosis.

The aims of this study are to assess the reliability of two office techniques, the ophthalmoscope and the Dermlite dermatoscope, and to detect nailfold capillaroscopy abnormalities in systemic sclerosis (SSc). Two separate studies were performed. In the first, the nailfolds of two fingers on one hand of 13 SSc patients and two normals were examined by four rheumatologists using an ophthalmoscope. In the second, the nailfolds of the two fingers of each hand of six SSc patients and two normals were examined by six rheumatologists with a Dermlite dermatoscope. Widefield capillary microscopy was performed by one observer in the ophthalmoscope study to assess validity. The examiners determined the presence or absence of dilated loops, giant capillary loops, and/or avascular areas on each digit. The kappa coefficient was calculated to demonstrate agreement. With the ophtalmoscope, the inter-observer kappa coefficients were 0.43, 0.54, and 0.19; the average intra-observer agreements were 0.61, 0.56, and 0.31; and the ophthalmoscope-microscope agreement were 0.63, 0.52, and <0.1 for dilated capillaries, giant capillaries, and avascular areas, respectively. With the dermatoscope, the kappa values for inter-observer reliability were 0.63, 0.40, and 0.20; and intra-observer reliability was 0.71, 0.55, and 0.40 for dilated capillaries, giant capillaries, and avascular areas, respectively. The ophthalmoscope and the dermatoscope provide moderate to substantial reliability to detect the presence of giant and dilated capillaries but poor inter-observer agreement for avascular areas. The ophthalmoscope is valid when compared to the microscope for detecting giant or dilated capillaries. We conclude that these techniques are useful office tools to detect capillary abnormalities in SSc.