Abstract
Objectives
SSc is associated with increased health-care resource utilization and economic burden. The Collaborative National Quality and Efficacy Registry (CONQUER) is a US-based ...collaborative that collects longitudinal follow-up data on SSc patients with <5 years of disease duration enrolled at scleroderma centres in the USA. The objective of this study was to investigate the relationship between gastrointestinal tract symptoms and self-reported resource utilization in CONQUER participants.
Methods
CONQUER participants who had completed a baseline and 12-month Gastrointestinal Tract Questionnaire (GIT 2.0) and a Resource Utilization Questionnaire (RUQ) were included in this analysis. Patients were categorized by total GIT 2.0 severity: none-to-mild (0–0.49); moderate (0.50–1.00), and severe-to-very severe (1.01–3.00). Clinical features and medication exposures were examined in each of these categories. The 12-month RUQ responses were summarized by GIT 2.0 score categories at 12 months.
Results
Among the 211 CONQUER participants who met the inclusion criteria, most (64%) had mild GIT symptoms, 26% had moderate symptoms, and 10% severe GIT symptoms at 12 months. The categorization of GIT total severity score by RUQ showed that more upper endoscopy procedures and inpatient hospitalization occurred in the CONQUER participants with severe GIT symptoms. These patients with severe GIT symptoms also reported the use of more adaptive equipment.
Conclusion
This report from the CONQUER cohort suggests that severe GIT symptoms result in more resource utilization. It is especially important to understand resource utilization in early disease cohorts when disease activity, rather than damage, primarily contributes to health-related costs of SSc.
Pregnancy in scleroderma Steen, Virginia D
Rheumatic diseases clinics of North America,
05/2007, Letnik:
33, Številka:
2
Journal Article
Recenzirano
Pregnancy in systemic sclerosis may be uneventful with good maternal and fetal outcomes. Scleroderma is a multisystem disease and complications do occur; however, careful antenatal evaluations, ...discussion of potential problems, and participation in a high-risk obstetric monitoring program are important to optimize the best outcome. Women who have diffuse scleroderma are at a greater risk for developing serious cardiopulmonary and renal problems early in the disease so they should be encouraged to delay pregnancy until the disease stabilizes. All patients who become pregnant during this high-risk time should be monitored extremely carefully, particularly for renal crisis.
The objectives of this study were to develop a standard classification of digital ulcers (DUs) in systemic sclerosis (SSc) for use in observational or therapeutic studies and to assess the ...reliability of these definitions as well as of the measurement of ulcer area. Ten North American rheumatologists with expertise in SSc reviewed multiple photos of DUs, examined four SSc subjects with DUs, and came to a consensus on the definitions for digital, active, healed, and indeterminate ulcers. These ten raters then examined the right hand of ten SSc subjects twice and the left hand once to classify ulcers and to measure ulcer area. Weighted and Fleiss kappa were used to calculate intra- and interrater agreement on classification of ulcers, and intraclass correlation coefficient (ICC) was used to assess agreement on ulcer area. Because the traditional ICC calculations relied on a small number of ulcers, ICCs were recalculated using the results of linear mixed models to evaluate the variance components of observations on all the data. Intrarater kappa for classifying DU as not an ulcer/healed ulcer versus active/indeterminate ulcer was substantial (0.76), and interrater kappa was moderate (0.53). The ICC for ulcer area using the linear mixed models was moderate both for intrarater (0.57) and interrater (0.48) measurements. A consensus for the classification of DUs in SSc was developed, and after a training session, rheumatologists with expertise in SSc are able to reliably classify DUs and to measure ulcer area.
To evaluate patients with systemic sclerosis (SSc) who have gastric antral vascular ectasia (GAVE), to further characterize this disease association, and to identify factors that may predict which ...patients with SSc are at greatest risk for the development of GAVE.
Patients with a diagnosis of both SSc and GAVE were identified from the Division of Rheumatology at Georgetown University and Thomas Jefferson University. A chart review was conducted to obtain the demographic data.
Twenty-eight patients were included in this analysis, including 17 with diffuse cutaneous (dcSSc) and 11 with limited cutaneous SSc (lcSSc). The mean disease duration at diagnosis with GAVE was 21.5 months for dcSSc and 84.3 months for lcSSc (p = 0.025). Seventy-six percent of patients with dcSSc developed GAVE within 18 months of first scleroderma symptom onset. Over half of patients with early GAVE also had rapidly progressive cutaneous disease. Only 4% had antitopoisomerase I antibody. Although only 1 patient was tested and had positive RNA polymerase (RNAP) III, RNAP III may be overrepresented in this GAVE population. Mean hematocrit levels were 23.8% in dcSSc and 29% in lcSSc.
dcSSc is associated with earlier development of GAVE, as well as more severe anemia requiring more therapeutic interventions. Rapid progression of cutaneous disease may suggest earlier development of GAVE. Absence of antitopoisomerase I antibodies and presence of antibodies to RNAP III/speckled antinuclear antibody pattern may be useful to identify the subset of patients with SSc with increased risk for GAVE.
Systemic sclerosis–associated interstitial lung disease is challenging to diagnose and treat. Patients and physicians can perceive the disease differently and have different views on its management. ...Communication issues between them can lead to suboptimal disease management. Despite a clear need for improvement in the speed and accuracy of the diagnostic workup, the heterogeneity of clinical symptoms renders the process long and challenging. When considering treatment options, physicians may be more focused on the evidence supporting a particular treatment or on a patient’s pulmonary function test results, as opposed to the realities of the patient’s difficulties with symptoms or the psychosocial effects of systemic sclerosis–associated interstitial lung disease. Disease management plans should be determined by the patient’s own preferences and goals as well as the objective clinical situation. Health care providers must consider their patients as partners on a journey in which treatment decisions are reached jointly. This review will focus on the perspectives of physicians and patients in relation to the diagnosis and management of systemic sclerosis–associated interstitial lung disease. Similarities and differences in these perspectives will be identified, and strategies for achieving optimal disease management will be proposed.
Pulmonary arterial hypertension (PAH) is one of the leading causes of death in patients with systemic sclerosis (SSc). Given the high prevalence and poor survival of SSc-PAH, and that aggressive ...management of mild disease may be associated with better outcomes, screening is critical. Right heart catheterization (RHC) is the gold standard for the definitive diagnosis of PAH, and should be performed in those patients in whom this diagnosis is suspected. Once a diagnosis of PAH is confirmed by RHC, treatment with PAH-specific therapies should be initiated as soon as possible.
Studies have shown a high degree of body image dissatisfaction among patients with systemic sclerosis (SSc). We aimed to identify demographic and phenotypic characteristics that correlate with body ...image dissatisfaction.
Ninety-eight patients with SSc were recruited from Georgetown University Medical Center 2003-2004. Anonymous surveys collected demographic information (age, race, gender, duration/type of SSc) and assessed degree of body image dissatisfaction on a scale of 0-3 in relation to phenotypic features of SSc (hand contractures, finger ulcers, pigmentation changes, lip wrinkling/thinning, telangiectasias). A composite total distress score was derived. Parametric and nonparametric T tests were used to compare groups.
Of 98 patients, 86 were female and 12 male. The majority of patients were 30-60 years old. The sample was 62% Caucasian, 27% African American, and the rest identified as "other". Twenty-seven percent had limited SSc, 48% diffuse, and 25% "other". African American patients had greater total body image dissatisfaction (p=0.002), specifically with respect to digital ulcers, pruritus, and pigmentation changes, than Caucasian participants. Patients with diffuse SSc had greater body image dissatisfaction than those with limited disease (p=0.002).
Our results suggest that African American patients with SSc and those with diffuse subtype suffer a higher degree of body image dissatisfaction. Screening for and addressing this issue in SSc patients is prudent. Further study is needed to understand racial differences in body image dissatisfaction among patients with SSc.
To describe the differences between patients with systemic sclerosis (SSc) having childhood versus adult onset evaluated at a single medical center.
Patients were divided into those with childhood ...onset (first SSc symptom or finding before age 16 yrs) and those with early adult and late adult onset. The 3 groups were compared with respect to disease classification, clinical, laboratory and serologic data, and survival.
One hundred eleven childhood onset SSc cases seen between 1960 and 2003 were compared with 2559 adult onset SSc cases (1087 with onset age 16-40 and 1472 with onset after age 40 yrs) first evaluated between 1972 and 2001. Age distribution at onset was unimodal, suggesting that childhood disease is part of the spectrum of adult onset SSc. A significantly greater proportion of childhood onset patients had overlap syndromes, most frequently with polymyositis-dermatomyositis (PM-DM), and skeletal muscle involvement. Children with diffuse cutaneous (dc) SSc had significantly lower maximum mean total skin thickness scores than adult patients with dcSSc. Renal involvement was uncommon in childhood onset cases, and the frequency increased with age of onset. Serum anti-PM-Scl and anti-U1RNP antibodies were detected significantly more frequently in childhood than in adult onset cases. In contrast, anti-RNA polymerase III and anticentromere antibodies were found significantly more frequently in adults. Survival was significantly better among childhood than all adult onset cases combined, but similar to survival in young adult onset SSc cases. Scleroderma heart disease was a frequent cause of death among children with SSc.
Patients with juvenile onset SSc more frequently have an overlap syndrome with PM-DM, higher frequency of skeletal muscle involvement, serum anti-PM-Scl and anti-U1RNP antibody, fatal cardiac disease, and improved survival compared with adult onset SSc cases.
Objective
The University of California in San Diego Shortness of Breath Questionnaire (UCSD SOBQ) has been used to assess dyspnea‐related activity limitation in patients with airway and parenchymal ...lung disease. We sought to assess the construct validity and responsiveness of the UCSD SOBQ in systemic sclerosis (SSc; scleroderma) patients with incident pulmonary hypertension (PH) and those at high risk of developing PH.
Methods
We used data from 179 patients enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry with pre‐PH (defined by criteria on pulmonary function tests and/or echocardiogram) or definite PH with mean pulmonary artery pressure ≥25 mm Hg by right‐sided heart catheterization within 6 months of enrollment. For this analysis, we included those subjects with complete data for self‐reported measures at baseline and at 12 months.
Results
At baseline, the UCSD SOBQ had strong correlations in the expected direction with the disability index (DI) of the Health Assessment Questionnaire (HAQ) (r = 0.71, P < 0.0001), dyspnea assessment by visual analog scale (r = 0.71, P < 0.0001), and the Short Form 36 (SF‐36) health survey physical component summary (PCS) score (r = −0.77, P < 0.0001), as well as a moderate correlation with the 6‐minute walk test distance (r = −0.33, P < 0.0001), Borg dyspnea score (r = 0.47, P < 0.0001), and diffusing capacity of carbon monoxide (r = −0.33, P < 0.0001). Change in the UCSD SOBQ at 12 months correlated in the expected direction with change in the HAQ DI (r = 0.54, P < 0.0001) and change in the SF‐36 PCS (r = −0.44, P < 0.0001). Multivariate analysis adjusting for age, sex, and race identified male sex as a significant predictor of death (odds ratio OR 7.00, 95% confidence interval 95% CI 1.55–31.76), while the UCSD SOBQ showed a strong trend toward significance (OR 1.82, 95% CI 0.97–3.41).
Conclusions
The UCSD SOBQ demonstrates good construct validity and responsiveness to change in SSc patients with pulmonary vascular disease.
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