Including Distal Motor Function within the NIHSS: Correlation with Motor Arm Function and IV rt-PA Treatment Response.

OBJECTIVES: The Distal Motor Function (DMF) sub-score of the NIH Stroke Scale (NIHSS) was measured in the NINDS rt-PA Stroke Trials but is currently not included in the NIHSS. The correlation of DMF with the NIHSS Motor Arm Function (MAF) sub-score, the effect of IV tPA treatment on DMF, and whether adding DMF changes the utility of the NIHSS have not been analyzed. MATERIALS AND METHODS: MAF and DMF sub-scores were retrieved from the original NINDS rt-PA Stroke Trials for both sides of the body at baseline, 2 hours, 24 hours, 7-10 days, and 3 months after IV tPA treatment. MAF and DMF scores were correlated using Spearman correlation. Clustering of DMF and MAF scores was determined using a Bentler Comparative Fit Index (CFI) to estimate variation in NIHSS when adding DMF. The effect of IV tPA on DMF and MAF was assessed using a linear model comparing changes in scores from baseline to 3 months. RESULTS: MAF and DMF were highly correlated (p < 0.0001) across all time points for both dichotomous and continuous data on both sides. Intravenous tPA accounted for 21% of the change in DMF (p < 0.014, R2 = 0.0157, N = 423) and 39% of the change in MAF (p < 0.093, R2 = 0.0125, N = 547) from 0 to 3 months. On adding DMF to NIHSS, CFI decreased from 0.98 to 0.80 and DMF clustered with MAF, indicating that addition of DMF is unlikely to produce any discrepancy to NIHSS. CONCLUSIONS: Including DMF to the NIHSS does not appear to be of additional value. After IV tPA treatment, proximal and distal motor function in upper extremity strongly correlate over time but greater improvement in MAF is noted. Further research is needed on the role of IV tPA on minor strokes with deficits of DMF.

D-cycloserine combined with cue exposure therapy fails to attenuate subjective and physiological craving in cocaine dependence.

BACKGROUND: Based on preclinical studies showing that the partial N-methyl-D-aspartate (NMDA) agonist D-cycloserine (DCS) facilitates extinction of cocaine self-administration and cocaine-induced conditioned place preference, we evaluated whether 50 mg of DCS would reduce craving to cocaine cues when combined with cue exposure (CE) in cocaine dependent humans. METHODS: In this double-blind placebo-controlled pilot study, 47 cocaine dependent participants were randomized to DCS or placebo (PBO), plus CE. Participants received DCS or PBO 30 minutes prior to two CE sessions, conducted one day apart. Craving and heart rate was assessed prior to CE sessions, during CE trials, and after CE trials. These measures were assessed again at a 1-week follow-up (session 3) after the second CE session. RESULTS: DCS failed to significantly attenuate cocaine cue reactivity based on subjective craving and physiological reactivity (heart rate) compared to PBO. The CE protocol, consisting of repeated exposure to drug cues combined with skills training, resulted in extinction to cocaine cues as suggested by decreased craving within and between sessions in both treatment conditions. All participants exhibited elevated heart rate with repeated exposures, demonstrating a potentiation in heart rate between sessions. CONCLUSIONS: 50 mg of DCS may not be effective for extinguishing reactivity to drug cues for individuals with cocaine dependence. SCIENTIFIC SIGNIFICANCE: Future studies examining the effect of DCS on facilitating extinction to drug cues should examine variations in cue exposure length, number of CE presentations, and timing of DCS dose administration prior to cue exposures, which may differentially impact drug cue reactivity.

Raynaud phenomenon and mortality: 20+ years of follow-up of the Charleston Heart Study cohort.

BACKGROUND: Raynaud phenomenon (RP) is a temporary vasoconstrictive condition that often manifests itself in the fingers in response to cold or stress. It often co-occurs with certain chronic diseases that impact mortality. Our objective was to determine whether RP has any independent association with survival. METHODS: From 1987-1989, a total of 830 participants of the Charleston Heart Study cohort completed an in-person RP screening questionnaire. Two definitions of RP were used: a broad definition that included both blanching (pallor) and cyanotic color changes and a narrow definition that included only blanching. All-cause and cardiovascular disease (CVD) mortality were compared between subjects with and without RP using race-specific survival models that adjusted for age, sex, baseline CVD, and 10-year risk of coronary heart disease. RESULTS: Using the narrow RP definition, we identified a significant interaction between older age and the presence of RP on all-cause mortality. In the broad RP definition model, the presence of RP was not associated with CVD mortality among blacks; however, among whites, the presence of RP was associated with a 1.6-fold increase in the hazard associated with CVD-related death (hazard ratio: 1.55, 95% confidence interval: 1.10-2.20, P=0.013). CONCLUSION: RP was independently associated with mortality among older adults in our cohort. Among whites, RP was associated with increased CVD-related death. It is possible that RP may be a sign of undiagnosed vascular disease.

Outcome of patients with negative and unsatisfactory cytologic specimens obtained by endobronchial ultrasound-guided transbronchial fine-needle aspiration of mediastinal lymph nodes.

BACKGROUND: Endobronchial ultrasound-guided transbronchial fine-needle aspiration (EBUS-FNA) has gained acceptance as the diagnostic procedure of choice with which to sample hilar and mediastinal lymph nodes (LNs) for diagnosing and staging patients with lung cancer. Studies have shown that EBUS has a high positive predictive value; however, its negative predictive value (NPV) varies significantly. The aim of the current study was to evaluate the clinical outcome surrounding negative and nondiagnostic EBUS-FNA of mediastinal LNs. METHODS: A retrospective chart review of cases of EBUS-FNA performed between 2008 and the middle of 2011 was conducted. Mediastinal LNs with cytologic diagnoses of negative for malignant cells and unsatisfactory were selected for the study. Each LN was followed for up to 1 year with imaging or biopsy/surgical resection. A true-negative result was defined as a LN that did not enlarge on repeat imaging or was negative for malignancy on repeat biopsy or surgery during the follow-up period. RESULTS: Among 1418 LNs sampled, 479 from 228 patients met the search criteria, including 394 LN (82.3%) with the cytologic diagnosis of negative for malignant cells and 85 (17.8%) with a diagnosis of unsatisfactory. A total of 104 patients (45.6%) were followed with imaging, and 124 patients (54.3%) underwent repeat biopsy/surgery. A total of 445 LNs met the definition of a true-negative finding, resulting in an overall NPV of 92.9% (95% confidence interval [95% CI], 90.6%-95.2%). The NPVs of a negative and unsatisfactory diagnosis were 93.9% (95% CI, 91.6%-96.3%) and 88.2% (95% CI, 81.4%-95.1%), respectively. CONCLUSIONS: The vast majority of LNs with a cytologic diagnosis of negative and unsatisfactory were likely to be true-negative findings. In these patients, a more conservative approach to follow-up may be appropriate.

Genes associated with SLE are targets of recent positive selection.

The reasons for the ethnic disparities in the prevalence of systemic lupus erythematosus (SLE) and the relative high frequency of SLE risk alleles in the population are not fully understood. Population genetic factors such as natural selection alter allele frequencies over generations and may help explain the persistence of such common risk variants in the population and the differential risk of SLE. In order to better understand the genetic basis of SLE that might be due to natural selection, a total of 74 genomic regions with compelling evidence for association with SLE were tested for evidence of recent positive selection in the HapMap and HGDP populations, using population differentiation, allele frequency, and haplotype-based tests. Consistent signs of positive selection across different studies and statistical methods were observed at several SLE-associated loci, including PTPN22, TNFSF4, TET3-DGUOK, TNIP1, UHRF1BP1, BLK, and ITGAM genes. This study is the first to evaluate and report that several SLE-associated regions show signs of positive natural selection. These results provide corroborating evidence in support of recent positive selection as one mechanism underlying the elevated population frequency of SLE risk loci and supports future research that integrates signals of natural selection to help identify functional SLE risk alleles.

The utility of nodule volume in the context of malignancy prediction for small pulmonary nodules.

BACKGROUND: An estimated 150,000 pulmonary nodules are identified each year, and the number is likely to increase given the results of the National Lung Screening Trial. Decision tools are needed to help with the management of such pulmonary nodules. We examined whether adding any of three novel functions of nodule volume improves the accuracy of an existing malignancy prediction model of CT scan-detected nodules. METHODS: Swensen's 1997 prediction model was used to estimate the probability of malignancy in CT scan-detected nodules identified from a sample of 221 patients at the Medical University of South Carolina between 2006 and 2010. Three multivariate logistic models that included a novel function of nodule volume were used to investigate the added predictive value. Several measures were used to evaluate model classification performance. RESULTS: With use of a 0.5 cutoff associated with predicted probability, the Swensen model correctly classified 67% of nodules. The three novel models suggested that the addition of nodule volume enhances the ability to correctly predict malignancy; 83%, 88%, and 88% of subjects were correctly classified as having malignant or benign nodules, with significant net improved reclassification for each (P<.0001). All three models also performed well based on Nagelkerke R2, discrimination slope, area under the receiver operating characteristic curve, and Hosmer-Lemeshow calibration test. CONCLUSIONS: The findings demonstrate that the addition of nodule volume to existing malignancy prediction models increases the proportion of nodules correctly classified. This enhanced tool will help clinicians to risk stratify pulmonary nodules more effectively.

Comparing the symptoms of posttraumatic stress disorder with the distress and fear disorders.

New theoretical models of mood and anxiety disorders have been proposed to better understand the relations and patterns leading to their high diagnostic comorbidities. These models have highlighted two new groupings of the disorders, focused on the prevalence of fear and distress symptoms. The present study investigated the fit of the symptoms of posttraumatic stress disorder (PTSD) in these new models. The relations between the two primary sets of symptom scales of PTSD and the diagnoses of other comorbid disorders were examined in a large multisite sample of veterans from primary care clinics. The results suggested that there was no reliable difference in the predictive power of any of the PTSD symptom scales across the two diagnostic groups. New transdiagnostic models, assessment practices, and treatment approaches may provide better understanding of symptom overlap and diagnostic comorbidity in PTSD and related disorders.

The health education for lupus study: a randomized controlled cognitive-behavioral intervention targeting psychosocial adjustment and quality of life in adolescent females with systemic lupus erythematosus.

INTRODUCTION: To examine in a randomize controlled feasibility clinical trial the efficacy of a cognitive-behavioral intervention designed to manage pain, enhance disease adjustment and adaptation and improve quality of life among female adolescents with systemic lupus erythematosus. METHODS: Female adolescents (n = 53) ranging in age from 12 to 18 years were randomly assigned to 1 of 3 groups including a cognitive-behavioral intervention, an education-only arm and a no-contact control group. Participants were assessed at baseline, postintervention and at 3- and 6-month intervals after completion of the intervention. RESULTS: No significant differences were revealed among the 3 treatment arms for any of the dependent measures at any of the assessment points. For the mediator variables, a posthoc secondary analysis did reveal increases in coping skills from baseline to postintervention among the participants in the cognitive-behavioral intervention group compared with both the no-contact control group and the education-only group. CONCLUSION: Although no differences were detected in the primary outcome, a possible effect on coping of female adolescents with systemic lupus erythematosus was detected in this feasibility study. Whether the impact of training in the area of coping was of sufficient magnitude to generalize to other areas of functioning, such as adjustment and adaptation, is unclear. Future phase III randomized trials will be needed to assess additional coping models and to evaluate the dose of training and its influence on pain management, adjustment and health-related quality of life.

Demographic differences in the treatment and control of glucose in type 2 diabetic patients: implications for health care practice.

OBJECTIVE: Identifying modifiable covariables that reduce demographic disparities in controlling type 2 diabetes could inform efforts to improve health equity. RESEARCH DESIGN AND METHODS: This retrospective study utilized electronic health record data on 22,285 adults with type 2 diabetes seen at 110 outpatient clinics in the Southeast U.S. from 2004-2008. Demographic differences in diabetes control and modifiable covariables which reduce those disparities were quantified using descriptive and logistic regression analysis. RESULTS: Patients were 55.8 +/- 14.6 (SD) years old, 57.5% women, 61.0% white: 39.0% black and had baseline body mass index 34. +/- .3 kg/ m2 and HbA1c 7.61 +/- 1.9%. The percentage with HbAlc <7% was higher in Whites than blacks (55.6% vs. 44.7%, P < .0001) and rose with age in all patients from 45.3% at <50, to 50.0% at 50-64, and 59.6% at > or =65 years, P < .001. white vs. black race (odds ratio [OR] 1.59, 95% confidence interval [CI] 1.51-1.68) and age/ 10 years (OR 1.20/10 years, 95% CI 1.17-1.22) were predictors of HbAlc <7% in univariable logistic regression. In multivariable analysis, three modifiable covariables (initial HbAlc, therapeutic inertia, visit frequency) accounted for 47.9% of variance in diabetes control. When accounting for these modifiable covariables, the independent impact of race/ethnicity (OR 1.21, 95% CI 1.13-1.30) and age (OR 1.13, 95% Cl 1.11-1.16) on HbA1c control declined. CONCLUSIONS: Race and age-related difference in diabetes control declined significantly when modifiable covariates were considered. Greater attention to early diagnosis and treatment, ensuring regular healthcare visits and overcoming therapeutic inertia could improve diabetes control and health equity.

Initial monotherapy and combination therapy and hypertension control the first year.

Initial antihypertensive therapy with single-pill combinations produced more rapid blood pressure control than initial monotherapy in clinical trials. Other studies reported better cardiovascular outcomes in patients achieving lower blood pressure during the first treatment year. We assessed the effectiveness of initial antihypertensive monotherapy, free combinations, and single-pill combinations in controlling untreated, uncontrolled hypertensives during their first treatment year. Electronic record data were obtained from 180 practice sites; 106 621 hypertensive patients seen from January 2004 to June 2009 had uncontrolled blood pressure, were untreated for ≥ 6 months before therapy, and had ≥ 1 one-year follow-up blood pressure data. Control was determined by the first follow-up visit with blood pressure <140/<90 mm Hg for patients without diabetes mellitus or chronic kidney disease and <130/<80 mm Hg for patients with either or both conditions. Multivariable hazards regression ratios (HRs) and 95% CIs for time to control were calculated, adjusting for age, sex, baseline blood pressure, body mass index, diabetes mellitus, chronic kidney disease, cardiovascular disease, initial therapy, final blood pressure medication number, and therapeutic inertia. Patients on initial single-pill combinations (N = 9194) were more likely to have stage 2 hypertension than those on free combinations (N = 18 328) or monotherapy (N = 79 099; all P<0.001). Initial therapy with single-pill combinations (HR, 1.53 [95% CI, 1.47-1.58]) provided better hypertension control in the first year than free combinations (HR, 1.34; [95% CI, 1.31-1.37]) or monotherapy (reference) with benefits in black and white patients. Greater use of single-pill combinations as initial therapy may improve hypertension control and cardiovascular outcomes in the first treatment year.

Motives for using: a comparison of prescription opioid, marijuana and cocaine dependent individuals.

Identification of the motives for drug use is critical to the development of effective interventions. Furthermore, consideration of the differences in motives for drug use across substance dependent populations may assist in tailoring interventions. To date, few studies have systematically compared motives for substance use across drug classes. The current study examined motives for drug use between non-treatment seeking individuals with current prescription opioid, marijuana, or cocaine dependence. Participants (N=227) completed the Inventory of Drug-Taking Situations (IDTS; Annis, Turner & Sklar,1997), which contains eight subscales assessing motives for drug use. The findings revealed that prescription opioid dependent individuals scored significantly higher than all other groups on the Physical Discomfort, Testing Personal Control and Conflict with Others subscales. Both the prescription opioid and cocaine dependent groups scored significantly higher than the marijuana group on the Urges or a Temptation to Use subscale. In contrast, marijuana dependent individuals scored highest on the Pleasant Emotions and Pleasant Times with Others subscales. The marked differences revealed in motives for drug use could be used in the development and implementation of specific treatment interventions for prescription opioid, marijuana and cocaine dependent individuals.

Premature atherosclerosis is associated with hypovitaminosis D and angiotensin-converting enzyme inhibitor non-use in lupus patients.

The ultimate goal is to identify and target modifiable risk factors that will reduce major cardiovascular events in African American lupus patients. As a first step toward achieving this goal, this study was designed to explore risk factor models of preclinical atherosclerosis in a predominantly African American group of patients with systemic lupus erythematosus (SLE) using variables historically associated with endothelial function in nonlupus populations. Fifty-one subjects with SLE but without a history of clinical cardiovascular events were enrolled. At entry, a Framingham risk factor history and medication list were recorded. Sera and plasma samples were analyzed for lipids, lupus activity markers and total 25-hydroxyvitamin D (25 OH)D) levels. Carotid ultrasound measurements were performed to determine total plaque area (TPA) in both carotids. Cases had TPA values above age-matched controls from a vascular prevention clinic population. Logistic regression and machine learning analyses were performed to create predictive models. 25(OH)D levels were significantly lower, and SLE disease duration was significantly higher in cases. 25(OH)D levels inversely correlated with age-adjusted TPA. Angiotensin-converting enzyme (ACE) inhibitor nonuse associated with case status. Logistic regression models containing ACE inhibitor use, 25(OH)D levels and low-density lipoprotein levels had a diagnostic accuracy of 84% for predicting accelerated atherosclerosis. Similar results were obtained with machine learning models, but hydroxychlo-roquine use associated with controls in these models. This is the first study to demonstrate an association between atherosclerotic burden and 25(OH)D insufficiency or ACE inhibitor nonuse in lupus patients. These findings provide strong rationale for the study of ACE inhibitors and vitamin D replenishment as preventive therapies in this high-risk population.

The Movement Disorders task force review of dysautonomia rating scales in Parkinson's disease with regard to symptoms of orthostatic hypotension.

Orthostatic hypotension is defined as a blood pressure fall of > 20 mm Hg systolic and/or 10 mm Hg diastolic within 3 minutes of an upright position. The Movement Disorders Society commissioned a task force to assess existing clinical rating scales addressing symptoms of orthostatic hypotension in Parkinson's disease. Seven neurologists and a clinimetrician assessed each scale's previous use and critiqued its clinimetric properties. A scale was "recommended" if it had been applied to populations of patients with Parkinson's disease, with data on its use in studies beyond the group that developed the scale, and was found to be clinimetrically valid. A scale was considered "suggested" if it had been applied to Parkinson's disease, but only 1 of the other criteria was applied. A scale was "listed" if it met only 1 criterion. Symptoms of orthostatic hypotension are generally assessed in scales on wider autonomic or nonmotor symptoms. Some scales designed to detect orthostatic hypotension-related symptoms provide information on their severity: the AUTonomic SCale for Outcomes in PArkinson's Disease and the COMPosite Autonomic Symptom Scale met criteria for recommended with some limitations; the Novel Non-Motor Symptoms Scale and the Orthostatic Grading Scale were classified as suggested. The Self-completed Non-Motor Symptoms Questionnaire for Parkinson's Disease was classified as suggested as a tool for screening orthostatic symptoms. However, these and the listed scales need further validation and application before they can be recommended for clinical use in patients with Parkinson's disease.

Movement Disorder Society-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS): scale presentation and clinimetric testing results.

We present a clinimetric assessment of the Movement Disorder Society (MDS)-sponsored revision of the Unified Parkinson's Disease Rating Scale (MDS-UPDRS). The MDS-UDPRS Task Force revised and expanded the UPDRS using recommendations from a published critique. The MDS-UPDRS has four parts, namely, I: Non-motor Experiences of Daily Living; II: Motor Experiences of Daily Living; III: Motor Examination; IV: Motor Complications. Twenty questions are completed by the patient/caregiver. Item-specific instructions and an appendix of complementary additional scales are provided. Movement disorder specialists and study coordinators administered the UPDRS (55 items) and MDS-UPDRS (65 items) to 877 English speaking (78% non-Latino Caucasian) patients with Parkinson's disease from 39 sites. We compared the two scales using correlative techniques and factor analysis. The MDS-UPDRS showed high internal consistency (Cronbach's alpha = 0.79-0.93 across parts) and correlated with the original UPDRS (rho = 0.96). MDS-UPDRS across-part correlations ranged from 0.22 to 0.66. Reliable factor structures for each part were obtained (comparative fit index > 0.90 for each part), which support the use of sum scores for each part in preference to a total score of all parts. The combined clinimetric results of this study support the validity of the MDS-UPDRS for rating PD.

Autoantibody prevalence and lupus characteristics in a unique African American population.

OBJECTIVE: The Gullah population of the Sea Islands of South Carolina is a unique group of African Americans who, due to geographic and cultural factors, remained isolated with minimal genetic admixture until the 1950s. Because of the unique homogeneous nature of the Gullah, we sought to define the genetic and environmental factors contributing to systemic lupus erythematosus (SLE) in this population. METHODS: Using data from our ongoing cohort study of lupus in the Gullah population, which we established in 2003, disease characteristics and serologic profiles were summarized for 184 patients with SLE, 144 unaffected first-degree relatives, and 144 matched unrelated, unaffected control subjects. These findings were compared with those in 2 other large cohorts of African Americans with SLE. RESULTS: In the Gullah cohort, we observed a high prevalence of SLE multiplex families (26.6%), malar rash (56.0%), discoid rash (34.2%), photosensitivity (60.9%), and oral/nasal ulcerations (43.5%), but a lower prevalence of hematologic and pleuropericardial disease than has been reported in other African American cohorts. Overall renal and central nervous system involvement, number of American College of Rheumatology disease criteria met, and SLE Damage Index scores were similar to those reported in other cohorts. Of interest, male and female first-degree relatives and male and female control subjects in this cohort had similar rates of antinuclear antibody positivity, whereas lupus-specific antibodies were more prevalent in the women than in the men. CONCLUSION: These data indicate that the severity of lupus in the Gullah population is similar to that in other African American populations, whereas skin disease and familial disease prevalence are increased in the Gullah. These findings suggest that there is an increased genetic influence on overall disease in this cohort compared with that in other African American cohorts, which confirms the unique nature of this cohort.

Association of serum nitrate and nitrite levels with longitudinal assessments of disease activity and damage in systemic lupus erythematosus and lupus nephritis.

OBJECTIVE: Reactive intermediate production is an essential component of the innate immune response that is induced during disease activity in murine lupus. This study was undertaken to determine whether a marker of systemic nitric oxide (NO) production correlates with prospectively studied disease activity in human systemic lupus erythematosus (SLE) and lupus nephritis patients. METHODS: Eighty-three SLE patients and 40 control subjects were studied longitudinally. The SLE group included 23 patients with lupus nephritis documented by renal biopsy and 26 with a history of lupus nephritis. During each visit, following a 24-hour low-nitrate diet, traditional markers of disease activity and damage were determined. Serum nitrate plus nitrite (NOx) levels were determined by chemiluminescence detection. RESULTS: NOx levels were higher in SLE patients than in controls during the first visit. In univariate longitudinal analyses, NOx levels were associated with SLE Disease Activity Index scores. In multivariate analyses, NOx levels were associated with serum levels of C3 and creatinine and the urinary protein:creatinine ratio. Among patients with lupus nephritis, those with proliferative lesions had higher NOx levels, and higher NOx levels were associated with accumulation of renal damage and lack of response to therapy. CONCLUSION: This is the first study to prospectively demonstrate longitudinal associations between serum NOx levels and markers of SLE and lupus nephritis disease activity. The more pronounced association with proliferative lupus nephritis and with longitudinal response to lupus nephritis therapy provides a rationale for the study of reactive intermediates as biomarkers of disease activity and therapeutic targets in proliferative lupus nephritis.

Ethnic disparities among patients with pulmonary hypertension associated with systemic sclerosis.

OBJECTIVE: To examine a cohort of patients with systemic sclerosis (SSc) and pulmonary hypertension (PH) for ethnic disparities in clinical presentation, disease detection, or management. METHODS: Encounters of patients with SSc seen at the Medical University of South Carolina were recorded in a computerized database from November 1997 through January 2004. Patients were evaluated for discrepancy in disease manifestation and treatment. Evaluation criteria included patient ethnicity (by self report), age, disease duration from onset of first non-Raynaud's symptom, presence or absence of PH, incidence of diastolic dysfunction and left ventricular hypertrophy among patients with PH, severity of interstitial lung disease, and treatment course. RESULTS: African Americans were more likely than Caucasians to have diffuse cutaneous SSc (dcSSc) (69.9% vs 42.9%, p < 0.001) and they presented with PH (defined as right ventricular systolic pressure > 40 mm Hg by echocardiogram or mean pulmonary artery pressure > 25 mm Hg by right heart catheterization (RHC) at a younger age (60.9 yrs vs 49.0 yrs, p < 0.001). There were no ethnic disparities in time from onset of the first non-Raynaud's symptom to detection of PH, method of PH detection, or treatment modalities. Patients with PH were more likely to have diastolic dysfunction than those without PH (52.3% vs 35.9%, p = 0.011). CONCLUSION: In this cohort of patients, African Americans were more likely to have dcSSc. Among patients with PH, African Americans presented at a younger age than their Caucasian counterparts. Incidence of diastolic dysfunction was higher in the PH population. There were no significant ethnic disparities in time of progression to PH or in treatment modalities employed in our cohort.

Racial variation in clinical and immunological manifestations of systemic sclerosis.

OBJECTIVE: To clarify which racial differences in disease manifestations can be attributed to differences in other factors such as gender, education, disease classification, and disease duration. METHODS: The study included white and black patients with systemic sclerosis (SSc) treated at a university hospital rheumatology clinic between November 1997 and April 2003. Demographic, clinical, and immunological measurements were obtained on each subject. Using multivariable statistical techniques we assessed differences in disease manifestations between white and black patients after adjusting for gender and classification and duration of disease. RESULTS: Two hundred sixty-three patients (199 whites, 64 blacks) were enrolled in the study. Blacks experienced an earlier age at disease onset than whites and were significantly more likely to have diffuse disease, digital ulcers, digital pitting, impaired lung function, and anti-RNP, and anti-Ro antibodies. Whites were significantly more likely to have anti-centromere antibodies. CONCLUSION: After adjusting for gender, disease classification, and disease duration, whites and blacks with SSc differ in some clinical and immunological manifestations of disease. Whether these differences can be attributed to genetic or environmental factors remains unknown.

Vitamin D deficiency in systemic lupus erythematosus.

Evidence from animal models and prospective studies of RA, multiple sclerosis, and type-1 diabetes suggest an important role for vitamin D as a modifiable environmental factor in autoimmune disease. This role has not been well studied in human SLE. We compared serum 25-hydroxyvitamin D (25(OH)D) levels between recently diagnosed SLE cases and matched controls, and examined disease characteristics in relationship to 25(OH)D among cases. Data from a population-based cohort of 123 recently diagnosed SLE patients and 240 controls were used. We found a trend toward lower 25(OH)D levels in cases compared to controls, which was statistically significant in Caucasians (p=0.04), controlling for age, sex, season, and smoking. Overall, 67% of the subjects were vitamin D deficient, with mean levels significantly lower among African Americans (15.9 ng/ml) compared to Caucasians (31.3 ng/ml). Critically low vitamin D levels (<10 ng/ml) were found in 22 of the SLE cases, with presence of renal disease being the strongest predictor (OR 13.3, p<0.01) followed by photosensitivity (OR 12.9, p<0.01). These results suggest vitamin D deficiency as a possible risk factor for SLE and provide guidance for future studies looking at a potential role of vitamin D in the prevention and/or treatment of SLE.

Demographic and clinical factors associated with in-hospital death among patients with systemic sclerosis.

OBJECTIVE: To examine demographic and clinical predictors of in-hospital death of patients with systemic sclerosis (SSc) and determine to what extent apparent racial differences may be attributed to socioeconomic factors. METHODS: Data were obtained on all hospitalizations in South Carolina for patients who were ever hospitalized between 1996 and 2000 with a diagnosis of SSc. Multiple logistic regression was used to examine predictors of in-hospital death among whites, blacks, and other patients. RESULTS: Proportions of in-hospital deaths among blacks (23.0%) and others (27.7%) were higher than among whites (15.6%), a finding that remained after adjustment for other sociodemographic and clinical factors (black/white odds ratio: 1.70, 95% confidence interval: 1.01-2.86; other/white OR 2.06, 95% CI 1.04-4.09). Other factors associated with in-hospital death included transfer status, emergency admission, length of stay, number of hospitalizations during the time period, and presence of congestive heart failure (OR 1.79; 95% CI 1.06-3.03) or hypertension (OR 0.41; 95% CI 0.23-0.71). CONCLUSION: Black and other non-white patients with SSc appear to experience an elevated risk of death during their hospital stays. Further research is necessary to understand the reasons for these disparities.