Lynne Taylor

Reliability and Convergent Validity of Two Outcome Instruments for Pemphigus

A major obstacle in performing multicenter controlled trials for pemphigus is the lack of a validated disease activity scoring system. Here, we assess the reliability and convergent validity of the PDAI (pemphigus disease area index). A group of 10 dermatologists scored 15 patients with pemphigus to estimate the inter- and intra-rater reliability of the PDAI and the recently described ABSIS (autoimmune bullous skin disorder intensity score) instrument. To assess convergent validity, these tools were also correlated with the Physicians Global Assessment (PGA). Reliability studies demonstrated an intra-class correlation coefficient (ICC) for inter-rater reliability of 0.76 (95% confirdence interval (CI)=0.61-0.91) for the PDAI and 0.77 (0.63-0.91) for the ABSIS. The tools differed most in reliability of assessing skin activity, with an ICC of 0.39 (0.17-0.60) for the ABSIS and 0.86 (0.76-0.95) for the PDAI. Intra-rater test-retest reliability demonstrated an ICC of 0.98 (0.96-1.0) for the PDAI and 0.80 (0.65-0.96) for the ABSIS. The PDAI also correlated more closely with the PGA. We conclude that the PDAI is more reproducible and correlates better with physician impression of extent. Subset analysis suggests that for this population of mild-to-moderate disease activity, the PDAI captures more variability in cutaneous disease than the ABSIS.

Quality of life in cutaneous lupus erythematosus

BACKGROUND Little is known about quality of life in patients with cutaneous lupus erythematosus. OBJECTIVE We sought to determine how cutaneous lupus affects quality of life and which independent variables are associated with poor quality of life. METHODS A total of 157 patients with cutaneous lupus completed surveys related to quality of life, including the Skindex-29 and the Short Form-36. RESULTS Quality of life in cutaneous lupus is severely impaired, particularly with respect to emotional well-being. Patients with cutaneous lupus have worse quality of life than those with other common dermatologic conditions, such as acne, nonmelanoma skin cancer, and alopecia. With respect to mental health status, patients with cutaneous lupus have similar or worse scores than patients with hypertension, type 2 diabetes mellitus, recent myocardial infarction, and congestive heart failure. Factors related to poor quality of life include female gender, generalized disease, severe disease, distribution of lesions, and younger age. LIMITATIONS The study was done at a single referral-only center. CONCLUSION Patients with cutaneous lupus have very impaired quality of life, particularly from an emotional perspective.

Development of the CLASI as a Tool to Measure Disease Severity and Responsiveness to Therapy in Cutaneous Lupus Erythematosus

OBJECTIVE To determine how to use the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) to classify patients according to disease severity (mild, moderate, and severe) and to identify which patients respond to therapy. DESIGN Cohort. SETTING The connective-tissue disease clinic at the Hospital of the University of Pennsylvania, Philadelphia. PATIENTS Seventy-five patients with clinical or histopathologic evidence of cutaneous lupus erythematosus or systemic lupus erythematosus were included in the study. MAIN OUTCOME MEASURES The CLASI, Skindex-29, and the physicians subjective assessment of severity and improvement were completed at every visit. RESULTS Disease severity was assessed with 45 patient visits. Mild, moderate, and severe disease corresponded with CLASI activity score ranges of 0 to 9, 10 to 20, and 21 to 70, respectively. Improvement in disease activity was assessed in 74 patients. A clinical improvement was associated with a mean 3-point or 18% decrease in the CLASI activity score. However, receiver operating characteristic analysis demonstrated an increased percentage of patients correctly classified when a 4-point (sensitivity, 39%; specificity, 93%; correctly classified, 76%) or 20% (sensitivity, 46%; specificity, 78%; correctly classified, 67%) decrease in the CLASI activity score was used instead to identify improvement. CONCLUSION The CLASI can be used to classify patients into groups according to disease severity and to identify clinically significant improvements in disease activity.

Nonspecific Presentation of Pneumonia in Hospitalized Older People: Age Effect or Dementia?

OBJECTIVES: Older adults, when presenting with pneumonia, are often thought to present with nonspecific symptoms instead of more suggestive symptom(s). However, studies designed to determine whether age is associated with nonspecific presentations have yielded contradictory results. Many studies have not distinguished between the effects of preexisting cognitive impairment that results from dementia and the effects of age. The aim of this study is to determine whether there are significant differences in the presentation of pneumonia in demented versus nondemented patients across two age groups. We hypothesized that the nonspecific presentation of pneumonia in older people is due to dementia rather than to chronological age.

Interstitial Lung Disease in Classic and Skin-Predominant Dermatomyositis: A Retrospective Study With Screening Recommendations

OBJECTIVES (1) To determine the prevalence of interstitial lung disease (ILD) and isolated low diffusing capacity for carbon monoxide (DLCO) in a large cohort of outpatients with dermatomyositis. (2) To compare the pulmonary abnormalities of patients with classic dermatomyositis and those with skin-predominant dermatomyositis. DESIGN Retrospective cohort study. SETTING University hospital outpatient dermatology referral center. Patients Medical records of 91 outpatients with adult-onset dermatomyositis seen between May 26, 2006, and May 25, 2009, were reviewed. MAIN OUTCOME MEASURES Presence of ILD on thin-slice chest computed tomographic (CT) scans and DLCO. RESULTS Of the 71 patients with dermatomyositis who had CT or DLCO data, 16 (23%; 95% confidence interval [CI], 13%-33%) had ILD as defined by CT results [corrected]. All patients with ILD had a reduced DLCO, and the ILD prevalence was not different between patients with skin-predominant dermatomyositis (10 of 35 [29% ]) and those with classic dermatomyositis (6 of 36 [17% ]) (P = .27). Eighteen of 71 patients with dermatomyositis (25%; 95% CI, 15%-36%) (7 of 35 [20%] with skin-predominant dermatomyositis; 11 of 36 [31%] with classic dermatomyositis; P = .41) had a low DLCO in the absence of CT findings showing ILD. The prevalence of malignant disease was higher in patients with classic dermatomyositis than in those with skin-predominant dermatomyositis (P = .02), and no patients with skin-predominant dermatomyositis had internal malignant disease. CONCLUSIONS Radiologic ILD and isolated DLCO reductions, which may signify early ILD or pulmonary hypertension, are common in dermatology outpatients with both classic and skin-predominant dermatomyositis. Because DLCO testing is both inexpensive and sensitive for pulmonary disease, it may be appropriate to screen all patients with dermatomyositis with serial DLCO measurements and base further testing on DLCO results.

Modification of the Cutaneous Dermatomyositis Disease Area and Severity Index, an outcome instrument

Background  Validated outcome measures in dermatology help standardize and improve patient care. A scoring system of skin disease severity in dermatomyositis known as the Cutaneous Dermatomyositis Disease Area and Severity Index (CDASI) has been developed.

Extending the Pipeline for Minority Physicians: A Comprehensive Program for Minority Faculty Development.

Medical schools must become more successful in training minority faculty. Minority faculty development programs at schools of medicine must involve trainees from the undergraduate years (if not before) through junior faculty and must involve MD and combine-degree (MD-PhD) students. The authors describe the comprehensive minority faculty development program at the University of Pennsylvania School of Medicine, which involves minority undergraduates, medical students, residents, fellows, and faculty. This program provides the administrative staff and research methodologists to assist trainees at all levels across all departments in the school of medicine. The principal student recruitment program is the undergraduate premedicine enrichment program. The medical student component provides general counseling, research development, and activities to enhance performance in the clinical courses. The components for advanced trainees (residents, fellows, and postdoctoral trainees) and faculty consist of training in research methods, mentoring, teaching skills, and scientific writing skills. Through this program, the University of Pennsylvania School of Medicine has increased the number of under-represented minority faculty by 32% since 1993-94 and created an environment conducive to the professional growth and development of minority faculty.

Parent and Adolescent Knowledge of HPV and Subsequent Vaccination

OBJECTIVE: Human papillomavirus (HPV) vaccination has been shown to have important health benefits, but vaccination rates are low. Parental and adolescent knowledge could possibly promote vaccination, but the relationship between knowledge and subsequent vaccination is unclear. This study examines how strongly HPV vaccination among high-risk adolescents is related to their or their parents’ previous knowledge. METHODS: A longitudinal cohort study enrolled participants from low-income, predominantly African American neighborhoods. Baseline questionnaires measuring knowledge of HPV and HPV vaccination, as well other variables, were completed by 211 adolescents and 149 parents of another adolescent sample. Adolescent vaccination was tracked prospectively for 12 months after baseline by using clinic reporting data. Analyses tested if parent or adolescent knowledge was associated with or predictive of adolescent HPV vaccination. RESULTS: On average, parents and adolescents answered slightly less than 50% of knowledge items correctly at baseline, with 5% of parents and 10% of adolescents not answering any knowledge items correctly. Within 12 months, 20 of 149 (13.4%) of the parents’ daughters received an HPV vaccination and 32 of 211 (15.2%) of the other adolescent sample did so. Neither parental nor adolescent knowledge was associated with or predictive of adolescent vaccination. For example, when testing the relationship between adolescent vaccination and parental knowledge scores, all R2 values were <0.005. Results were independent of available potential confounders. CONCLUSIONS: Those with higher levels of knowledge were not more likely to obtain vaccination for themselves or their daughters. Ideally, future interventions will target factors related to vaccination.

Quality of life and disease severity in a cutaneous lupus erythematosus pilot study.

The results of our study were surprising in that quality of life did not easily correlate with improvement or deterioration of the disease. We found a moderate correlation (Spearman r=0.55) between the change in CLASI activity scores and the change in Skindex scores. In patients 4 and 6, with SCLE, the complete resolution of the active disease without significant scarring was associated with only minimal improvement in their Skindex scores (see table 1 for detailed results). In patient 3 there was moderate improvement of the Skindex even though the skin condition as measured by the CLASI did not significantly improve. Although there was improvement of activity in patient 2, there was increased damage, with worsening of the Skindex. The improved activity in patient 7 and 8 correlated with an improved Skindex score, despite a worsening of the damage score. Only patients 1, 5, and 6 had both the CLASI and Skindex correlate as expected with parallel improvement of both scores. This small monocentric study cannot validate or devalue the Skindex as a measure for quality of life in CLE. The correlation between what physicians and indeed patients perceive as objective improvement or deterioration of a skin condition may not correlate with the patient’s quality of life. Our observations may imply that our treatment goals should extend beyond the obvious control of the disease, which is reliably measured by the CLASI. The Quality of Life (QoL) does not uniformly improve as the activity of the disease wanes. This may mean that

Comparing GPS, Log, Survey, and Accelerometry to Measure Physical Activity.

OBJECTIVES We explored how objectively measured global positioning system (GPS) and accelerometer data match with travel logs and questionnaires in predicting trip duration and physical activity (PA). METHODS 99 participants wore GPS devices and accelerometers, and recorded all trips in a log for 5 consecutive days. Participants also completed a self-administered questionnaire on PA and travel behaviors. RESULTS There was good agreement between GPS and log for assessment of trip duration, although log measures overestimated trip duration (concordance correlation coefficient 0.53 [0.47, 0.59]; Bland-Altman estimate 0.76 [0.16, 3.71] comparing GPS to log). Log measures underestimated light PA and overestimated moderate PA compared to accelerometry when greater than zero moderate PA was reported. CONCLUSIONS It is often not feasible to deploy accelerometry or GPS devices in population research because these devices are expensive and require technical expertise and data processing. Questionnaires and logs provide inexpensive tools to assess PA and travel with reasonable concordance with objective measures. However, they have shortcomings in evaluating the presence and amount of light and moderate PA. Future questionnaires and logs should be developed to evaluate sensitivity to light and moderate PA.