Stevens-Johnson Syndrome (SJS) As the Initial Presentation in a Patient With a New Diagnosis of HIV.

A woman in her 40s presented to the emergency department with a diffuse rash consistent with Stevens-Johnson syndrome (SJS). There was no identifiable inciting factor. However, she was newly diagnosed with human immunodeficiency virus (HIV) during that same hospital admission. The leading theory for why she developed SJS given her lack of classic precipitating factors is an immune dysregulation as a result of HIV. Most cases of SJS/toxic epidermal necrolysis (TEN) in patients with HIV are related to highly active antiretroviral therapy and prophylaxis with trimethoprim-sulfamethoxazole. There is a lack of literature regarding SJS as the initial presentation of HIV without known underlying etiology or inciting factors.

Sexual Harassment Experiences Across the Academic Medicine Hierarchy.

Background Current estimates of sexual harassment across the academic hierarchy are subject to recall bias and have limited comparability between studies due to inconsistent time frames queried for each stage of training. No studies have surveyed medical students, residents/fellows, and faculty collectively and many studies exclude a wide range of sexual harassment behaviors. We assessed the incidence of sexual harassment across the different stages of academic medicine over the same time frame and within the same institutional culture. Methodology Medical students, residents/fellows, and faculty at the same academic medical campus completed a prospective online study of sexual harassment experiences in 2018. We used a tool that comprehensively assessed sexual harassment behaviors and asked about the perpetrators. Pearson's chi-square and Fisher's exact tests (for cell counts <5) were used to compare responses by academic status and gender. Participants were also asked to suggest ways to improve knowledge about university/hospital policies, support services, and reporting process on sexual harassment. Results One-third of 515 respondents (18% of invitations) reported experiencing sexual harassment in 2018. Overall, 52% of medical students, 31% of residents/fellows, and 25% of faculty respondents experienced sexual harassment. Of these, 46% of women and 19% of men reported sexual harassment experiences. The most common experiences across all levels of academic hierarchy were offensive and sexually suggestive comments or jokes and offensive and intrusive questions about one's private life or physical appearance. The most common perpetrators were "student, intern, resident, or fellow," followed by "patient or patient's family member." To improve knowledge about the policies and services regarding sexual harassment, participants suggested facilitating easy access to resources, increasing awareness, assuring confidentiality, protecting against retaliation, and continued education and reminders about the topic. Conclusions Sexual harassment may be more prevalent than the literature suggests and incidence tends to decrease with increasing academic hierarchy. Harassment can often be subtle and can pass under the radar.

The Mismatch of Nutrition and Lifestyle Beliefs and Actions Among Physicians: A Wake-Up Call.

Background. There is a notable lack of education on nutrition and physical activity guidelines in medical schools and postgraduate training. The purpose of this study is to assess the nutrition and exercise knowledge and personal health behaviors of physicians in the Department of Medicine at a large academic center. Methods. We conducted a survey study in the Department of Medicine at the University of Florida in 2018. The survey instrument included questions on demographics, medical comorbidities, baseline perception of health and fitness, and knowledge of nutrition concepts. The Duke Activity Status Index assessed activity/functional capacity and the validated 14-point Mediterranean Diet Survey evaluated dietary preferences. Data were analyzed using descriptive statistics and the χ2 test was used to perform comparisons between groups. Statistical significance was determined at P < .05. Results. Out of 331 eligible physicians, 303 (92%) participated in the study. While all respondents agreed that eating well is important for health, less than a fourth followed facets of a plant-based Mediterranean diet. Only 25% correctly identified the American Heart Association recommended number of fruit and vegetable servings per day and fewer still (20%) were aware of the recommended daily added sugar limit for adults. Forty-six percent knew the American Heart Association physical activity recommendations and 52% reported more than 3 hours of personal weekly exercise. Reported fruit and vegetable consumption correlated with perceived level of importance of nutrition as well as nutrition knowledge. Forty percent of physicians (102/253) who considered nutrition at least somewhat important reported a minimum of 2 vegetable and 3 fruit servings per day, compared with 7% (3/44) of those who considered nutrition less important ("neutral," "not important," or "important, but I don't have the time to focus on it right now"; P < .0001). Conclusions. This study highlights the need for significant improvement in education of physicians about nutrition and physical activity and need for physicians to focus on good personal health behaviors, which may potentially improve with better education.

An Imposter Twice Over: A Case of IgG4-Related Disease.

IgG4-RD has the ability to mimic other pathologic conditions, requiring that health care professionals have a high index of suspicion to make a proper diagnosis.

Citrulline dependence of anti-cyclic citrullinated peptide antibodies in systemic lupus erythematosus as a marker of deforming/erosive arthritis.

OBJECTIVE: Anti-cyclic citrullinated peptide (CCP) antibodies are a serological marker for rheumatoid arthritis (RA); up to 10%-15% of patients with systemic lupus erythematosus (SLE) are also positive. While anti-CCP in RA is citrulline-dependent, anti-CCP in some other diseases is citrulline-independent and reacts with both CCP and the unmodified (arginine-containing) cyclic arginine peptide (CAP). We investigated the citrulline dependence of anti-CCP and its significance in the arthritis of SLE. METHODS: IgG anti-CCP was compared by ELISA to anti-CAP in sera from patients with SLE (n = 335) and RA (n = 47) and healthy controls (n = 35). SLE patients were divided into 5 groups based on their joint involvement: subset I: deforming/erosive arthritis (n = 20); II: arthritis fulfilling (or likely fulfilling) American College of Rheumatology criteria for RA but without erosions (n = 18); III: joint swelling but not fulfilling RA criteria (n = 39); IV: arthritis without documented joint swelling (n = 194); and V: no arthritis (n = 58). RESULTS: Anti-CCP (> 1.7 units) was found in 68% (32/47) of patients with RA and 17% (55/329) of those with SLE. It was more common in SLE patients with deforming/erosive arthritis (38%). High anti-CCP (> 10 units) was found in RA (26%) and deforming/erosive SLE (12%). High anti-CCP/CAP ratios (> 2, indicating a selectivity to CCP) were found in 91% of anti-CCP-positive RA and 50% of anti-CCP-positive SLE patients with deforming/erosive arthritis. Patients from subset II did not have high anti-CCP/CAP. CONCLUSION: Citrulline dependence or high levels (> 10) of anti-CCP were common in SLE patients with deforming/erosive arthritis, while most anti-CCP in SLE patients was citrulline-independent. This may be useful in identifying a subset of SLE patients with high risk for development of deforming/erosive arthritis.

Autoantibodies to RNA helicase A: a new serologic marker of early lupus.

OBJECTIVE: To investigate the clinical and immunologic significance of autoantibodies to RNA helicase A (RHA) in patients with systemic rheumatic diseases. METHODS: The study group comprised 1,119 individuals enrolled in the University of Florida Center for Autoimmune Diseases registry from 2000 to 2005. Diagnoses were based on standard criteria. Autoantibodies were analyzed by immunoprecipitation and Western blot assays. RESULTS: Anti-RHA was observed in 17 (6.2%) of 276 patients with systemic lupus erythematosus (SLE), 2 patients with antiphospholipid antibodies, and 3 other patients, but anti-RHA was not observed in any patient with polymyositis/dermatomyositis, systemic sclerosis, rheumatoid arthritis, or Sjögren's syndrome. Anti-RHA was present in only 2.9% of African American patients, compared with 6.0% of white patients and 12-25% of patients of other races; this was in striking contrast to the frequency of anti-Sm in African American patients (27.2%). Among patients with SLE, anti-RHA was common in young patients (26% of those whose initial visit was at an age younger than 20 years versus 3-4% of those who were initially seen at ages 20-49 years) and at an early stage of disease (23% of those whose first clinic visit was within 1 year of disease onset versus 2-8% of those whose first visit was at least 1 year after disease onset). In 9 of 11 patients, levels of anti-RHA decreased to <10% of the initial value within 9-37 months, while levels of coexisting anti-Ro or anti-Su remained the same. New specificities developed in 2 patients (anti-nuclear RNP and anti-Sm, and anti-ribosomal P, respectively). These data suggest that the level of anti-RHA diminishes over time, and that anti-RHA is regulated via a mechanism different from that for other lupus-related autoantibodies. CONCLUSION: Anti-RHA is a new serologic marker for SLE. It is produced mainly in young non-African Americans at an early stage of their disease. Anti-RHA has a unique tendency to diminish over time. The production of anti-RHA may depend on a process restricted to early SLE, or it may be highly sensitive to treatment.

Activity-dependent change in morphology of the glial tubular lattice of the crayfish medial giant nerve fiber.

An evaluation of electron micrographs of stimulated nerve fibers used to investigate the effect of action potential generation on the structure-function relationship between axons and its associated glial cells revealed that what was at first thought to be stimulation-induced damage to the glia was, in fact, limited to volume expansion and disaggregation of the glial tubular lattice. All other structures appeared well preserved and otherwise normal. Using a 4-point subjective scale for evaluation by two investigators, 50-Hz stimulation for 2 min was observed to cause a volume expansion and disaggregation of the tubular lattice. Quantitatively, the internal diameter of the stimulated tubular lattice increased 65% above the unstimulated control (50.96 +/- 2.09 nm and 30.81 +/- 0.87 nm, respectively, P < or = 0.001). Stimulation had its greatest effect on tubular lattice volume and organization in the adaxonal glial layer and a decreasing effect as distance from the giant axon increased. These effects are reversible since the tubular lattice diameter and degree of disaggregation preserved 10 min after the cessation of stimulation were not found to be different from their unstimulated paired controls. Axons injected with TEA, a voltage-gated potassium channel blocker, prevented stimulation-induced volume expansion and disaggregation of tubular lattice structure. These results are consistent with an active uptake of K+ with obligated water or, alternatively, hyperosmotic K+ uptake and a fixation-induced increase in water permeation. Either mechanism of K+ uptake would result in tubular lattice volume expansion and disaggregation and suggests that the tubular lattice serves a larger role than a simple trans-glial diffusion pathway.

Diagnostic accuracy for lupus and other systemic autoimmune diseases in the community setting.

BACKGROUND: Most individuals with autoimmune and other immune disorders undergo initial evaluation in the community setting. Since misdiagnosis of systemic autoimmune diseases can have serious consequences, we evaluated community physicians' accuracy in diagnosing autoimmune diseases and the consequences of misdiagnosis. METHODS: We studied the patients referred to our Autoimmune Disease Center for 13 months (n = 476). We estimated the degree of agreement with the final diagnosis (kappa statistic) and the accuracy indexes (sensitivity, specificity, and predictive values) of the referring physicians' diagnoses. RESULTS: We found a 49% agreement between the referring and final diagnoses (kappa = 0.36). Of 263 patients referred with a presumptive diagnosis of systemic lupus erythematosus (SLE), 125 received a diagnosis of other conditions (kappa = 0.34). Of those referred with SLE, 76 (29%) were seropositive for antinuclear antibodies but did not have autoimmune disease. The degree of agreement for referring rheumatologists (kappa = 0.55) was better than that for nonrheumatologists (kappa = 0.32). Stepwise logistic regression indicated that rheumatologists were 4 times more likely to make an accurate diagnosis of SLE than were nonrheumatologists (P<.003). Thirty-nine patients who were seropositive for antinuclear antibodies but had no autoimmune disease had been treated with corticosteroids at dosages as high as 60 mg/d. CONCLUSIONS: Many patients with a positive antinuclear antibody test are incorrectly given a diagnosis of SLE and sometimes treated with toxic medications. The data support the importance of continuing medical education for community physicians in screening for autoimmune diseases and identifying patients who may benefit from early referral to a specialist.