Successful rituximab therapy in adult-onset IgA vasculitis with diffuse alveolar hemorrhage and renal failure: a case report.

Background: Immunoglobulin A vasculitis (IgAV) is a rare condition that most commonly presents during childhood. Patients with adult-onset IgAV are more likely to exhibit severe symptoms at presentation with worse renal outcomes. Pulmonary manifestations of adult-onset IgAV have been described rarely in the literature and often indicate higher morbidity and mortality. Given the rarity of alveolar hemorrhage in IgAV, the literature describing the clinical entity and offering management recommendations is insufficient. Case Description: We describe a patient with known adult-onset IgAV who presented with one month of abdominal pain, bloody stools, new skin lesions, and progressive shortness of breath. She developed rapidly worsening hypoxic respiratory failure associated with a hemoglobin drop and diffuse pulmonary infiltrates on imaging. Bronchoscopy demonstrated progressively hemorrhagic effluent on bronchoalveolar lavage (BAL) consistent with a diagnosis of diffuse alveolar hemorrhage (DAH). She developed acute renal failure requiring the initiation of emergent renal replacement therapy. Given concomitant DAH and acute renal failure, methylprednisolone and rituximab (RTX) therapy were initiated. With this treatment regimen, she exhibited marked improvement in respiratory function and complete renal recovery. Conclusions: This case highlights the importance of considering DAH as a rare and life-threatening pulmonary manifestation of adult-onset IgAV. Our case demonstrates the novel and successful use of RTX in combination with steroids to treat a patient with adult-onset IgAV presenting with concomitant DAH and renal failure.

Azathioprine-induced hypersensitivity reaction mimicking sepsis in a patient with systemic lupus erythematosus.

A 21-year-old woman with a history of systemic lupus erythematosus presented to the emergency department with acute-onset nausea, vomiting, and fevers. Two weeks prior, she was started on azathioprine 50 mg daily by her outpatient rheumatologist; the dose was up-titrated to 100 mg when repeat blood work showed no drug toxicity. The morning after increasing her dose, she was awoken by recurrent emesis. At presentation, she was febrile, tachycardic, and hypotensive. Her exam showed mild, generalised abdominal tenderness but was otherwise unremarkable. Lab work demonstrated elevated inflammatory markers, elevated liver transaminases, and stable hypocomplementemia. Chest X-ray and computed tomography abdomen/pelvis were unrevealing. She was given intravenous fluids and broad-spectrum antibiotics, and azathioprine was held. A thorough infectious workup returned negative. A flare of her systemic lupus erythematosus was considered but deemed an unlikely explanation of her systemic inflammatory response syndrome. With azathioprine discontinuation, she made a rapid, near-complete recovery within 24 h of admission, suggesting a diagnosis of azathioprine hypersensitivity syndrome. This case exemplifies the difficulty in distinguishing azathioprine hypersensitivity from mimickers such as infection and underlying autoimmune disease flare. Prompt recognition of hypersensitivity can lead to appropriate discontinuation of the drug and prevent future morbidity.

The Fuel Gauge: A Simple Tool for Assessing General Surgery Resident Well-Being.

OBJECTIVE: Resident well-being is an increasingly relevant issue in medical education; however, there is no consensus on how to best measure well-being. The "fuel gauge," is a simple, easy-to-use tool developed to measure resident well-being and previously applied in an Internal Medicine Residency Program at our institution. The current study sought to evaluate its acceptability and usefulness in a surgery program. DESIGN: Weekly fuel gauge data was retrospectively collected from August 2017 through December 2018 along with resident Postgraduate Year designations. SETTING: This study was conducted at a single, large general surgery residency program that rotates through a variety of hospitals, including a University hospital, a large county hospital, a Veterans Affairs hospital, and a freestanding Children's hospital. PARTICIPANTS: Categorical general surgery residents at every level of training as well as preliminary interns and off service intern rotators from urology, oral and maxillofacial surgery, and otolaryngology were eligible for the study. Fuel gauge submissions which did not denote a score were excluded from analysis. RESULTS: Out of 130 residents, 103 (79.2%) completed at least 1 fuel gauge assessment with a weekly mean response rate of 41.5%. Low scores were submitted by 39.8% of resident participants. Narrative feedback was provided in 6.2% of submissions with increased length associated with decreased fuel gauge score. CONCLUSIONS: The fuel gauge was well accepted by a large general surgery program with no decline in participation rates over the study period. The tool provided residents with a direct line of communication with their program's administration, and a feasible way for the program director's office to monitor and identify residents who were struggling with regard to their well-being.

2019 Update of the American College of Rheumatology Recommended Rheumatoid Arthritis Disease Activity Measures.

OBJECTIVE: To provide updated American College of Rheumatology (ACR) recommendations on rheumatoid arthritis (RA) disease activity measurements to facilitate a treat-to-target approach in routine clinical care. METHODS: A working group conducted a systematic literature review from the time of the prior ACR recommendations literature search. Properties of disease activity measures were abstracted, and study quality was assessed using the Consensus-Based Standards for the selection of Health Measurement Instruments 4-point scoring method, allowing for overall level of evidence assessment. Measures that fulfilled a minimum standard were identified, and through a modified Delphi process preferred measures were selected for regular use in most clinic settings. RESULTS: The search identified 5,199 articles, of which 110 were included in the review. This search identified 46 RA disease activity measures that contained patient, provider, laboratory, and/or imaging data. Descriptions of the measures, properties, study quality, level of evidence, and feasibility were abstracted and scored. Following a modified Delphi process, 11 measures fulfilled a minimum standard for regular use in most clinic settings, and 5 measures were recommended: the Disease Activity Score in 28 Joints with Erythrocyte Sedimentation Rate or C-Reactive Protein Level, Clinical Disease Activity Index, Simplified Disease Activity Index, Routine Assessment of Patient Index Data 3, and Patient Activity Scale-II. CONCLUSION: We have updated prior ACR recommendations for preferred RA disease activity measures, identifying 11 measures that met a minimum standard for regular use and 5 measures that were preferred for regular use in most clinic settings.

Effects of gender in resident evaluations and certifying examination pass rates.

BACKGROUND: Though the proportion of female Internal Medicine (IM) residents and faculty has increased, there is minimal large scale modern data comparing resident performance by gender. This study sought to examine the effects of resident and faculty gender on resident evaluations. METHODS: Retrospective observational study over 5 years in a single IM program. IM certifying examination pass rates were obtained from the American Board of IM. RESULTS: Four hundred eighty-eight residents (195 women, 293 men), evaluated by 430 attending physicians (163 women, 270 men) were included. Twelve thousand six hundred eighty-one evaluations between 2007 and 2012 were analyzed. Female residents scored higher in two domains (Medical Interviewing, and Interpersonal and Communication Skills) (p < 0.01 for each), with no significant difference between genders for the other domains (Medical Knowledge, Overall Patient Care, Physical Examination, Procedural Skills, Professionalism, Practice Based Learning and Improvement, System Based Practices and Overall score). There were no differences in scoring between female and male attending physicians. There were no differences in certifying examination scores between women and men among graduating residents. National pass rates for women were not statistically different to pass rates for men from 1987 to 2015. CONCLUSIONS: Data from one large academic medical center demonstrate higher ratings for female residents on performance domains reflecting bedside care and interpersonal skills, with similar scores for medical knowledge and remaining domains. No significant difference was seen locally in certifying examination scores, nor in recent national pass rates, an objective measure of medical knowledge. Despite imbalanced female representation in areas of medicine, our data suggest that gender-based disparities in Internal Medicine resident medical knowledge and physician competency are no longer present.

Fatal case of macrophage activation syndrome (MAS) in a patient with dermatomyositis and cytomegalovirus (CMV) viraemia.

We describe a case of an adult with dermatomyositis (DM) who presents with a rash, high fevers, tachycardia and hypotension, initially concerning for an infectious aetiology or a DM flare. She was found to have cytomegalovirus viraemia which improved after starting valganciclovir. After extensive workup and lack of improvement with broad-spectrum antimicrobial therapy, intravenous immunoglobulin and steroids, the patient was diagnosed with macrophage activation syndrome after bone marrow biopsy and levels of soluble CD25 (soluble interleukin (IL)-2 receptor) and IL2 were obtained. Unfortunately, despite therapy with dexamethasone, anakinra and etoposide, the patient decompensated and the patient's family opted for comfort care. The patient subsequently expired in the intensive care unit.

Resident Fuel Levels: Reframing, Assessing, and Addressing Well-Being.

BACKGROUND: To optimize resident learning, programs need to readily assess resident well-being. There is a lack of easy-to-use, acceptable instruments for this task. OBJECTIVE: We created a well-being "fuel gauge," and assessed the acceptability and feasibility of this weekly electronic communication pipeline for residents to report and discuss their well-being. METHODS: A well-being fuel gauge assessment was administered weekly over the course of 1 academic year (July 2016 to June 2017) in a large internal medicine residency program. The well-being gauge asked residents to report their fuel levels using a 1 to 5 Likert-type scale (1, empty; 3, half tank; and 5, full tank). Residents who provided low scores (1 or 2) were contacted by program leadership, and the program director sent weekly e-mail updates that addressed residents' comments on their well-being fuel gauge. RESULTS: Of 163 residents, 149 (91%) provided data on their well-being fuel gauge, with a 53% average weekly response rate. Fifty-four percent of residents (80 of 149) reported a low score over the course of the year, and 4 residents only used the assessment to report a low score. Comments on average consisted of 280 characters (SD = 357) and were lengthier and more prevalent with lower fuel gauge scores. We analyzed the relationship between scores and comments. CONCLUSIONS: The well-being fuel gauge was well accepted by most residents and was easy to administer and to oversee by program directors. It facilitated ongoing monitoring of well-being and follow-up to address factors contributing to low well-being.

Core Curriculum to Facilitate the Expansion of a Rheumatology Practice to Include Nurse Practitioners and Physician Assistants.

OBJECTIVE: Due to an aging population, increasing prevalence of rheumatic disease, and a growing supply and demand gap of rheumatology providers, innovative solutions are needed to meet the needs of persons with rheumatic conditions. Nurse practitioners (NPs) and physician assistants (PAs) have been identified as a group of health professionals who could help address the workforce shortage. The Executive Committee of the Association of Rheumatology Health Professionals (ARHP), a division of the American College of Rheumatology (ACR), charged a task force to facilitate the preparation of NPs/PAs to work in a rheumatology practice setting. METHODS: The task force, consisting of private practice and academic rheumatologists, and NPs and PAs, from both adult and pediatric settings, conducted a needs assessment survey of current NPs and PAs to identify mechanisms for acquiring rheumatology knowledge. Through face-to-face and webinar meetings, and incorporating stakeholder feedback, the task force designed a rheumatology curriculum outline to enrich the training of new NPs and PAs joining rheumatology practice. RESULTS: Informed by the needs assessment data and stakeholders, an NP/PA rheumatology curriculum outline was developed and endorsed by the ACR Board of Directors for use by community-based and academic rheumatology practices, whether pediatric or adult, who desire to add NPs and PAs to their practice setting. CONCLUSION: As rheumatology is facing workforce shortages, the ACR/ARHP rheumatology curriculum outline can be utilized to train NPs and PAs and create more efficient integration of NPs and PAs into rheumatology practice.

Overview of the American College of Rheumatology's Electronic Health Record-Enabled Registry: The Rheumatology Informatics System for Effectiveness.

The Rheumatology Informatics System for Effectiveness (RISE) Registry was developed by the American College of Rheumatology (ACR) to serve US rheumatologists for the significant challenges of a rapidly changing healthcare environment. More than 400 rheumatologists have sent data from more than 3 million encounters of more than 650,000 patients as of August 11, 2016, through their electronic medical records (EMRs), with no additional work or interference with workflow on the part of the rheumatologists. RISE includes patients with all diagnoses seen by participating rheumatologists, at no cost to the rheumatologist.

Rheumatology Informatics System for Effectiveness: A National Informatics-Enabled Registry for Quality Improvement.

OBJECTIVE: The Rheumatology Informatics System for Effectiveness (RISE) is a national electronic health record (EHR)-enabled registry. RISE passively collects data from EHRs of participating practices, provides advanced quality measurement and data analytic capacities, and fulfills national quality reporting requirements. Here we report the registry's architecture and initial data, and we demonstrate how RISE is being used to improve the quality of care. METHODS: RISE is a certified Centers for Medicare and Medicaid Services Qualified Clinical Data Registry, allowing collection of data without individual patient informed consent. We analyzed data between October 1, 2014 and September 30, 2015 to characterize initial practices and patients captured in RISE. We also analyzed medication use among rheumatoid arthritis (RA) patients and performance on several quality measures. RESULTS: Across 55 sites, 312 clinicians contributed data to RISE; 72% were in group practice, 21% in solo practice, and 7% were part of a larger health system. Sites contributed data on 239,302 individuals. Among the subset with RA, 34.4% of patients were taking a biologic or targeted synthetic disease-modifying antirheumatic drug (DMARD) at their last encounter, and 66.7% were receiving a nonbiologic DMARD. Examples of quality measures include that 55.2% had a disease activity score recorded, 53.6% a functional status score, and 91.0% were taking a DMARD in the last year. CONCLUSION: RISE provides critical infrastructure for improving the quality of care in rheumatology and is a unique data source to generate new knowledge. Data validation and mapping are ongoing and RISE is available to the research and clinical communities to advance rheumatology.

Outcome assessments in rheumatoid arthritis.

Increasing evidence suggests low disease activity or remission is achievable in rheumatoid arthritis (RA). Using a treat to target strategy (T2T) has been shown to achieve these targets of remission or low disease activity in RA. In order to successfully treat to target, rheumatologists need reliable measures of disease activity to switch and/or escalate therapy to achieve or maintain therapeutic targets. Multiple disease-activity measures have been developed for both research and clinical practice. For clinical practice, the American College of Rheumatology (ACR) has recommended the PAS, PAS II, RAPID 3, CDAI, DAS 28, and SDAI for measuring disease activity in rheumatoid arthritis. Each of these measures has strengths and limitations, but they all accurately reflect disease activity, discriminate well between disease states, and are feasible to perform in the clinical setting. Implementation in the clinical setting can be optimized through leveraging technology and systems redesign. Tools such as web-based and smartphone applications have been developed to increase the ease with which these measures can be deployed. Disease-activity measurement in rheumatoid arthritis is included in the rheumatoid arthritis quality measures group in the Centers for Medicare and Medicaid Services' incentive-based Physician Quality Reporting System.

Rheumatoid arthritis disease activity measures: American College of Rheumatology recommendations for use in clinical practice.

OBJECTIVE: Although the systematic measurement of disease activity facilitates clinical decision making in rheumatoid arthritis (RA), no recommendations currently exist on which measures should be applied in clinical practice in the US. The American College of Rheumatology (ACR) convened a Working Group (WG) to comprehensively evaluate the validity, feasibility, and acceptability of available RA disease activity measures and derive recommendations for their use in clinical practice. METHODS: The Rheumatoid Arthritis Clinical Disease Activity Measures Working Group conducted a systematic review of the literature to identify RA disease activity measures. Using exclusion criteria, input from an Expert Advisory Panel (EAP), and psychometric analysis, a list of potential measures was created. A survey was administered to rheumatologists soliciting input. The WG used these survey results in conjunction with the psychometric analyses to derive final recommendations. RESULTS: Systematic review of the literature resulted in identification of 63 RA disease activity measures. Application of exclusion criteria and ratings by the EAP narrowed the list to 14 measures for further evaluation. Practicing rheumatologists rated 9 of these 14 measures as most useful and feasible. From these 9 measures, the WG selected 6 with the best psychometric properties for inclusion in the final set of ACR-recommended RA disease activity measures. CONCLUSION: We recommend the Clinical Disease Activity Index, Disease Activity Score with 28-joint counts (erythrocyte sedimentation rate or C-reactive protein), Patient Activity Scale (PAS), PAS-II, Routine Assessment of Patient Index Data with 3 measures, and Simplified Disease Activity Index because they are accurate reflections of disease activity; are sensitive to change; discriminate well between low, moderate, and high disease activity states; have remission criteria; and are feasible to perform in clinical settings.

Association between biologic therapies for chronic plaque psoriasis and cardiovascular events: a meta-analysis of randomized controlled trials.

CONTEXT: Ustekinumab and briakinumab, monoclonal antibodies to the shared p40 subunit of interleukin (IL)-12 and IL-23, have shown efficacy in treating chronic plaque psoriasis (CPP). Preliminary reports of major adverse cardiovascular events (MACEs) in psoriasis patients receiving anti-IL-12/23 agents have prompted concern. OBJECTIVE: To evaluate a possible association between biologic therapies for CPP and MACEs via meta-analysis. DATA SOURCES: Randomized controlled trials (RCTs) of anti-IL-12/23 (ustekinumab and briakinumab) agents and anti-tumor necrosis factor α (TNF-α) agents (adalimumab, etanercept, and infliximab) used in treating CPP were reviewed using the Cochrane Central Register of Controlled Trials, ClinicalTrials.gov, and Ovid MEDLINE from database inception to May 2011. The results of registered nonpublished completed studies were procured through abstract publications or poster presentations. STUDY SELECTION: Randomized, placebo-controlled, double-blind, monotherapy studies (with safety outcome data for MACE) of IL-12/23 antibodies and anti-TNF-α agents in adults. Studies of psoriatic arthritis were excluded. DATA EXTRACTION: Two investigators independently searched data while 6 investigators reviewed the abstracted data. RESULTS: A total of 22 RCTs comprising 10 183 patients met the predefined inclusion criteria. The primary outcome measure was MACE, a composite end point of myocardial infarction, cerebrovascular accident, or cardiovascular death during the placebo-controlled phase of treatment in patients receiving at least 1 dose of study agent or placebo. Absolute risk differences were used as an effect measure. There was no evidence of statistical heterogeneity across the studies using the I(2) statistic (I(2) = 0), allowing for combination of trial results using the Mantel-Haenszel fixed-effects method. During the placebo-controlled phases of the anti-IL-12/23 studies, 10 of 3179 patients receiving anti-IL-12/23 therapies experienced MACEs compared with zero events in 1474 patients receiving placebo (Mantel-Haenszel risk difference, 0.012 events/person-year; 95% confidence interval [CI], -0.001 to 0.026; P =.12). In the anti-TNF-α trials, only 1 of 3858 patients receiving anti-TNF-α agents experienced a MACE compared with 1 of 1812 patients receiving placebo (Mantel-Haenszel risk difference, -0.0005 events/person-year; 95% CI, -0.010 to 0.009; P = .94). CONCLUSIONS: Compared with placebo, there was no significant difference in the rate of MACEs observed in patients receiving anti-IL-12/IL-23 antibodies or anti-TNF-α treatments. This study may have been underpowered to identify a significant difference.

The risk of acute myocardial infarction with etodolac is not increased compared to naproxen: a historical cohort analysis of a generic COX-2 selective inhibitor.

BACKGROUND: This study compares the risk of acute myocardial infarction among patients exposed to etodolac, naproxen, celecoxib, and rofecoxib. METHODS: A retrospective cohort study in 38 258 veteran patients (26 376 patient-years) measured the adjusted odds ratios of acute myocardial infarction during exposure to etodolac, naproxen, celecoxib, or rofecoxib. RESULTS: Diagnosis of acute myocardial infarction was confirmed in 100 patients who were exposed to a study nonsteroidal anti-inflammatory drug. Compared to naproxen, the increased risk of acute myocardial infarction was not significant for etodolac (OR = 1.32, P = .27), whereas celecoxib (OR = 2.18, 95% CI 1.09-4.35, P = .03) and rofecoxib (OR = 2.16, 95 CI 1.04-4.46, P = .04) were significant. A post hoc analysis indicates that patients with a prior history of acute myocardial infarction had a significant, 4.26-fold risk for another acute myocardial infarction if taking celecoxib or rofecoxib. CONCLUSION: Etodolac is not associated with a statistically increased risk of acute myocardial infarction compared to naproxen.