Search

Objective: To provide evidence‐based recommendations and expert guidance for the management of antineutrophil cytoplasmic antibody–associated vasculitis (AAV), including granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). Methods: Clinical questions regarding the treatment and management of AAV were developed in the population, intervention, comparator, and outcome (PICO) format (47 for GPA/MPA, 34 for EGPA). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel. Results: We present 26 recommendations and 5 ungraded position statements for GPA/MPA, and 15 recommendations and 5 ungraded position statements for EGPA. This guideline provides recommendations for remission induction and maintenance therapy as well as adjunctive treatment strategies in GPA, MPA, and EGPA. These recommendations include the use of rituximab for remission induction and maintenance in severe GPA and MPA and the use of mepolizumab in nonsevere EGPA. All recommendations are conditional due in part to the lack of multiple randomized controlled trials and/or low‐quality evidence supporting the recommendations. Conclusion: This guideline presents the first recommendations endorsed by the American College of Rheumatology and the Vasculitis Foundation for the management of AAV and provides guidance to health care professionals on how to treat these diseases. [ABSTRACT FROM AUTHOR]

Objective: To provide evidence‐based recommendations and expert guidance for the management of giant cell arteritis (GCA) and Takayasu arteritis (TAK) as exemplars of large vessel vasculitis. Methods: Clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for GCA and TAK (27 for GCA, 27 for TAK). Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to rate the quality of the evidence. Recommendations were developed by the Voting Panel, comprising adult and pediatric rheumatologists and patients. Each recommendation required ≥70% consensus among the Voting Panel. Results: We present 22 recommendations and 2 ungraded position statements for GCA, and 20 recommendations and 1 ungraded position statement for TAK. These recommendations and statements address clinical questions relating to the use of diagnostic testing, including imaging, treatments, and surgical interventions in GCA and TAK. Recommendations for GCA include support for the use of glucocorticoid‐sparing immunosuppressive agents and the use of imaging to identify large vessel involvement. Recommendations for TAK include the use of nonglucocorticoid immunosuppressive agents with glucocorticoids as initial therapy. There were only 2 strong recommendations; the remaining recommendations were conditional due to the low quality of evidence available for most PICO questions. Conclusion: These recommendations provide guidance regarding the evaluation and management of patients with GCA and TAK, including diagnostic strategies, use of pharmacologic agents, and surgical interventions. [ABSTRACT FROM AUTHOR]

Background:The availability of pediatric subspecialty services is a problem evident throughout the United States. Access to pediatric gastroenterology services, especially in rural areas, can be scarce. Telemedicine has been proposed as a tool capable of decreasing healthcare costs while extending medical care. Objective:The purpose of this article is to review available literature regarding the utility of telemedicine as it applies to pediatric gastroenterology, specifically its role in eliminating healthcare disparities. Methods:Research articles were identified through a PubMed search with key words focusing on telemedicine initiatives in pediatric gastroenterology, pediatric subspecialty, rural pediatric care, and adult gastroenterology. Studies were categorized based on the following areas of application: financial, time management, communication/community, and patient health and satisfaction. Results:We reached the conclusion that evidence-supported trends in available literature provide a framework for pediatric gastroenterology telemedicine initiatives that can provide resource-sparing, community-enriching, and physician-improving services that ultimately serve to better patient health. [ABSTRACT FROM AUTHOR]

Objective: Granulomatosis with polyangiitis (Wegener's) (GPA) is a type of antineutrophil cytoplasmic antibody-associated vasculitis that often entails severe end-organ damage and treatment-related complications that frequently lead to hospitalization and death. Nationwide trends in hospitalizations and in-hospital mortality over the past 2 decades are unknown and were evaluated in this study.Methods: Using the National Inpatient Sample, the largest all-payer inpatient database in the US, trends in hospitalizations with a discharge diagnosis of GPA (formerly Wegener's granulomatosis; International Classification of Disease, Ninth Revision, Clinical Modification code 446.4) between 1993 and 2011 were studied. Analyses were performed using hospital-level sampling weights to obtain US national estimates.Results: From 1993 to 2011, the annual hospitalization rate for patients with a principal diagnosis of GPA increased by 24%, from 5.1 to 6.3 per 1 million US persons (P < 0.0001 for trend); however, in-hospital deaths in this group declined by 73%, from 9.1% to 2.5% (P < 0.0001 for trend), resulting in a 66% net reduction in the annual in-hospital mortality rate. The median length of stay declined by 20%, from 6.9 days in 1993 to 5.5 days in 2011 (P = 0.0002 for trend). Infection was the most common principal discharge diagnosis when GPA was a secondary diagnosis, including among those who died during hospitalization.Conclusion: The findings from these nationally representative, contemporary inpatient data indicate that the in-hospital mortality of GPA has declined substantially over the past 2 decades, while the overall hospitalization rate for GPA increased slightly. Infection remains a common principal hospitalization diagnosis among GPA patients, including hospitalizations resulting in mortality. [ABSTRACT FROM AUTHOR]

Objective: The morbidity and mortality of hospitalized GCA patients have been unexplored. The aim of this study was to analyse inpatient complications experienced by patients with GCA. Methods: We used the Nationwide Inpatient Sample database to study a large group of patients admitted for pneumonia, myocardial infarction (MI), ischaemic stroke and femoral neck fracture. Patients were divided into two groups based on whether or not they had a diagnosis of GCA upon admission. Outcomes evaluated included inpatient mortality and the occurrence of adrenal insufficiency, deep vein thrombosis, pulmonary embolism and delirium. Results: From 2008 to 2011, 8203447 patients ≥50 years of age were discharged from US hospitals after admission with pneumonia, MI, stroke and femoral neck fracture. Among these patients, 9311 (0.11%) had GCA. Admissions for pneumonia, stroke and hip fracture were more frequent in GCA patients compared with those without GCA, accounting for 41.5% vs 39.4%, 24.9% vs 19.8% and 15.4% vs 14.2% of hospitalizations, respectively (P ≤ 0.001). Admissions for MI were more common in non-GCA patients (26.6% vs 18.2%, P< 0.001). During hospitalization, 4.1% of the GCA patients died, compared with 4.8% of those without GCA [odds ratio (OR) 0.73, P< 0.001). The GCA population suffered significantly more often from deep vein thrombosis (OR 2.08, P< 0.001), pulmonary embolism (OR 1.58, P< 0.001), delirium (OR 1.60, P< 0.001) and adrenal insufficiency (OR 4.95, P< 0.001). Conclusion: Hospitalized GCA patients have lower mortality compared with the general inpatient population but greater risk of venous thromboembolism, delirium and adrenal insufficiency. [ABSTRACT FROM AUTHOR]

Abstract: Long-term retreat rates of Puget Sound''s unconsolidated sediment shorelines have been difficult to quantify, and little systematic research has been completed to constrain retreat in this area. We put forward a new application of cosmogenic 10Be exposure dating to assess long-term shoreline retreat on Whidbey Island, WA by dating lag boulders exposed on the shore platform as the shoreline erodes. Production of 10Be in shoreline boulders is modulated by both tidal submergence and topographic shielding from the retreating bluff. By modeling the combined effect of these variables on 10Be production, the timing of exposure can be determined and used to calculate long-term (103–104 yr) bluff retreat rates. In rare cases, retreat rates are underestimated due to inherited 10Be. Within the study area, average retreat rates ranged between 0 and 8cmyr−1. Our results demonstrate the utility of cosmogenic nuclides for determining long-term shoreline retreat rates in areas with thick sediment cover, where large numbers of samples can be collected, and where the pre-depositional history of the boulders is uncomplicated. [Copyright &y& Elsevier]

The notion of “counterinsurgency” (COIN) has for some years been the central concept driving military operations in Afghanistan, and before that, in Iraq. It constitutes the dominant idea influencing much current military planning of the major Western powers. This study questions the assumptions and relevance of the thinking behind counterinsurgency doctrine. It suggests that the ultimate effect of its dominance is to reduce the highly contingent nature of war to a list of techniques, the application of which are regarded as a sufficient precondition whenever states deem that they are confronted by conflicts that can be described as an “insurgency.” Such assumptions are both arbitrary and risk crowding out necessary, although by their nature very difficult, political judgments that are required for the effective construction and implementation of strategies that seek to ensure that the ends sought are proportional to the means employed. [ABSTRACT FROM PUBLISHER]

Objective To identify biomarkers that distinguish between active antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and remission in a manner superior or complementary to established markers of systemic inflammation. Methods Markers of vascular injury and angiogenesis were measured before and after treatment in a large clinical trial in AAV: 163 subjects enrolled in the Rituximab in ANCA-Associated Vasculitis trial were screened for the present study. Serum levels of E-selectin, intercellular adhesion molecule 3 matrix metalloproteinase protein 1 (MMP-1), MMP-3, MMP-9, P-selectin, thrombomodulin, and vascular endothelial growth factor were measured at study screening (time of active disease) and at month 6. Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels had been measured at the time of the clinical visit. The primary outcome measure was the difference in marker level between screening and month 6 among patients whose disease was in remission (Birmingham Vasculitis Activity Score for Wegener's granulomatosis [BVAS/WG] score of 0) at month 6. Results All patients had severe active vasculitis at screening (mean ± SD BVAS/WG score 8.6 ± 3.2). Among the 123 patients whose disease was clinically in remission at month 6, levels of all markers except E-selectin showed significant declines. MMP-3 levels were also higher among the 23 patients with active disease at month 6 than among the 123 patients whose disease was in remission. MMP-3 levels correlated weakly with ESR and CRP levels. Conclusion Many markers of vascular injury and angiogenesis are elevated in severe active AAV and decline with treatment, but MMP-3 appears to distinguish active AAV from remission better than the other markers studied. Further study of MMP-3 is warranted to determine its clinical utility in combination with conventional markers of inflammation and ANCA titers. [ABSTRACT FROM AUTHOR]

The article presents a discussion of the moral obligations that physicians have to work towards the elimination of social inequities and health disparities that adversely effect public health. A discussion of the implications that the moral obligations have on medical education and on medical ethics education is presented. Research which has investigated the moral and ethical obligations of physicians is discussed.

A review of the article "Bioethicists Can and Should Contribute to Addressing Racism" by M. Danis, Y. Wilson and A. White is presented.

Small and medium sized towns often lack resources for the data collection required by many transportation planning methods. In many cases, though, data from a regional or national source may be transferred to the small town or used in the form of national averages. We focus on the case of towns on the fringe of large metropolitan areas and compare various data reuse techniques. We defined a quantitative categorization method that indicates for a given town if local data transfer or national data transfer is preferable. The analysis is based on two surveys of North Carolina metropolitan areas and their surroundings (Charlotte and Raleigh-Durham) representing 5,000 households in 90 municipalities ranging in size from a few hundred population to half a million. Our results show that planners working in FRINGE towns can defensibly use the NCHRP 365 trip rates dictated by the nearby urban center, or existing trip rates derived from surveys of the urban center itself. [ABSTRACT FROM AUTHOR]

The article discusses a study on the prevalence of and number of individuals affected by arthritis and other rheumatic conditions in the U.S. The study used the National Health Interview Survey (NHIS) for the analysis of overall arthritis. The best available prevalence estimates were applied to the corresponding 2005 U.S. population estimates from the Census Bureau to estimate the number affected with each condition. The study found that more than 21% of adults in the U.S. have self-reported doctor-diagnosed arthritis.

This article borrows from the work of Clausewitz to posit two general approaches to the pursuit of limitation in war, one being political and the other technical. The political approach seeks to control escalation via consensual arrangements with the adversary, while the technical approach seeks to forestall escalation by peremptorily destroying the enemy's military capability. It is argued that the 'Revolution in Military Affairs' (RMA) instantiates the second, technical, approach to war limitation. By exploiting advanced technology, the US armed forces hope to limit the costs of war by defeating their adversaries in a rapid, decisive manner. The problems associated with this approach are discussed in relation to the nature of the conflicts that the US is likely to face in the foreseeable future. The article concludes by suggesting that such conflicts will require that more attention be paid to the political sources of limitation in war. [ABSTRACT FROM AUTHOR]

Cosmogenic-nuclide exposure ages for 13 glacially transported boulders atop the Martha''s Vineyard moraine, MA, USA, indicate that the southeastern margin of the Laurentide ice sheet reached its maximum extent during the last glaciation 23,200±500 yr ago. Another 10 age determinations from the younger Buzzards Bay moraine near Woods Hole, MA, indicate that this moraine complex was formed 18,800±400 yr ago. These ages correlate approximately with the terminations of cooling cycles recorded in Greenland ice cores and coeval ice-rafting events, suggesting that the marginal position of this sector of the ice sheet was tightly coupled to North Atlantic climate during the last glacial maximum. [Copyright &y& Elsevier]

This paper tracks an ongoing, 15-year initiative to reform and assess a general education programme at a regional comprehensive university in the US. The paper identifies four key phases in the process, chronicling emergent challenges, describing key setbacks and highlighting noteworthy successes. The authors identify five key lessons that may prove useful to other institutions planning or currently attempting to integrate assessment into their general education programme, and conclude that designing and implementing general education assessment is more challenging than assessing other programmes in post-secondary settings because it is influenced by more internal and external constituencies than any other assessment process. [ABSTRACT FROM AUTHOR]

Congestive heart failure (CHF) is a common and increasing problem among older persons. Symptoms associated with CHF include edema, fatigue, dyspnea, limited exercise tolerance, and significant activity limitation. Using a comprehensive review of literature, this article describes the public health burden arising out of CHF in the United States. It describes CHF in terms of functional limitation and disability in the elderly following the National Center for Medical Rehabilitation and Research (NCMRR) disability model. The use of home based interventions (HBI) to assist people with CHF is discussed. [ABSTRACT FROM PUBLISHER]

Provides a review of the types of incentive programs which are implemented by school districts across the nation. Purpose of a school incentive reward program; Accrediting criteria; Analysis of information; Remediation of cited deficiencies through an analysis of underlying problems and the development and implementation of an improvement plan.

Focuses on the influence of speech courses on communication apprehension in the U.S. Reason for avoiding interpersonal communication; Behaviors associated with anomia; Approaches for the reduction of apprehension.

Discusses how Affordable Housing Programs (AHP) can help local housing authorities (LHA) in the United States increase revenues and create an alternative housing choice. Traditional role of the LHA; Goal of the AHP; Financial benefits; Implementation.

The article focuses on racial and ethnic differences as factors affecting the quality of medical care given to patients with ST-segment elevation myocardial infarction (STEMI) based on several studies in the U.S. Nonwhite patients wait longer for percutaneous coronary intervention (PCI) compared to their white counterparts. The American Heart Association (AHA) and the American College of Cardiology (ACC) set STEMI treatment to 90 minutes or less. Developing a more diverse clinical workforce may solve the problem.

Examines the 'u' curve relationship between blood pressure (BP) and cerebrovascular (CV) morbidity in hemodialysis patients in the United States. Correlation of low systolic BP with an increase in CV and non-CV mortality; Association of post-dialysis hypertension with an increase in CV mortality; Relationship between low diastolic BP and increased mortality.

Objective: To provide evidence-based recommendations and expert guidance for the management of Kawasaki disease (KD), focusing on clinical scenarios more commonly addressed by rheumatologists., Methods: Sixteen clinical questions regarding diagnostic testing, treatment, and management of KD were developed in the Patient/Population, Intervention, Comparison, and Outcomes (PICO) question format. Systematic literature reviews were conducted for each PICO question. We used the Grading of Recommendations, Assessment, Development and Evaluation method to assess the quality of evidence and formulate recommendations. Each recommendation required consensus from at least 70% of the Voting Panel., Results: We present 1 good practice statement, 11 recommendations, and 1 ungraded position statement to guide the management of KD and clinical scenarios of suspected KD. These recommendations for KD are focused on situations in which input from rheumatologists may be requested by other managing specialists, such as in cases of treatment-refractory, severe, or complicated KD. The good practice statement affirms that all patients with KD should receive initial treatment with intravenous immunoglobulin (IVIG). In addition, we developed 7 strong and 4 conditional recommendations for the management of KD or suspected KD. Strong recommendations include prompt treatment of incomplete KD, treatment with aspirin, and obtaining an echocardiogram in the setting of unexplained macrophage activation syndrome or shock. Conditional recommendations include use of IVIG with other adjuvant agents for patients with KD and high-risk features of IVIG resistance and/or coronary artery aneurysms. These recommendations endorse minimizing risk to the patient by using established therapy promptly at disease onset and identifying situations in which adjunctive therapy may be warranted., Conclusion: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and use of echocardiography in patients with suspected or confirmed KD., (© 2022 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA.)

Objective: Myocardial infarction and ischemic stroke are leading causes of cardiovascular (CV) morbidity and mortality in ANCA-associated vasculitis (AAV), especially for the 20% with end-stage renal disease (ESRD). We assessed the impact of renal transplantation on the risk of myocardial infarction and stroke among patients with ESRD due to AAV., Methods: We identified patients from the United States Renal Data System with ESRD due to AAV between 2000 and 2016. We examined the association between renal transplantation and the risk of non-fatal and fatal myocardial infarction or ischemic stroke among waitlisted patients using Medicare claims and death data through 2017. We used time-varying Cox proportional hazards models with age as the time scale to estimate hazard ratios (HR) and 95% confidence intervals (CIs) for myocardial infarction and ischemic stroke events among patients who received a renal transplant compared to those who remained on the waitlist., Results: Of 1029 waitlisted patients, 593 (58%) were transplanted over a mean of 5.7 years. There were 17 events (4.6/1,000 person-years) in the transplanted group and 40 events (13.7/1,000 person-years) in the group that remained waitlisted. A renal transplant was associated with a 78% lower risk of myocardial infarction or ischemic stroke (HR=0.22, 95% CI 0.11 to 0.47). These findings persisted across sex and age groups and when censoring patients after living donor transplantation., Conclusions: Among AAV patients with ESRD, renal transplantation can substantially reduce the risk of myocardial infarction and ischemic stroke. Improving access to transplantation for this population may further improve outcomes., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Objective: To provide evidence-based recommendations and expert guidance for the management of systemic polyarteritis nodosa (PAN)., Methods: Twenty-one clinical questions regarding diagnostic testing, treatment, and management were developed in the population, intervention, comparator, and outcome (PICO) format for systemic, non-hepatitis B-related PAN. Systematic literature reviews were conducted for each PICO question. The Grading of Recommendations Assessment, Development and Evaluation methodology was used to assess the quality of evidence and formulate recommendations. Each recommendation required ≥70% consensus among the Voting Panel., Results: We present 16 recommendations and 1 ungraded position statement for PAN. Most recommendations were graded as conditional due to the paucity of evidence. These recommendations support early treatment of severe PAN with cyclophosphamide and glucocorticoids, limiting toxicity through minimizing long-term exposure to both treatments, and the use of imaging and tissue biopsy for disease diagnosis. These recommendations endorse minimizing risk to the patient by using established therapy at disease onset and identify new areas where adjunctive therapy may be warranted., Conclusion: These recommendations provide guidance regarding diagnostic strategies, use of pharmacologic agents, and imaging for patients with PAN., (© 2021 American College of Rheumatology. This article has been contributed to by US Government employees and their work is in the public domain in the USA.)

Objective: The management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) has evolved substantially over the last 2 decades. We sought to characterize AAV treatment patterns in the United States., Methods: We identified patients with AAV in the Rheumatology Informatics System for Effectiveness (RISE) registry who had at least 2 rheumatology clinician visits between January 1, 2015, and December 31, 2017. Demographics, medications, laboratory test results, and billing codes were extracted from the medical record. Demographic and prescription trends were assessed overall and across US regions., Results: We identified 1462 patients with AAV, 259 (18%) with new or relapsing AAV. The majority were classified as having granulomatosis with polyangiitis (75%). The mean age was 59.8 years and 59% were female. The majority of patients were in the South (45%) followed by the Mid-West (32%), West (12%), and Northeast (8%). Patients had a median of 3 visits and follow-up of 579 days. The most commonly prescribed medications during the study period were glucocorticoids (86%) followed by rituximab (45%), methotrexate (33%), azathioprine (32%), and mycophenolate mofetil (18%); cyclophosphamide (CYC) was rarely used (7%). At the most recent visits in RISE, 47% of patients were on glucocorticoids. Prescription trends were similar across regions., Conclusion: To our knowledge, this is the first study to evaluate the demographics and management of AAV by rheumatologists outside of major referral centers. Management strategies vary widely, but CYC is rarely used. These observations can be used to inform future research priorities. Additional studies are needed to characterize AAV severity in RISE as well as patient and provider treatment preferences., (Copyright © 2021 by the Journal of Rheumatology.)

Objective: IgG4-related disease (IgG4-RD) can cause fibroinflammatory lesions in nearly any organ. Correlation among clinical, serologic, radiologic, and pathologic data is required for diagnosis. This work was undertaken to develop and validate an international set of classification criteria for IgG4-RD., Methods: An international multispecialty group of 86 physicians was assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR). Investigators used consensus exercises, existing literature, derivation and validation cohorts of 1,879 subjects (1,086 cases, 793 mimickers), and multicriterion decision analysis to identify, weight, and test potential classification criteria. Two independent validation cohorts were included., Results: A 3-step classification process was developed. First, it must be demonstrated that a potential IgG4-RD case has involvement of at least 1 of 11 possible organs in a manner consistent with IgG4-RD. Second, exclusion criteria consisting of a total of 32 clinical, serologic, radiologic, and pathologic items must be applied; the presence of any of these criteria eliminates the patient from IgG4-RD classification. Third, 8 weighted inclusion criteria domains, addressing clinical findings, serologic results, radiology assessments, and pathology interpretations, are applied. In the first validation cohort, a threshold of 20 points had a specificity of 99.2% (95% confidence interval [95% CI] 97.2-99.8%) and a sensitivity of 85.5% (95% CI 81.9-88.5%). In the second, the specificity was 97.8% (95% CI 93.7-99.2%) and the sensitivity was 82.0% (95% CI 77.0-86.1%). The criteria were shown to have robust test characteristics over a wide range of thresholds., Conclusion: ACR/EULAR classification criteria for IgG4-RD have been developed and validated in a large cohort of patients. These criteria demonstrate excellent test performance and should contribute substantially to future clinical, epidemiologic, and basic science investigations., (© 2019, American College of Rheumatology.)

Objective: To examine temporal trends in mortality rates in a large nationwide cohort of patients with end-stage renal disease (ESRD) due to granulomatosis with polyangiitis (Wegener's) (GPA-ESRD)., Methods: We identified cases of GPA-ESRD reported in the US Renal Data System between 1995 and 2014, using coding by nephrologists for the cause of ESRD. The cohort was divided into four 5-year subcohorts based on the year of onset of ESRD (1995-1999, 2000-2004, 2005-2009, and 2010-2014) to assess trends in mortality rates and hazard ratios (HRs) for overall death and cause-specific death, adjusting for potential confounders., Results: Between 1995 and 2014, there were 5,929 incident cases of GPA-ESRD. The mortality rate (incidence per 100 patient-years) declined from 19.0 in 1995-1999 to 15.3 in 2010-2014 (P for trend = 0.01). The multivariable-adjusted HR for mortality in the 2010-2014 cohort was 0.77 (95% confidence interval [95% CI] 0.66-0.90) (P for trend < 0.001 versus the 1995-1999 cohort). The corresponding cause-specific HRs for mortality, after accounting for competing risk, were 0.61 (95% CI 0.47-0.80) for CVD-related death and 0.42 (95% CI 0.28-0.63) for death due to infection (P for trend < 0.001)., Conclusion: In this study of nearly all patients in whom GPA-ESRD developed in the US over 2 decades, we observed significant improvements in mortality among GPA-ESRD patients. The number of cause-specific deaths due to CVD and the number due to infections declined significantly during the study period. These findings are encouraging and likely reflect improved management of both GPA and ESRD., (© 2018, American College of Rheumatology.)

Background: Renal transplantation is the optimal treatment for selected patients with end-stage renal disease (ESRD). However, the survival benefit of renal transplantation among patients with ESRD attributed to granulomatosis with polyangiitis (GPA) is unknown., Methods: We identified patients from the United States Renal Data System with ESRD due to GPA (ESRD-GPA) between 1995 and 2014. We restricted our analysis to waitlisted subjects to evaluate the impact of transplantation on mortality. We followed patients until death or the end of follow-up. We compared the relative risk (RR) of all-cause and cause-specific mortality in patients who received a transplant versus non-transplanted patients using a pooled logistic regression model with transplantation as a time-varying exposure., Results: During the study period, 1525 patients were waitlisted and 946 received a renal transplant. Receiving a renal transplant was associated with a 70% reduction in the risk of all-cause mortality in multivariable-adjusted analyses (RR=0.30, 95% CI 0.25 to 0.37), largely attributed to a 90% reduction in the risk of death due to cardiovascular disease (CVD) (RR=0.10, 95% 0.06-0.16)., Discussion: Renal transplantation is associated with a significant decrease in all-cause mortality among patients with ESRD attributed to GPA, largely due to a decrease in the risk of death to CVD. Prompt referral for transplantation is critical to optimise outcomes for this patient population., Competing Interests: Competing interests: ZSW has received funding from a Scientist Development Award from the Rheumatology Research Foundation, a Fund for Medical Discovery Award from the Executive Committee on Research at Massachusetts General Hospital, and an NIH Loan Repayment Award., (© Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Although chronic inflammatory disorders of the ocular adnexa are relatively common, their pathogenesis is in many cases poorly understood. Recent investigation suggests that many cases of sclerosing orbital inflammation are a manifestation of IgG4-related disease; however, most patients reported have been Asian, and it is not clear whether the results of studies from the Far East can be reliably extrapolated to draw conclusions about Western patients. We evaluated 38 cases previously diagnosed as orbital inflammatory pseudotumor or chronic dacryoadenitis to determine whether our cases fulfill the criteria for IgG4-RD (IgG4-related dacryoadenitis when involving the lacrimal gland, and IgG4-related sclerosing orbital inflammation when involving orbital soft tissue). Fifteen patients had IgG4-related dacryoadenitis or orbital inflammation. These patients included 9 men and 6 women, aged 24 to 77 years (median, 64 y). Lesions involved orbital soft tissue (8 cases), lacrimal gland (6 cases), and canthus (1 case). In 1 case, focal in situ follicular neoplasia was seen in a background of IgG4-RD. In another case, a clonal IGH gene rearrangement was detected. Four patients with IgG4-RD had evidence of IgG4-RD in other anatomic sites. Five patients, 1 man and 4 women, aged 26 to 74 years (median 50 y) had orbital lesions (2 involving lacrimal gland, 3 involving soft tissue) suspicious for, but not diagnostic of, IgG4-RD. Of 16 patients with IgG4-RD or probable IgG4-RD with information available regarding the course of their disease, 11 patients experienced recurrent or persistent orbital disease. However, no patient developed lymphoma, and no patient died of complications of IgG4-RD. Eighteen patients had lesions not representing IgG4-RD. They included 6 male and 12 female individuals aged 6 to 77 years (median, 47 y). These patients had a variety of diseases, including granulomatosis with polyangiitis (3 cases), Rosai-Dorfman disease (1 case), nonspecific chronic inflammation and fibrosis involving lacrimal gland or soft tissue (12 cases), and others. Clinical and pathologic findings among our patients with IgG4-RD involving the orbit are similar to those previously described in Asian patients. Careful evaluation of histologic and immunophenotypic features and clinical correlation are required to distinguish orbital IgG4-RD from other sclerosing inflammatory lesions in the orbit.

Objective: Standard treatment for severe granulomatosis with polyangiitis (Wegener's) (GPA) is daily oral cyclophosphamide (CYC), a cytotoxic agent associated with ovarian failure. In this study, we assessed the rate of diminished ovarian reserve in women with GPA who received CYC versus methotrexate (MTX)., Methods: Patients in the Wegener's Granulomatosis Etanercept Trial received either daily CYC or weekly MTX and were randomized to etanercept or placebo. For all women ages <50 years, plasma samples taken at baseline or early in the study were evaluated against samples taken later in the study to compare levels of anti-Müllerian hormone (AMH) and follicle-stimulating hormone (FSH), endocrine markers of remaining egg supply. Diminished ovarian reserve was defined as an AMH level of <1.0 ng/ml., Results: Of 42 women in this analysis (mean age 35 years), 24 had CYC exposure prior to enrollment and 28 received the drug during the study. At study entry, women with prior CYC exposure had significantly lower AMH, higher FSH, and a higher rate of early menstruation cessation. For women with normal baseline ovarian function, 6 of 8 who received CYC during the trial developed diminished ovarian reserve, compared to 0 of 4 who did not receive CYC (P < 0.05). Changes in AMH correlated inversely with cumulative CYC dose (P < 0.01), with a 0.74 ng/ml decline in AMH level for each 10 gm of CYC., Conclusion: Daily oral CYC, even when administered for less than 6 months, causes diminished ovarian reserve, as indicated by low AMH levels. These data highlight the need for alternative treatments for GPA in women of childbearing age., (Copyright © 2011 by the American College of Rheumatology.)

Two factors--accountability for care and an increased adoption of interoperable electronic health records (EHRs)--will significantly increase the importance of data for healthcare organizations. These factors have significant data ramifications in four areas: payment reform, data use policies, IT strategy, and data management. As data become a strategic asset, healthcare finance executives will need to become significant contributors to their organizations' EHR initiatives and data management strategies.

Business intelligence--technology to manage and leverage an organization's data--can enhance healthcare organizations' financial and operational performance and quality of patient care. Effective BI management requires five preliminary steps: Establish business needs and value. Obtain buy-in from managers. Create an end-to-end vision. Establish BI governance. Implement specific roles for managing data quality.

Damage denotes the aspects of chronic disease that do not reverse with therapy. This concept is particularly important for the primary systemic vasculitides, since the careful differentiation between activity and damage may help avoid unnecessary exposure to cytotoxic medications. Damage significantly influences both longterm prognosis and quality of life. Because the primary systemic vasculitides have diverse manifestations, the use of a damage assessment instrument is crucial to ensure reproducibility. The Vasculitis Damage Index (VDI) is the only validated measure for damage assessment in vasculitis. Use of the VDI in recent clinical trials has shown that it may not adequately determine the full spectrum of damage experienced by patients with vasculitis of small- and medium-size vessels. We propose reexamining the way in which damage is assessed, focusing on vasculitides of small- and medium-size vessels, and outline an initiative to create a substantially revised and improved damage assessment instrument using data-driven approaches. This initiative is part of a larger international effort to create a unified approach to disease assessment for the primary systemic vasculitides.