14-month-old female with anti-MDA5 juvenile dermatomyositis complicated by liver disease: a case report.

BACKGROUND: Juvenile Dermatomyositis (JDM) is a rare disorder with subtypes associated with different myositis-specific antibodies (MSAs) including anti-MDA5. Hepatic involvement in JDM is rare and has not previously been documented in anti-MDA5 JDM. There is a lack of formal research on treatment protocols for anti-MDA5 JDM, though tofacitinib is a highly regarded emerging therapy. CASE PRESENTATION: A previously healthy 14-month-old Hispanic female presented to a pediatric rheumatology clinic with eight months of worsening rash, weakness, periorbital edema, intermittent fevers, and weight loss. Her physical exam was notable for fever, thinning of hair, heliotrope rash, periorbital edema, violaceous macules on her bilateral elbows, forearms, arms, and knees, arthritis, Gottron's sign, and hepatomegaly. The patient was admitted, and symptoms progressed to include hypoxemia. Subsequent workup was notable for ground glass opacities of bilateral lung fields on chest CT, myositis visualized on MRI and confirmed with muscle biopsy, and liver biopsy showing nonspecific signs of liver injury. After a thorough infectious disease workup to rule out concomitant infection, the patient was started on high-dose steroids and induction with cyclophosphamide. She responded well with disease remission maintained with tofacitinib in the outpatient setting. DISCUSSION AND CONCLUSIONS: Our patient is notable due to her young age at presentation, histopathologically confirmed liver injury, and response to treatment. The case adds to the growing body of literature supporting tofacitinib for anti-MDA5 JDM in the pediatric population. Future research can better standardize effective treatment protocols and define the mechanism of liver involvement. For patients with nonspecific liver injury, muscular, and cutaneous disease, anti-MDA5 JDM should be considered in the differential diagnosis with treatment options including tofacitinib for confirmed cases.

Addressing the Concern of Orange-Yellow Fungus Growth on Palm Kernel Cake: Safeguarding Dairy Cattle Diets for Mycotoxin-Producing Fungi.

Palm kernel cake (PKC), a byproduct of palm oil extraction, serves an important role in Ecuador's animal feed industry. The emergence of yellow-orange fungal growth in PKC on some cattle farms in Ecuador sparked concerns within the cattle industry regarding a potential mycotoxin-producing fungus on this substrate. Due to the limited availability of analytical chemistry techniques in Ecuador for mycotoxin detection, we chose to isolate and identify the fungus to determine its association with mycotoxin-producing genera. Through molecular identification via ITS region sequencing, we identified the yellow-orange fungus as the yeast Candida ethanolica. Furthermore, we isolated two other fungi-the yeast Pichia kudriavzevii, and the fungus Geotrichum candidum. Molecular identification confirmed that all three species are not classified as mycotoxin-producing fungi but in contrast, the literature indicates that all three have demonstrated antifungal activity against Aspergillus and Penicillium species, genera associated with mycotoxin production. This suggests their potential use in biocontrol to counter the colonization of harmful fungi. We discuss preventive measures against the fungal invasion of PKC and emphasize the importance of promptly identifying fungi on this substrate. Rapid recognition of mycotoxin-producing and pathogenic genera holds the promise of mitigating cattle intoxication and the dissemination of mycotoxins throughout the food chain.

Improving Pneumococcal Vaccination Rates in Immunosuppressed Pediatric Patients with Rheumatic Disease.

Background: Patients with rheumatic diseases are at a high risk of invasive pneumococcal disease due to immunosuppression. We conducted a quality improvement project, and the first aim was to increase the percentage of patients with systemic lupus erythematosus and mixed connective tissue disease that is up to date on pneumococcal vaccinations from 9.6% to 80% within one year. Subsequently, the second aim was to increase the percentage of patients on immunosuppression with systemic lupus erythematosus, mixed connective tissue disease, juvenile dermatomyositis and systemic vasculitis that is up to date on pneumococcal vaccinations from 62.6% to 80% within one year. Methods: Two process measures were up-to-date vaccination status on (1) 13-valent pneumococcal conjugated vaccine (PCV13) and (2) 23-valent pneumococcal polysaccharide vaccine (PPSV23). Our outcome measure was being fully up to date on both pneumococcal vaccinations. Interventions included an immunization algorithm, reporting of eligible patients, education, reminders, and pre-visit planning. Results: There were shifts in the centerline for all quality measures in both phases of this project. The combined pneumococcal vaccination rate for Phase 1 increased from 9.6% to 91.1%, and this centerline was sustained. Pneumococcal vaccination rates also significantly increased for Phase 2: 68.8% to 93.4% for PCV13, 65.2% to 88.5% for PPSV23, and 62.6% to 86.5% for the combined pneumococcal vaccination rate. Conclusions: Quality improvement methodology significantly increased and sustained pneumococcal vaccination rates in our high-risk, immunosuppressed patients. We continue to prioritize this important initiative to mitigate the risk of invasive pneumococcal disease.

Association Between Interleukin 6 and C-Reactive Protein Serum Levels and Body Composition Compartments and Components in Breast Cancer Survivors.

Obesity is highly prevalent in breast cancer (BC) survivors. Adipose tissue promotes inflammation, affecting recurrence, morbidity, and quality of life. This study aimed to determine the relationship of body composition parameters with the levels of C-reactive protein (CRP) and interleukin 6 (IL-6) in female BC survivors. Additionally, we evaluated the association of log-transformed serum concentrations of CRP and IL-6 with the appendicular skeletal lean mass index (ASMI). The results showed that CRP was positively associated with body fat percentage (BFP; ß adjusted = .08, 95% CI: .02-.14) in all participants, and with fat mass index (FMI; ß = .24, 95% CI: .08-.40) only in premenopausal women. IL-6 was positively associated with FMI (ß adjusted = .16, 95% CI: .03-.29), while ASMI decreased as CRP levels increased (ß adjusted = -.30, 95% CI: -.53 to -.06). Interventions to improve body composition in BC survivors should also consider the role of inflammatory markers in changes in body composition to avoid sarcopenic obesity (SO) and the risk of BC recurrence.

Capturing the Range of Disease Involvement in Localized Scleroderma: The Localized Scleroderma Total Severity Scale.

OBJECTIVE: Juvenile localized scleroderma (jLS) is a chronic autoimmune disease commonly associated with poor outcomes, including contractures, hemiatrophy, uveitis, and seizures. Despite improvements in treatment, >25% of patients with jLS have functional impairment. To improve patient evaluation, our workgroup developed the Localized scleroderma Total Severity Scale (LoTSS), an overall disease severity measure. METHODS: LoTSS was developed as a weighted measure by a consensus process involving literature review, surveys, case vignettes, and multicriteria decision analysis. Feasibility was assessed in larger Childhood Arthritis and Rheumatology Research Alliance groups. Construct validity with physician assessment and inter-rater reliability was assessed using case vignettes. Additional evaluation was performed in a prospective patient cohort initiating treatment. RESULTS: LoTSS severity items were organized into modules that reflect jLS disease patterns, with modules for skin, extracutaneous, and craniofacial manifestations. Construct validity of LoTSS was supported by a strong positive correlation with the Physician Global Assessment (PGA) of severity and damage and weak positive correlation with PGA-Activity, as expected. LoTSS was responsive, with a small effect size identified. Moderate-to-excellent inter-rater reliability was demonstrated. LoTSS was able to discriminate between patient subsets, with higher scores identified in those with greater disease burden and functional limitation. CONCLUSION: We developed a new LS measure for assessing cutaneous and extracutaneous severity and have shown it to be reliable, valid, and responsive. LoTSS is the first measure that assesses and scores all the major extracutaneous manifestations in LS. Our findings suggest LoTSS could aid assessment and management of patients and facilitate outcome evaluation in treatment studies.

Pediatric systemic juvenile idiopathic arthritis-related lung disease: Description of clinical cohort and review of management.

CONTEXT: Since the early 2010s, there has been an increased awareness of interstitial lung disease in systemic juvenile idiopathic arthritis (sJIA-LD) in pediatric patients. Despite the increase in prevalence of sJIA-LD, little is known about this disease process and effective therapeutic management. OBJECTIVES: To identify and characterize the disease process and management of interstitial lung disease related to systemic juvenile idiopathic arthritis. STUDY DESIGN: In this single-center, retrospective case series of 9 patients, we analyze demographic, clinical, radiographic, and laboratory data to corroborate common clinical characteristics and describe an approach for diagnosis and monitoring of sJIA-LD. DATA EXTRACTION: All data was extracted through electronic medical records and individually reviewed by two pediatric pulmonologists and two pediatric rheumatologists. RESULTS: Our results were similar to other described cases of sJIA-LD as patients in our cohort were more likely to be younger, have a history of macrophage activation syndrome and prior use of biologic therapies. In contrast to prior studies, they did not present with peripheral lymphadenopathy and hepatosplenomegaly. LIMITATIONS: The cohort size was small and data is reflective of one center's approach to management of a rare lung disease process. CONCLUSION: Interstitial lung disease due to sJIA is rare and management can be difficult in these complex patients.More research is necessary to understand the increased incidence and treatment of sJIA-LD in pediatric population.

Residency Application Advising: Medical Student & Faculty Advisor Perceived Barriers & Expectations.

INTRODUCTION: Given the rapidly changing landscape of residency applications, many medical students struggle to identify guidance from faculty advisors. Additionally, faculty advisors may find it difficult to maintain up-to-date knowledge on changes such as the new supplemental application. These gaps could potentially lead to inequitable advising. The objective of this study was to identify both students' and faculty's perceived barriers and expectations for residency application advising. METHODS: Anonymous surveys were administered to both fourth-year medical students and faculty advisors at a single institution within 2 mo of the residency application deadline. Survey questions assessed student and faculty barriers to establishing the advisor-advisee relationships, as well as expectations of the advisor role. Surveys were analyzed using descriptive statistics. RESULTS: We identified that the majority of students (57%) did not have a faculty advisor within weeks of the application deadline, and an equal amount felt that finding an advisor was either somewhat difficult or extremely difficult. Of all the students, 60% felt their biggest barrier was not knowing how to find an advisor. Though faculty felt equipped to advise students, 75% of faculty in the participating specialties had advising concerns regarding the supplemental application or were unaware of the changes. CONCLUSIONS: We identified gaps in the residency application advising process from both student and faculty perspectives. Future work involves increasing awareness of the resources and opportunities available to students to improve advising relationships. Standardized training tools and resources for faculty will result in more consistent and reliable faculty advising.

BMI is Not Associated with Chest-Specific Body Image, Complications or Revisions in Gender-Affirming Mastectomy: A Single-Center Cross-Sectional Study.

OBJECTIVE: To analyze the impact of Body Mass Index (BMI) on clinical and patient-reported outcomes following gender-affirming mastectomy (GM). BACKGROUND: BMI is a barrier for obese patients seeking GM despite increasing evidence that it is safe in this population. Currently little is known about the impact of BMI on chest-specific body image and satisfaction following GM. METHODS: This single-center, cross-sectional study included individuals 18 years and older who underwent GM between 1990-2020 and were at least 2 years post-operative. Patient-reported chest-specific body image was measured using the BODY-Q and Gender Congruence and Life Satisfaction (GCLS) chest subscales. Satisfaction was measured using the Holmes-Rovner Satisfaction with Decision (SWD) scale. Clinical and demographic variables were identified from chart review. Bivariate analysis was performed to determine if BMI was associated with chest-specific body image, satisfaction, complications within 30 days or revisions in GM. RESULTS: Two hundred twenty-seven individuals meeting eligibility criteria were contacted to participate and one hundred thirty-seven responded (60.4% response rate). The mean age was 29.1 (SD=9.0) and mean BMI was 30.9 (SD=8.0), with 26.4% (N=60) of the cohort having a BMI>35. Chest-specific body image, and satisfaction with decision did not vary by BMI or breast resection weight. Complications and revisions were not associated with BMI. CONCLUSION: Individuals undergoing GM reported high rates of satisfaction following GM regardless of BMI. Complication and revision rates did not vary significantly by BMI or breast resection weight. Surgeons should re-evaluate the role BMI plays in patient selection and counseling for GM.

Long-Term Regret and Satisfaction With Decision Following Gender-Affirming Mastectomy.

Importance: There has been increasing legislative interest in regulating gender-affirming surgery, in part due to the concern about decisional regret. The regret rate following gender-affirming surgery is thought to be approximately 1%; however, previous studies relied heavily on ad hoc instruments. Objective: To evaluate long-term decisional regret and satisfaction with decision using validated instruments following gender-affirming mastectomy. Design, Setting, and Participants: For this cross-sectional study, a survey of patient-reported outcomes was sent between February 1 and July 31, 2022, to patients who had undergone gender-affirming mastectomy at a US tertiary referral center between January 1, 1990, and February 29, 2020. Exposure: Decisional regret and satisfaction with decision to undergo gender-affirming mastectomy. Main Outcomes and Measures: Long-term patient-reported outcomes, including the Holmes-Rovner Satisfaction With Decision scale, the Decision Regret Scale, and demographic characteristics, were collected. Additional information was collected via medical record review. Descriptive statistics and univariable analysis using Fisher exact and Wilcoxon rank sum tests were performed to compare responders and nonresponders. Results: A total of 235 patients were deemed eligible for the study, and 139 responded (59.1% response rate). Median age at the time of surgery was 27.1 (IQR, 23.0-33.4) years for responders and 26.4 (IQR, 23.1-32.7) years for nonresponders. Nonresponders (n = 96) had a longer postoperative follow-up period than responders (median follow-up, 4.6 [IQR, 3.1-8.6] vs 3.6 [IQR, 2.7-5.3] years, respectively; P = .002). Nonresponders vs responders also had lower rates of depression (42 [44%] vs 94 [68%]; P < .001) and anxiety (42 [44%] vs 97 [70%]; P < .001). No responders or nonresponders requested or underwent a reversal procedure. The median Satisfaction With Decision Scale score was 5.0 (IQR, 5.0-5.0) on a 5-point scale, with higher scores noting higher satisfaction. The median Decision Regret Scale score was 0.0 (IQR, 0.0-0.0) on a 100-point scale, with lower scores noting lower levels of regret. A univariable regression analysis could not be performed to identify characteristics associated with low satisfaction with decision or high decisional regret due to the lack of variation in these responses. Conclusions and Relevance: In this cross-sectional survey study, the results of validated survey instruments indicated low rates of decisional regret and high levels of satisfaction with decision following gender-affirming mastectomy. The lack of dissatisfaction and regret impeded the ability to perform a more complex statistical analysis, highlighting the need for condition-specific instruments to assess decisional regret and satisfaction with decision following gender-affirming surgery.

The Development of a Multiplex PCR Assay for Fast and Cost-Effective Identification of the Five Most Significant Pathogenic Prototheca Species.

A multiplex PCR system (m-PCR) has been developed to accurately differentiate the five most important pathogenic Prototheca species, including the three species associated with infection in dairy cattle (P. ciferrii, P. blaschkeae, and P. bovis) and the two species associated with human infections (P. wickerhamii and P. cutis). The method is low-cost since it employs a simple "heat-shock" method in a TE buffer for DNA extraction. Furthermore, it requires only primers, a Taq polymerase, an agarose gel, and a molecular weight marker for identification. The method was based on published Prototheca cytochrome B sequences and was evaluated using reference strains from each of the five Prototheca species. The validity of the method was confirmed by identifying 50 strains isolated from milk samples. The specificity was tested in silico and with experimental PCR trials, showing no cross-reactions with other Prototheca species, as well as with bacteria, fungi, cows, algae, animals, or humans. The method could detect mixed infections involving two or three Prototheca species, providing a rapid test that delivers results within three hours.

Coinfección COVID-19 y tuberculosis: experiencia de una terapia intensiva durante el periodo enero 2020-junio 2022 / COVID-19 and tuberculosis co-infection: experience of an intensive care unit during the period of January-june 2022

Antecedentes: Se ha demostrado que la coinfección tu-berculosis y COVID-19 presenta peor evolución clínica. La inmunidad protectora se debilita frente a esta situación, generando fallo en el control de ambas infecciones, reac-tivación de formas latentes de tuberculosis y progresión exacerbada de los casos activos. Asimismo, la terapia con corticoides utilizada dentro del tratamiento de infecciones graves por COVID-19 puede generar inmunosupresión y precipitar la progresión de la tuberculosis.Objetivos: Describir las características clínicas, presenta-ción y evolución de los pacientes críticos con coinfección COVID-19 y tuberculosis. Evaluar la incidencia y letalidad de la asociación COVID-19 y tuberculosis en cuidados in-tensivos. Materiales y métodos: Se realizó un estudio retrospectivo, descriptivo. Se revisaron 12 historias clínicas de pacientes con coinfección COVID-19-tuberculosis sobre 1014 histo-rias clínicas de pacientes ingresados con diagnóstico de COVID-19, durante el periodo comprendido enero 2020 y junio 2022. Se utilizó estadística descriptiva. Resultados y discusión: Sobre un total de 1014 historias clínicas, se encontraron 12 pacientes con coinfección (in-cidencia de 0,011). La letalidad global en cuidados inten-sivos fue del 75%, a los 45 días fue del 83,3%, duplicando la letalidad general de los pacientes COVID-19 no coinfec-tados ingresados durante el mismo periodo (75% versus 37%). Los pacientes que requirieron ingreso a ventilación RESUMENARTÍCULO ORIGINALmecánica tuvieron una letalidad del 100% y aquellos que tenían infección por virus de inmunodeficiencia adquirida presentaron una letalidad de 100%. Resulta importante describir los hallazgos y alertar sobre la evolución desfavorable de aquellos pacientes que pre-sentan esta asociación a fin de optimizar el manejo y espe-cialmente recomendar la búsqueda de coinfección cuando el criterio clínico lo requiera

Background: Coinfection with tuberculosis and COVID-19 has been shown to have a worse clinical course. Protective immunity is weakened in this situation, leading to failure to control both infections, reactivation of latent forms of TB and exacerbated progression of active cases. Furthermore, corticosteroid therapy used in the treatment of severe COVID-19 infections can lead to immunosuppression and precipitate TB progression.Objectives: To describe the clinical characteristics, presentation and evolution of critically ill patients with COVID-19 and tuberculosis co-infection.To evaluate the incidence and lethality of COVID-19 and tuberculosis association in intensive care.Materials and methods: A retrospective, descriptive study was conducted. Twelve medical records of patients aged 18 years or older admitted to intensive care with a diagnosis of COVID-19 during the period January 2020 to July 2022 were reviewed. Descriptive statistics were used.Results and discussion: Out of a total of 1014 medical records, 12 patients were found with co-infection (incidence 0.011). The global intensive care case fatality was 75%, at 45 days it was 83.3%. This was twice the overall case fatality of non-co-infected COVID-19 patients admitted during the same period (75% versus 37%). Patients requiring admission to mechanical ventilation had a 100% case fatality and those with acquired immunodeficiency virus infection had a 100% case fatality.It is important to describe the findings and to alert to the worse evolution of those patients presenting with this association, in order to improve management and recommend searching for co-infection when clinical criteria require it

High Prevalence of Prototheca bovis Infection in Dairy Cattle with Chronic Mastitis in Ecuador.

The genus Prototheca, a unicellular, non-photosynthetic, yeast-like microalgae, is a pathogen of concern for the dairy industry. It causes bovine mastitis that currently cannot be cured, and hence generates significant economic losses in milk production. In this study, for the first time in Ecuador, we identify Prototheca bovis as the etiologic agent of chronic mastitis in dairy cattle. Milk samples (n = 458) of cows with chronic mastitis were cultured on Sabouraud Dextrose Agar (SDA). Microscopy and cytB gene sequencing were used to identify Prototheca, whereby Prototheca bovis was isolated from 15.1% (n = 69) of the milk samples, one of the highest infection rates that can be found in the literature in a "non-outbreak" situation. No other Prototheca species were found. We were unable to isolate the alga from environmental samples. We showed that P. bovis was relatively resistant to disinfectants used to sterilize milking equipment on the cattle farms where it was isolated. We discuss how to avoid future infection and also hypothesize that the real prevalence of Prototheca infection in bovine mastitis is probably much higher than what was detected. We recommend a protocol to increase the diagnostic yield in the bacteriology laboratory.

What is Currently Known about Odontogenic Keratocysts?

PURPOSE: To present updated information on odontogenic keratocyst (OKC) classification, etiology, genetic and molecular alterations, epidemiology, clinical presentation, radiographic characteristics, histological and immune histochemical features, differential diagnosis, treatment, and controversies, as well as a literature review of case frequencies in different countries. MATERIALS AND METHODS: Studies were selected using the key words 'odontogenic keratocyst,' 'odontogenic cysts,' 'odontogenic keratocyst and clinical study'. Full-text papers were reviewed on the basis of the inclusion and exclusion criteria. The literature search aimed to find articles that would show the frequency of OKC, dentigerous cyst, radicular cyst, and other cysts. RESULTS: OKC presents local aggression and high recurrence; therefore, a better understanding of its clinical characteristics and the genetic and molecular factors involved in this peculiar and controversial lesion is required. It is always essential to discuss treatment alternatives. Although OKC is an entity with a high recurrence, aggressive treatment is not advisable in all cases because factors such as commitment to anatomical structures and possible complications should be considered. However, periodic radiographic controls are advised. CONCLUSION: To reduce the high number of present cases worldwide, it is important to improve knowledge on this pathology so that accurate diagnoses can be achieved and appropriate treatment can be provided. OKC presents local aggression and high recurrence; therefore, a better understanding is needed of the clinical characteristics and genetic and molecular factors involved in OKC. Furthermore, it is always essential to discuss treatment alternatives.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease.

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.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Kawasaki Disease.

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.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis.

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.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

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.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.

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/Vasculitis Foundation Guideline for the Management of Giant Cell Arteritis and Takayasu Arteritis.

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.

2021 American College of Rheumatology/Vasculitis Foundation Guideline for the Management of Polyarteritis Nodosa.

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.