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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]

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 Rheumatoid arthritis is associated with an excess of agalactosylated (G0) IgG that is considered relatively proinflammatory. Assessment of this association in juvenile idiopathic arthritis (JIA) is complicated by age-dependent IgG glycan variation. The aim of this study was to conduct the first large-scale survey of IgG glycans in healthy children and patients with JIA, with a focus on early childhood, the time of peak JIA incidence. Methods IgG glycans from healthy children and disease-modifying antirheumatic drug-naive patients with JIA were characterized using high-performance liquid chromatography. Agalactosylated glycans were quantitated with reference to monogalactosylated (G1) species. Associations were sought between the G0:G1 ratio and disease characteristics. Results Among healthy children ages 9 months to 16 years (n = 165), the G0:G1 ratio was highly age dependent, with the ratio peaking to 1.19 in children younger than age 3 years and declining to a nadir of 0.83 after age 10 years (Spearman's ρ = 0.60, P < 0.0001). In patients with JIA (n = 141), the G0:G1 ratio was elevated compared with that in control subjects (1.32 versus 1.02; P < 0.0001). The G0:G1 ratio corrected for age was abnormally high in all JIA subtypes (enthesitis-related arthritis was not assessed), most strikingly in systemic JIA. Glycosylation aberrancy was comparable in patients with and those without antinuclear antibodies and in both early- and late-onset disease and exhibited at most a weak correlation with markers of inflammation. Conclusion IgG glycosylation is skewed toward proinflammatory G0 variants in healthy children, in particular during the first few years of life. This deviation is exaggerated in patients with JIA. The role for IgG glycan variation in immune function in children, including the predilection of JIA for early childhood, remains to be defined. [ABSTRACT FROM AUTHOR]

Objective To determine whether aggressive treatment initiated early in the course of rheumatoid factor (RF)-positive or RF-negative polyarticular juvenile idiopathic arthritis (JIA) can induce clinical inactive disease within 6 months. Methods Between May 2007 and October 2010, a multicenter, prospective, randomized, double-blind, placebo-controlled trial of 2 aggressive treatments was conducted in 85 children ages 2-16 years with polyarticular JIA of <12 months' duration. Patients received either methotrexate (MTX) 0.5 mg/kg/week (maximum 40 mg) subcutaneously, etanercept 0.8 mg/kg/week (maximum 50 mg), and prednisolone 0.5 mg/kg/day (maximum 60 mg) tapered to 0 by 17 weeks (arm 1), or MTX (same dosage as arm 1), etanercept placebo, and prednisolone placebo (arm 2). The primary outcome measure was clinical inactive disease at 6 months. An exploratory phase determined the rate of clinical remission on medication (6 months of continuous clinical inactive disease) at 12 months. Results By 6 months, clinical inactive disease had been achieved in 17 (40%) of 42 patients in arm 1 and 10 (23%) of 43 patients in arm 2 (χ2 = 2.91, P = 0.088). After 12 months, clinical remission on medication was achieved in 9 patients in arm 1 and 3 patients in arm 2 ( P = 0.053). There were no significant interarm differences in adverse events. Conclusion Although this study did not meet its primary end point, early aggressive therapy in this cohort of children with recent-onset polyarticular JIA resulted in clinical inactive disease by 6 months and clinical remission on medication within 12 months of treatment in substantial proportions of patients in both arms. [ABSTRACT FROM AUTHOR]

OBJECTIVES: We sought to analyze trends in admissions and to describe therapies used for acute Kawasaki disease over a 6-year period. METHODS: The Pediatric Health Information System provides patient data including demographic variables, International Classification of Diseases, Ninth Revision codes, and services billed to patients. Patient identifiers enable tracking of medication use in and across multiple admissions within a center. We analyzed data for patients with (1) a diagnosis code for Kawasaki disease, (2) intravenously administered immunoglobulin treatment during hospitalization, and (3) discharge between January 1, 2001, and December 30, 2006, from 27 hospitals contributing complete data over the study period. RESULTS: During the study period, 5197 Kawasaki disease admissions were identified for 4811 patients; numbers increased 32.6% from 2001 (n = 678) to 2006 (n = 899). Retreatment with intravenous immunoglobulin was administered to 712 patients (14.8%) over the study period. Other antiinflammatory therapies included intravenously administered methylprednisolone (5.8%), orally administered prednisone (2.8%), and infliximab (1%). Use of infliximab steadily increased from 0.0% (0 of 678 patients) in 2001 to 2.3% (21 of 899 patients) in 2006. Coronary artery aneurysms were coded for 3.3% of patients. Male patients, patients <1 year of age, and Hispanic patients were significantly more likely to have coding for coronary artery aneurysms. CONCLUSIONS: Our report provides the first large multicenter description of agents used in the treatment of intravenously administered immunoglobulin-resistant Kawasaki disease in the United States. Trends include increased numbers of admissions attributable to Kawasaki disease and increased usage of infliximab. [ABSTRACT FROM AUTHOR]

OBJECTIVE. The goal was to describe characteristics of children discharged from hospitals in the United States in 2000 with the diagnosis of acute rheumatic fever. METHODS. We explored characteristics of children <21 years of age who were hospitalized with a diagnosis of acute rheumatic fever by using the 2000 Kids' Inpatient Database, weighted to estimate the number and rate of acute rheumatic fever-associated hospitalizations in the United States. RESULTS. In 2000, an estimated 503 acute rheumatic fever hospitalizations occurred among children <21 years of age, at a rate of 14.8 cases per 100 000 hospitalized children, with a mean age of 10 years. In comparison with all Kids' Inpatient Database admissions, acute rheumatic fever hospitalizations were more common in the age group of 6 to 11 years and among male patients. Chorea was more common in female patients (61.7%). White patients were significantly underrepresented, whereas Asian/Pacific islander patients and patients of other races were overrepresented. Hospitalizations of patients with acute rheumatic fever were significantly more common in the Northeast and less common in the South. The highest rates of acute rheumatic fever hospitalizations occurred in Utah, Hawaii, Pennsylvania, and New York. Significantly more acute rheumatic fever admissions occurred in March. The expected payor was more likely to be private insurance and less likely to be Medicaid. Acute rheumatic fever hospitalizations were more likely to occur in teaching hospitals, freestanding children's hospitals, and children's units in general hospitals and in urban locations. The median length of stay for acute rheumatic fever hospitalizations was 3 days, and the median total charges were $6349. The in-hospital mortality rate was 0.6%. CONCLUSIONS. In 2000, we found that hospitalizations for acute rheumatic fever were infrequent and varied according to race, season, location, and type of hospital. [ABSTRACT FROM AUTHOR]

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: 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.)

Background: Multicenter studies on idiopathic or viral pericarditis and pericardial effusion (PPE) have not been reported in children. Colchicine use for PPE in adults is supported. We explored epidemiology and management for inpatient hospitalizations for PPE in US children and risk factors for readmission., Methods and Results: We analyzed patients in the Pediatric Health Information System database for (1) a code for PPE; (2) absence of codes for underlying systemic disease (eg, neoplastic, cardiac, rheumatologic, renal); (3) age ≥30 days and <21 years; and (4) discharge between January 1, 2007, and December 31, 2012, from 38 hospitals contributing complete data for each year of the study period. Among 11 364 hospitalizations with PPE codes during the study period, 543 (4.8%) met entry criteria for idiopathic or viral PPE. Significantly more boys were noted, especially among adolescents. No temporal trends were noted. Median age was 14.5 years (interquartile range 7.3 to 16.6 years); 78 patients (14.4%) underwent pericardiocentesis, 13 (2.4%) underwent pericardiotomy, and 11 (2.0%) underwent pericardiectomy; 157 (28.9%) had an intensive care unit stay, including 2.0% with tamponade. Median hospitalization was 3 days (interquartile range 2 to 4 days). Medications used at initial admission were nonsteroidal anti-inflammatory drugs (71.3%), corticosteroids (22.7%), aspirin (7.0%), and colchicine (3.9%). Readmissions within 1 year of initial admission occurred in 46 of 447 patients (10.3%), mostly in the first 3 months. No independent predictors of readmission were noted, but our statistical power was limited. Practice variation was noted in medical management and pericardiocentesis., Conclusions: Our report provides the first large multicenter description of idiopathic or viral PPE in children. Idiopathic or viral PPE is most common in male adolescents and is treated infrequently with colchicine., (© 2014 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.)

Objective: The purpose of this study was to examine the physical and psychosocial well-being of children who have had Kawasaki disease (KD), including the influence of coronary artery status on health and health perceptions., Methods: The Child Health Questionnaire (CHQ) measures overall physical and psychosocial well-being in children 5 to 18 years. To study the long-term impact of KD on overall health status, we mailed the CHQ to patients without a history of coronary artery abnormalities (normal group), with regressed aneurysms (regressed group), with current coronary aneurysms <8 mm (mild-moderate aneurysm group), and with giant aneurysms >or=8 mm (giant group)., Results: Of 201 questionnaires mailed, 174 were delivered and 110 (63%) were completed. Median age (range) at completion was 10.5 years (5.1-17.9 years) and at illness onset was 3.1 years (0.2-12 years). There were no significant differences in psychosocial summary scores in any of the Kawasaki groups when compared with the US population sample. Physical summary scores were also similar to the US population sample in the normal coronary, mild-moderate aneurysm, and regressed aneurysm groups. However, the giant aneurysm group had significantly lower physical summary scores compared with the US population sample. Among subscales, general health perceptions in the KD groups were lower than in the US population sample, reaching statistical significance in all but the mild to moderate aneurysm group. In addition, parents whose children have had KD reported a higher proportion of anxiety issues, allergies, and orthopedic/bone/joint issues in their children than did the general US population sample. We did not find any difference in the incidence of attentional, behavioral, or learning issues when compared with the US population sample., Conclusions: KD patients without coronary artery aneurysms were similar to the general population in their general physical and psychosocial health. However, the parents of children in all KD groups reported lower general health perceptions than parents in the US population sample, suggesting that long-term concerns about their children's health exist regardless of overall health status. In addition, children with giant coronary artery aneurysms had lower overall physical summary scores.