Improving Hepatitis B Screening Prior to Rituximab: A Quality Improvement Project.

Rituximab, used in the treatment of some rheumatic and kidney diseases, can lead to hepatitis B virus (HBV) reactivation; HBV screening is recommended for those starting this medication. We aimed to improve by 50% the proportion of patients undergoing HBV screening by implementing multimodal interventions to support clinicians in this evidence-based practice. We conducted a quality improvement project from November 2020 to June 2022 at a tertiary care pediatric hospital system, including patients with rheumatic and/or kidney diseases starting rituximab. Multimodal interventions targeting clinicians included electronic health tools (dot phrase, display of screening recommendations and screening results in rituximab order sets/therapy plans), educational meetings, and e-mail/paper reminders. The primary outcome was the proportion of patients with complete HBV screening, while the secondary outcome was utilization of each laboratory component, tracked using statistical process control charts. Pre- and post-intervention data were compared using Fisher's test. One hundred eighty-two patients who had been prescribed rituximab were included, of which 98 (54%) were post-intervention. The proportions of patients undergoing complete HBV screening (6% vs. 44%; p < 0.001), HBsAg collection (60% vs. 79%; p = 0.006), anti-HBsAb collection (14% vs. 54%; p < 0.001), and total anti-HBcAb collection (8% vs. 52%; p < 0.001) were significantly higher in the post-intervention period. Improvement was sustained over 18 months, with shifts and/or data points above the control limits in all measures. Forty-five patients were HBV-non-immune. In this study, multimodal interventions including electronic health tools and education of the provider significantly increased the proportion of patients screened for HBV prior to rituximab and identified immunization opportunities.

Impact of follow-up adherence on disease activity in childhood-onset systemic lupus erythematosus (cSLE).

BACKGROUND/PURPOSE: Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease, with a potential for significant disease damage, morbidity, and mortality. In comparison to the adult population, childhood-onset SLE (cSLE) tends to be more aggressive given the higher preponderance of renal and neuropsychiatric disease and increased disease activity. There is a paucity of literature examining relationship between disease activity, rheumatology follow-up visits, and health care utilization. The objective of this study is to determine whether adherence with outpatient clinic visits would affect disease activity in patients with childhood-onset systemic lupus erythematosus (cSLE). METHODS: 41 children <18 years of age at time of diagnosis with SLE who met Systemic Lupus International Collaborative Clinics (SLICC) criteria and not evaluated in clinic within the previous 120-day period were identified as eligible for inclusion. Patients were continuously searched between December 2021 and July 2022 for eligibility evaluation. Through retrospective chart review, we assessed disease activity (SLE Disease Activity Index) at the last clinic visit. The patients were stratified into two cohorts of lower and higher disease activity, with SLE disease activity index (SLEDAI) ≤ 3 and SLEDAI ≥ 4, respectively. Descriptive statistics and Willcox Rank Sum (numerical variables) and Fisher's test (categorical variables) were used to compare these two groups. RESULTS: Clinical, epidemiological, and serological data were compared between the two groups, with observed statistically significant differences to include current use of high dose prednisone associated with higher SLEDAI scores (p = 0.019). In nonparametric analysis, time to follow-up (p < 0.001), hospitalizations (p = 0.017), and Emergency Department visits (ED) (p < 0.001) were found to be associated with higher SLEDAI scores. CONCLUSION: Our findings suggest that cSLE patients with higher disease activity are at risk for increased health care utilization with respect to ED visits as well as hospitalizations in the setting of follow-up nonadherence. While further studies are required to enhance our understanding of this association, this links the importance of disease-related outcome and routine outpatient visits in this particularly vulnerable patient population.

Neurosarcoidosis in children: A systematic review and summary of cases, imaging and management.

Neurosarcoidosis is a rare disorder in children. We identified 30 pediatric NS cases through a systematic review. Twenty-one (70%) had systemic sarcoidosis with 30% having primary neurosarcoidosis. Eyes (37%), lymph nodes (37%) and lungs (30%) were most commonly involved. Isolated neurosarcoidosis were more likely in children (30%) than in adults (6%, p = 0.0005). Seizures and optic neuritis were also more common in children than adults (33% vs 14%, p = 0.002; and 30% versus 6%, p = 0.008, respectively). Evaluation, imaging, laboratory findings, and treatments are discussed. Additional research, including multi-center studies, is needed.

Giant Coronary Aneurysms in Multisystem Inflammatory Syndrome in Children Associated With SARS-CoV-2 Infection.

Multisystem inflammatory syndrome in children (MIS-C) can cause a myriad of cardiac manifestations, including coronary dilation and aneurysms; giant aneurysms are infrequent. We describe 3patients with giant coronary aneurysms associated with MIS-C, including the youngest case reported to date, treated with intravenous immunoglobulin, corticosteroids, and biologic agents. (Level of Difficulty: Intermediate.).

Acute Hemorrhagic Infancy of Edema: A Purpuric Rash in 6-Month-Old Infant.

Acute hemorrhagic edema of infancy (AHEI) is a cutaneous leukocytoclastic vasculitis presenting with purpura, ecchymosis, fever, and edema. Pediatricians must effectively differentiate AHEI from other diseases that have similar presentations but are more severe in order to treat appropriately without overutilizing expensive, unnecessary diagnostic tests. In this article, we describe a case of a 6-month-old previously well male who presented to our institution with a worsening rash and fever. In this case, the patient's age and benign clinical manifestations without systemic involvement favored the diagnosis of AHEI over more serious conditions. This case is a valuable example of the clinical findings of AHEI and the effectiveness of conservative therapy once a diagnosis is made for pediatricians, especially emergency and urgent care physicians.

Pituitary Sarcoidosis in a Pediatric Patient Successfully Treated With Adalimumab and Methotrexate.

Neurosarcoidosis is a rare phenomenon in the pediatric population, with only a few cases reported in the literature worldwide. While hypothalamo-pituitary involvement is known to occur, direct infiltration of the pituitary gland and isolated anterior pituitary dysfunction without diabetes insipidus is seldom observed. A high index of suspicion is required for diagnosis of neurosarcoidosis, and treatment can be challenging due to lack of standardized guidelines. We present the case of a 17-year-old female with known sarcoidosis of the lacrimal glands, who developed severe headache and neurologic symptoms secondary to granulomatous infiltration of the pituitary gland and infundibulum due to neurosarcoidosis. She was successfully treated with corticosteroids, methotrexate, and adalimumab, with complete radiologic resolution. This is the first documented pediatric case of neurosarcoidosis with radiologic granulomatous infiltration of the pituitary gland, manifesting as partial anterior hypopituitarism, in the form of central hypothyroidism, without diabetes insipidus.

Direct Healthcare Costs Associated with Oligoarticular Juvenile Idiopathic Arthritis at a Single Center.

Oligoarticular juvenile idiopathic arthritis (JIA) is a common disease in pediatric rheumatology. The management of oligoarticular JIA can result in a considerable economic burden. This study is a four-year, retrospective cost identification analysis performed to determine the annual direct cost of care for patients with oligoarticular JIA and possible predictive clinical factors. Direct healthcare costs were defined as those associated with office visits, laboratory studies, hospital admissions, joint injections, medications, infusions, radiology tests, and emergency room visits. Disease characteristics and patient information included ANA status, gender, age at diagnosis, duration from diagnosis to initial visit during the study period, and whether uveitis had been diagnosed. We identified 97 patients with oligoarticular JIA eligible for the study. The median age of diagnosis was 4.3 years. Positive ANA were noted in 75% of patients. 34% of patients received at least one intra-articular steroid injection. 32% of patients were prescribed a biologic during the study period, predominantly with other medications, while 23% of patients received only NSAIDs. 20% of patients were prescribed oral steroids. The average total direct medical cost in this study per year for an oligoarticular JIA patient was $3929 ± 6985. Medications accounted for 85% of annual direct medical costs. Clinic visits and laboratory testing accounted for 8% and 5%, respectively. Patient characteristics and demographics were tested for association with direct medical costs by the Wilcoxon rank sum test and Kruskal-Wallis test. Patients who were ANA positive had increased annual costs compared to patients who are ANA negative. ANA-positive patients were found to have statistically significant costs, particularly, in laboratory tests, procedural costs, radiology costs, and medication costs. The results reported here provide information when allocating healthcare resources and a better understanding of the economic impact oligoarticular JIA has on the United States healthcare system.

An Update on Treatment and Management of Pediatric Systemic Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic multisystem autoimmune disorder in which 20 % of patients are diagnosed in childhood. Childhood-onset SLE is associated with higher morbidity and mortality than adult-onset SLE. The aims of disease management with early immunosuppression are to decrease disease activity and improve quality of life. A multidisciplinary approach is necessary due to the complexity of lupus in pediatric patients. It is important to provide patients with high quality of care and to instill ownership of their disease process from a young age to prepare them to manage this life-long illness. This article reviews current management of SLE in children.