Pediatric rheumatology: addressing the transition to adult-orientated health care.

The transition from pediatric to adult health care is often a challenging process due to multiple interwoven complexities, especially for children with chronic medical conditions. Health care transition (HCT) is a process of moving from a pediatric to an adult model of health care with or without a transfer to a new clinician. This paper focuses on what is known about HCT for youth and young adults (Y/YA) with rheumatic diseases within a larger context of HCT recommendations. HCT barriers for youth, families, and providers and current evidence for a structured HCT processes are reviewed. Practical advice is offered on how to approach transition for Y/YA, what tools are available to assist in a successful transition process, and what are the areas of future research that are needed to improve the HCT evidence base

Galactorrhea associated with juvenile systemic lupus erythematosus: a review of the role of prolactin

This case report is based on the clinical observation of a patient with juvenile systemic lupus erythematosus (SLE) who developed transient galactorrhea. The subsequent literature review documented an interesting association between prolactin and rheumatic diseases and in particular, hyperprolactinemia and SLE. The discussion that follows the case report explores this relationship and proposes a hypothesis regarding why this patient with juvenile SLE developed galactorrhea

Infliximab versus second intravenous immunoglobulin for treatment of resistant Kawasaki disease in the USA (KIDCARE): a randomised, multicentre comparative effectiveness trial

Background Although intravenous immunoglobulin (IVIG) is effective therapy for Kawasaki disease, 10–20% of patients have recrudescent fever as a sign of persistent inflammation and require additional treatment. We aimed to compare infliximab with a second infusion of IVIG for treatment of resistant Kawasaki disease. Methods In this multicentre comparative effectiveness trial, patients (aged 4 weeks to 17 years) with IVIG resistant Kawasaki disease and fever at least 36 h after completion of their first IVIG infusion were recruited from 30 hospitals across the USA. Patients were randomly assigned (1:1) to second IVIG (2 g/kg over 8–12 h) or intravenous infliximab (10 mg/kg over 2 h without premedication), by using a randomly permuted block randomisation design with block size of two or four. Patients with fever 24 h to 7 days following completion of first study treatment crossed over to receive the other study treatment. The primary outcome measure was resolution of fever at 24 h after initiation of study treatment with no recurrence of fever attributed to Kawasaki disease within 7 days post-discharge. Secondary outcome measures included duration of fever from enrolment, duration of hospitalisation after randomisation, and changes in markers of inflammation and coronary artery Z score. Efficacy was analysed in participants who received treatment and had available outcome values. Safety was analysed in all randomised patients who did not withdraw consent. This clinical trial is registered with ClinicalTrials.gov, NCT03065244. Findings Between March 1, 2017, and Aug 31, 2020, 105 patients were randomly assigned to treatment and 103 were included in the intention-to-treat population (54 in the infliximab group, 49 in the second IVIG group). Two patients randomised to infliximab did not receive allocated treatment. The primary outcome was met by 40 (77%) of 52 patients in the infliximab group and 25 (51%) of 49 patients in the second IVIG infusion group (odds ratio 0·31, 95% CI 0·13–0·73, p=0·0076). 31 patients with fever beyond 24 h received crossover treatment: nine (17%) in the infliximab group received second IVIG and 22 (45%) in second IVIG group received infliximab (p=0·0024). Three patients randomly assigned to infliximab and two to second IVIG with fever beyond 24h did not receive crossover treatment. Mean fever days from enrolment was 1·5 (SD 1·4) for the infliximab group and 2·5 (2·5) for the second IVIG group (p=0·014). Mean hospital stay was 3·2 days (2·1) for the infliximab group and 4·5 days (2·5) for the second IVIG group (p<0·001). There was no difference between treatment groups for markers of inflammation or coronary artery outcome. 24 (44%) of 54 patients in the infliximab group and 33 (67%) of 49 in the second IVIG group had at least one adverse event. A drop in haemoglobin concentration of at least 2g/dL was seen in 19 (33%) of 58 patients who received IVIG as either their first or second study treatment (three of whom required transfusion) and in three (7%) of 43 who received only infliximab (none required transfusion; p=0·0028). Haemolytic anaemia was the only serious adverse events deemed definitely or probably related to study treatment, and was reported in nine (15%) of 58 patients who received IVIG as either their first or second study treatment and none who received infliximab only. Interpretation Infliximab is a safe, well tolerated, and effective treatment for patients with IVIG resistant Kawasaki disease, and results in shorter duration of fever, reduced need for additional therapy, less severe anaemia, and shorter hospitalisation compared with second IVIG infusion

Telemedicine use by pediatric rheumatologists during the COVID-19 pandemic.

BACKGROUND: To characterize various aspects of telemedicine use by pediatric rheumatology providers during the recent pandemic including provider acceptability of telehealth practices, clinical reliability, and clinical appropriateness. METHODS: An electronic survey was generated and disseminated amongst the Childhood Arthritis and Rheumatology Research Alliance (CARRA) listserv (n = 547). Survey items were analyzed via descriptive statistics by question. RESULTS: The survey response rate was 40.8% (n = 223) with the majority of respondents in an attending-level role. We observed that musculoskeletal components of the exam were rated as the most reliable components of a telemedicine exam and 86.5% of survey respondents reported engaging the patient or patient caregiver to help conduct the virtual exam. However, 65.7% of providers reported not being able to elicit the information needed from a telemedicine visit to make a complete clinical assessment. We also noted areas of disagreement regarding areas of patient engagement and confidentiality. We found that approximately one-third (35.8%) of those surveyed felt that their level of burnout was increased due to telemedicine. CONCLUSION: In general, providers found exam reliability (specifically around focused musculoskeletal elements) in telemedicine visits but overall felt that they were unable to generate the information needed to generate a complete clinical assessment. Additionally, there were suggestions that patient engagement and confidentiality varied during telemedicine visits when compared to in-person clinical visits. Further qualitative work is needed to fully explore telemedicine use in pediatric rheumatology. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s12969-021-00565-7

Telemedicine use by pediatric rheumatologists during the COVID-19 pandemic

Abstract Background To characterize various aspects of telemedicine use by pediatric rheumatology providers during the recent pandemic including provider acceptability of telehealth practices, clinical reliability, and clinical appropriateness. Methods An electronic survey was generated and disseminated amongst the Childhood Arthritis and Rheumatology Research Alliance (CARRA) listserv (n = 547). Survey items were analyzed via descriptive statistics by question. Results The survey response rate was 40.8% (n = 223) with the majority of respondents in an attending-level role. We observed that musculoskeletal components of the exam were rated as the most reliable components of a telemedicine exam and 86.5% of survey respondents reported engaging the patient or patient caregiver to help conduct the virtual exam. However, 65.7% of providers reported not being able to elicit the information needed from a telemedicine visit to make a complete clinical assessment. We also noted areas of disagreement regarding areas of patient engagement and confidentiality. We found that approximately one-third (35.8%) of those surveyed felt that their level of burnout was increased due to telemedicine. Conclusion In general, providers found exam reliability (specifically around focused musculoskeletal elements) in telemedicine visits but overall felt that they were unable to generate the information needed to generate a complete clinical assessment. Additionally, there were suggestions that patient engagement and confidentiality varied during telemedicine visits when compared to in-person clinical visits. Further qualitative work is needed to fully explore telemedicine use in pediatric rheumatology

Clinical outcomes and safety of anakinra in the treatment of multisystem inflammatory syndrome in children: a single center observational study

Abstract Background and objective Evidence for the treatment of multisystem inflammatory syndrome in children (MIS-C) is lacking. Anakinra, which targets IL-1-mediated inflammation, is reserved for refractory cases of MIS-C; however, its use in the treatment of MIS-C is not clearly established. Patients and methods To examine a role for anakinra in MIS-C, we performed a single center observational cohort study of all MIS-C patients diagnosed at our children’s hospital from May 15 to November 15, 2020. Demographics, clinical features, diagnostic testing, and cardiac function parameters were compared between MIS-C patients treated with intravenous immunoglobulin (IVIG) monotherapy and IVIG with anakinra (IVIG + anakinra). Results Among 46 patients with confirmed MIS-C, 32 (70%) were in the IVIG + anakinra group, of which 9 (28%) were also given corticosteroids (CS). No patients were treated with anakinra alone. MIS-C patients in the IVIG + anakinra group were enriched in a CV shock phenotype (p = 0.02), and those with CV shock were treated with higher doses of anakinra for a longer duration. Furthermore, MIS-C patients in the IVIG + anakinra group exhibited improvements in fever and cardiac function with or without CS. No significant adverse events were observed, and no differences in IL-1β levels were found among MIS-C patients in the IVIG + anakinra group. Conclusions Anakinra treatment, which was co-administered with IVIG primarily in patients with severe MIS-C, was associated with improvements in fever and cardiac function, and demonstrated a favorable side-effect profile. These findings suggest a role for adjunctive anakinra in the treatment of severe MIS-C