Interleukin (IL)-1/IL-6-Inhibitor-Associated Drug Reaction With Eosinophilia and Systemic Symptoms (DReSS) in Systemic Inflammatory Illnesses.

Background: After introducing IL-1/IL-6 inhibitors, some patients with Still and Still-like disease developed unusual, often fatal, pulmonary disease. This complication was associated with scoring as DReSS (drug reaction with eosinophilia and systemic symptoms) implicating these inhibitors, although DReSS can be difficult to recognize in the setting of systemic inflammatory disease.

Objective: To facilitate recognition of IL-1/IL-6 inhibitor-DReSS in systemic inflammatory illnesses (Still/Still-like) by looking at timing and reaction-associated features.

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.

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.

Successful Medical-Surgical Management of Intracardiac Thrombosis and Pulmonary Pseudoaneurysms in an Adolescent With Hughes-Stovin Syndrome.

We present an adolescent male with a single intracardiac mass and pulmonary emboli, complicated by peripheral venous thrombosis and subsequent development of pulmonary pseudoaneurysms, leading to diagnosis of Hughes-Stovin syndrome. Remission was achieved with cyclophosphamide, corticosteroids, and pseudoaneurysm resection and maintained with infliximab and methotrexate.

Postdischarge Glucocorticoid Use and Clinical Outcomes of Multisystem Inflammatory Syndrome in Children.

Importance: Minimal data are available regarding the postdischarge treatment of multisystem inflammatory syndrome in children (MIS-C).

Objectives: To evaluate clinical characteristics associated with duration of postdischarge glucocorticoid use and assess postdischarge clinical course, laboratory test result trajectories, and adverse events in a multicenter cohort with MIS-C.

Design, Setting, And Participants: This retrospective cohort study included patients with MIS-C hospitalized with severe illness and followed up for 3 months in an ambulatory setting.