Variation of Diagnostic Approaches and Treatment Practices for Hemophagocytic Lymphohistiocytosis/Macrophage Activation Syndrome Among Pediatric Subspecialists.

Objective: To assess the diagnostic and treatment practices among a variety of subspecialists at pediatric institutions in the US.

Study Design: Using a web-based survey, we assessed the consultation, diagnostic, and treatment preferences of providers from the different pediatric subspecialties who care for pediatric patients with hemophagocytic lymphohistiocytosis (HLH)/macrophage activating syndrome (MAS). Domains included demographics, provider training level and specialty, experience and comfort level with the diagnosis and treatment of HLH/MAS, and institutional approaches toward the diagnosis and management of HLH/MAS.

Modified Juvenile Spondyloarthritis Disease Activity Index in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry.

Objective: To validate the Juvenile Spondyloarthritis Disease Activity Index (JSpADA), and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA).

Methods: We utilized the Childhood Arthritis and Rheumatology Research Alliance Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no C-reactive protein [CRP]/erythrocyte sedimentation rate [ESR]; JSpADA7-no Schober; and JSpADA7-no CRP/ESR) using the Outcome Measures in Rheumatology principles of face validity, discriminative validity, and responsiveness to change.

Results: There were 51 subjects (64 visits) with complete JSpADA data with a mean age of 13.

Mental Health in Pediatric Rheumatology: An Opportunity to Improve Outcomes.

Mental health problems are more common in children with pediatric rheumatologic diseases (PRDs) than healthy peers. Mental health problems affect disease-related outcomes and health-related quality of life (HRQOL), so addressing these problems can improve clinical and psychosocial outcomes. Mental health screening tools are available, and there are resources available to aid in integrating mental health care into the clinical setting.

Development and Validation of a Juvenile Spondyloarthritis Disease Flare Measure: Ascertaining Flare in Patients With Inactive Disease.

Objective: Our objective was to develop and validate a composite disease flare definition for juvenile spondyloarthritis (SpA) that would closely approximate the clinical decision made to reinitiate or not reinitiate systemic therapy after therapy de-escalation.

Methods: Retrospective chart reviews of children with SpA who underwent systemic therapy de-escalation of biologic or conventional disease-modifying antirheumatic drugs were used to develop and validate the flare outcome. Data on independent cohorts for development (1 center) and validation (4 centers) were collected from large tertiary health care systems.

Advances in Juvenile Spondyloarthritis.

Purpose: To provide an overview of recent studies on pathogenesis, diagnosis, and management of juvenile spondyloarthritis (JSpA).

Recent Findings: Recent studies show differences in gut microbiome in patients with JSpA in comparison to healthy controls. There is increased recognition of the impact of the innate immune system on disease pathology.

Childhood Sjögren syndrome: features of an international cohort and application of the 2016 ACR/EULAR classification criteria.

Objective: Sjögren syndrome in children is a poorly understood autoimmune disease. We aimed to describe the clinical and diagnostic features of children diagnosed with Sjögren syndrome and explore how the 2016 ACR/EULAR classification criteria apply to this population.

Methods: An international workgroup retrospectively collected cases of Sjögren syndrome diagnosed under 18 years of age from 23 centres across eight nations.