Table 2.

Key research foci and questions for systemic JIA investigators^*

Clinical research

Develop classification criteria for DIHS/DRESS‐like reactions in systemic JIA and for SJIA‐LD

Collect HLA class II typing on large, diverse, well‐characterized populations of systemic JIA patients

Translational research

Determine if HHV‐6 (or other viral reactivation) occurs in DIHS/DRESS‐like reactions in systemic JIA

Determine if DIHS/DRESS‐like reactions or SJIA‐LD are associated with prior Aspergillus or EBV exposure or the presence of Akkermansia muciniphila in gut microbiome

Basic research

Determine if IL‐1/IL‐6 blockers activate CD4 T cells in a DRB1*15:XX‐dependent manner

Characterize the Th cell gene/cytokine expression profile of “high‐risk” systemic JIA versus typical systemic JIA

Understand mechanisms driving pulmonary alveolar proteinosis in systemic JIA

^*JIA = juvenile idiopathic arthritis; DIHS = drug‐induced hypersensitivity syndrome; DRESS = drug reaction with eosinophilia and systemic symptoms; SJIA‐LD = systemic JIA–associated lung disease; HHV‐6 = human herpesvirus 6; EBV = Epstein‐Barr virus; IL‐1 = interleukin‐1.