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Table 1 (abstract O27). Preliminary definitions of refractory disease courses in sJIA

From: Proceedings of the 27th European Paediatric Rheumatology Congress (PReS 2021)

Description

Proposed definitions

Refractory sJIA arthritis

Arthritis failing to respond to both IL-1 and IL-6 therapy, defined as continued disease activity requiring maintenance therapy with glucocorticoids (GC) (or inability to wean GC)

sJIA-MAS

· sJIA related MAS, requiring maintenance therapy or long term adjunctive therapy with GC

OR

· Recurrent (≥2 episodes) sJIA related MAS

sJIA-LD

· Suspected sJIA-LD: Objective findings on clinical exam (incl. but not limited to tachypnea, cough or clubbing); OR diffuse abnormalities on chest imaging*

· Probable sJIA-LD: Both clinical findings and chest imaging findings as above; OR pulmonary hypertension as measured by echocardiogram

· Definite sJIA-LD: tissue biopsy consistent with ILD, PAP/ELP or PAH

*not due to LD that preexisted sJIA diagnosis, infection or other identifiable cause