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 |