Table 1 Definitions of refractory SJIA
Clinical phenotype | Proposed definition | Reference |
|---|---|---|
Refractory SJIA (broadly) | Failure to respond to IL-1 AND IL-6 blocking biologics (failure = inability to resolve arthritis, systemic symptoms, or liver dysfunction, or being glucocorticoid dependent) OR ≥ 2 episodes of MAS in a 2 year period OR Development of SJIA-LD | Canna et al. (2020) [7] |
Persistent partial MAS | A patient with active SJIA and persistent inflammation (provided that infection or other causes have been ruled out) and newly worsening or persistently abnormal values for at least 6 weeks (with inability to taper medications because of worsening values) indicating: - Liver abnormalities - Disorder of hematopoiesis - Coagulopathy - Highly elevated serum IL-18 with modestly elevated CXCL9 | Canna et al. (2020) [7] |
Refractory SJIA arthritis | SJIA patients whose arthritis fails to respond to both IL-1 and IL-6 therapy, defined as continued arthritis disease activity requiring maintenance therapy with glucocorticoids | Erkens et al. (2021) [8] |
Refractory/recurrent SJIA-associated MAS | SJIA related MAS, requiring long term adjunctive therapy with glucocorticoids, OR Recurrent (≥ 2 episodes of) SJIA related MAS | Erkens et al. (2021) [8] |
SJIA-LD | Suspected SJIA-LD: Objective findings on clinical exam (including 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 lung disease that preexisted SJIA diagnosis, infection, or other identifiable cause. | Erkens et al. (2021)Â [8] |