Table 3 Proposed definitions of refractory SD
Erkens R et al, Rheum Dis Clin N Am, 2021 [16] | Ambler W et al, Ann Med, 2022 [56] |
|---|---|
1. Refractory sJIA-arthritis: • Patients with sJIA whose arthritis fails to respond to both IL-1 and IL-6 inhibitor therapy, defined as continued arthritis disease activity requiring maintenance therapy with GCs | Refractory sJIA: • Failure to respond to IL-1 and/or IL-6 inhibitors, or; • Need for ongoing treatment with long term GCs (> 6 months) with persistence of systemic and/or arthritic feature |
2. Refractory sJIA-MAS: • sJIA-related MAS, requiring long-term adjunctive therapy with GCs, or; • Recurrent (≥ 2 episodes) sJIA-related MAS | sJIA associated complications: • MAS • LD • Amyloidosis |
3. sJIA-LD: • Suspected sJIA-LD: objective findings on clinical examination (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 in suspected sJIA-LD, or pulmonary hypertension as measured by echocardiogram • Definite sJIA-LD: tissue biopsy consistent with interstitial LD, pulmonary alveolar proteinosis/endogenous lipoid pneumonia, or pulmonary artery hypertension |