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| | JIA | JSLE | JDM |
|
| Relative disease activity measures |
| Core response variables (CRV) | ACR [10]:
(1) Physician global assessment of disease activity
(2) Parent/patient assessment of overall wellbeing
(3) Functional ability (CHAQ)
(4) Number of joints with active arthritis
(5) Number of joints with limited range of motion
(6) ESR
For SJIA patients: absence of spiking fever (≤38°C during past week) | PRINTO [13]:
(1) Physician global assessment of disease activity
(2) Parent/patient assessment of overall wellbeing
(3) Renal involvement: 24-hour proteinuria
(4) Global disease activity tool (SLEDAI, ECLAM, or SLAM)
(5) HRQL (CHQ) | PRINTO [14]:
(1) Physician global assessment of disease activity
(2) Parent/patient assessment of overall wellbeing
(3) Muscle strength (CMAS, MMT)
(4) Functional ability (CHAQ)
(5) Global disease activity tool (DAS, MDAAT)
(6) HRQL (CHQ)
IMACS [12]:
(1) Physician global assessment of disease activity
(2) Muscle strength (MMT)
(3) Functional ability (CHAQ, CMAS)
(4) Muscle enzymes (≥2 of CPK, aldolase LDH, AST, ALT)
(5) Extramuscular disease (MDAAT) |
| Definition of improvement | ≥30% improvement from baseline in 3 of 6 CRVs, with ≤1 CRV worsening by >30% [10, 29] | ≥50% improvement from baseline in 2 of 5 CRVs, with ≤1 CRV worsening by >30% [30] | PRINTO [31]:
≥20% improvement in 3 of 6 CRVs, with ≤2 worsening by ≥20% (muscle strength excluded)
IMACS [32]:
≥15% improvement in muscle strength and physical function, ≥20% physician and patient global and extramuscular assessments, ≥30% improvement in muscle enzymes |
| Definition of flare | Worsening of 2 CRV by ≥40% without improvement in >1 CRV by ≥30% [33] | | |
|
| Absolute disease activity measures |
| Global disease activity tools | JADAS [6, 34]:
(1) Physician global assessment of disease activity
(2) Parent/patient global assessment of wellbeing
(3) Count of joints with active disease
(4) ESR | SLEDAI [35]
BILAG [36]
SLAM [37]
ECLAM [38] | DAS [39]
MDAAT [40]
CAT [41] |
| Minimal disease activity (MDA) | MDA [42]:
(1) Oligoarthritis: physician global assessment ≤2.5 cm and swollen joint count of 0
(2) Polyarthritis: physician global assessment ≤3.4 cm, parent global assessment ≤2.1 cm, and swollen joint count ≤1 | | |
| Inactive disease/remission | Wallace criteria [15, 43]
Inactive disease:
(1) No joints with active arthritis
(2) No fever, rash, serositis, splenomegaly, or generalized lymphadenopathy attributable to JIA
(3) No active uveitis
(4) Normal ESR and/or CRP | | PRINTO [44]:
Inactive disease on or off medication, ≥3/4 of:
(1) CPK ≤ 150
(2) CMAS ≥ 48
(3) MMT ≥ 78
(4) Physician global assessment ≤ 0.2 |
| | (5) Physician global assessment indicates no disease activity
Clinical remission:
(1) On medication—criteria for inactive disease met for minimum 6 continuous months while patient on medication
(2) Off medication—criteria for active disease met for minimum 12 continuous months while off all arthritis and uveitis medications | | |
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