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. Author manuscript; available in PMC: 2021 Feb 1.
Published in final edited form as: Semin Arthritis Rheum. 2019 Jun 28;50(1):149–155. doi: 10.1016/j.semarthrit.2019.06.014

Table 1:

Demographics and clinical features of 49 adult patients with juvenile idiopathic inflammatory myopathies

Clinical Diagnosis, %
 Juvenile dermatomyositis 75.5
 Juvenile polymyositis 10.2
 Juvenile connective tissue myositis (JCTM) 14.3
Age, years, median [IQR] 24.0 [19.0-28.6]
Race, %
 Caucasian 59.2
 African American 11.1
 Hispanic 17.7
 Othera 12.0
Female sex, % 81.6
Age at diagnosis, years, median [IQR] 11.0 [8.5-15.7]
Disease duration at cross-sectional evaluation, years, median [IQR] 11.5 [4.5-18.9]
Delay to diagnosis, months, median [IQR] 6.0 [2.3-13.7]
Any myositis specific autoantibodiesb, % 67.5
 Anti-p155/p140 (TIF-1) 27.5
 Anti-MJ (NXP2) 22.5
 Anti-Jo1 7.5
 Anti-MDA5 5.0
 Anti-SRP 5.0
Any myositis associated autoantibodiesb, % 40.0
 Anti-Ro60 20.0
 Anti-Ro52 17.1
 Anti-U1-RNP 12.5
 Otherc 7.5
Severity at onset Likert scale (0-4), median [IQR] 1.0 [1.0-2.0]
Disease Course, %
 Chronic continuous 63.3
 Polycyclic 30.6
 Monocyclic 6.1
Height percentile at cross-sectional evaluation 30.7 [10.0-60.1]
Current physician global activity score 10 cm VAS, median [IQR] 1.6 [1.0-3.7]
Current physician global damage score 10 cm VAS, median [IQR] 3.0 [2.0-4.4]
Current HAQ score, median [IQR] 0.4 [0.0-1.0]
Current MMT26 score, 0-260d, median [IQR] 229.0 [212.6-256.8]
ACR functional class, worst ever (1-4), median [IQR] 4.0 [3.0-4.0]
ACR functional class at cross-sectional evaluation (1-4), median [IQR] 2.0 [1.5-3.0]
Maximal number of medications ever used, median [IQR] 5.0 [3.0-8.0]
 Prednisone ever used, % 100
 Methotrexate ever used, % 86.0
 IV methylprednisolone ever used, % 60.5
 IVIG ever used,% 60.5
 DMARDs ever usede, % 59.1
 Biologic therapy ever usedf, % 31.8
 Cyclophosphamide ever used, % 17.5
Number of medications at cross-sectional evaluation, median [IQR] 3.0 [1.0-4.0]
 Prednisone at cross-sectional evaluation, % 56.5
 Methotrexate at cross-sectional evaluation, % 39.1
 IVIG at cross-sectional evaluation, % 38.6
 DMARDs at cross-sectional evaluation, % 32.6
 IV methylprednisolone at cross-sectional evaluation, % 15.6
 Biologic therapy at cross-sectional evaluation, % 9.1
 Cyclophosphamide at cross-sectional evaluation, % 2.3
a

Race was self-reported. “Other” includes Indian (n = 1), Yemeni (n = 1), Korean (n = 1), and Not Reported (n = 4).

b

All autoantibodies were tested in 40 patients, except Anti-Ro52, which was tested in 35 patients.

c

Other MAAs included anti-PM/Scl (2.5%), anti-La (2.5%), and anti-Smith (2.5%). 10%> of patients had multiple MAAs. The MSA anti-Mi-2 was not present in this cohort.

d

MMT26 scores were available in 47 patients

e

DMARDS included azathioprine (n = 14), cyclosporine (n = 12), mycophenolate mofetil (n = 13), and tacrolimus (n = 2).

f

Biologics included abatacept (n= 2), adalimumab (n = 1), anakinra (n = 1), etanercept (n = 3), infliximab (n = 2), and rituximab (n = 11).

Abbreviations: ACR: American College of Rheumatology; DMARDs: Disease-Modifying Anti-Rheumatic Drugs; HAQ: Health Assessment Questionnaire; IQR: interquartile range; IV: intravenous; IVIG: intravenous immunoglobulin; JCTM: Juvenile connective tissue myositis; MMT26: Manual Muscle Testing-26; VAS: visual analog scale