Table 2

Environmental exposures, extramuscular complications and disease severity assessments of cases in the EuroMyositis Registry

DermatomyositisPolymyositisAntisynthetase syndromeConnective tissue disease-overlap myositisInclusion body myositisImmune- mediated necrotising myopathyJuvenile dermatomyositisTotal
n (%)
Environmental exposures—% per diagnosis
Current or previous smoker (n=1646)33394245352920611(37)
Environmental toxin exposure (n=930)161721152840157 (17)
Extramuscular complications— % per diagnosis
Interstitial lung disease (n=2442)211771323106720 (30)
Cardiac involvement (n=1715)9911124107156 (9)
Malignancy ever (n=2788)
Of these—% with each type*:
20812131670374 (13)
 Breast2223171681419%
 Bowel87121421010%
 Ovarian13223007%
 Lung107149009%
 Other52636159728658%
‘Cancer -associated myositis’† (n=2701)933353132 (5)
Dysphagia (n=1945)43352653503616767 (39)
Disease severity assessments at last visit— median (IQR), nMedian (IQR), n
Duration of follow-up in years‡2.6 (1.5–6.1), 1862.8 (1.3–5.6), 1243.2 (1.8–6.5), 1233.8 (2.1–6.6), 913.3 (1.5–5.9), 332.2 (0.7–5.6), 231.3 (0.5–7.1), 163.0 (1.5–6.1), 596
MMT-8 score (0–80)75 (64–79), 15672 (61–78), 8975 (69–80), 9371 (59–77), 5563 (53–70), 2169 (57–77), 2178 (70–80), 773 (63–79), 442
Physician-completed global disease activity VAS (0–100)10 (1–26), 25810 (2–26), 1569 (1–24), 1638 (1–27), 10714 (4–29), 3916 (10–53), 3610 (0–23), 1310 (1–26), 772
Patient-completed global disease activity VAS (0–100)30 (5–55), 20345 (28–55), 10340 (10–55), 12347 (22–64), 8344 (24–67), 3242 (10–60), 303 (1–16), 1340 (11–57), 587
HAQ-DI (0–3)0.50 (0–1.25), 2390.88 (0.25–1.50), 1320.63 (0–1.25), 1420.88 (0.38–1.50), 981.82 (1.38–2.50), 380.56 (0.13–2.13), 260 (0–0.13), 170.75 (0.13–1.50), 692
Creatine kinase (as ratio of ULN)0.44 (0.29–0.97), 631.12 (0.45–3.20), 620.63 (0.35–2.06), 400.66 (0.31–1.90), 191.79 (1.17–2.02), 51.28 (0.63–2.79), 80.56 (0.39–1.85), 110.63 (0.37–1.78), 208
Extramuscular disease activity VAS (0–100)7 (0–22), 2374 (0–15), 1418 (0–18), 1496 (0–16), 1000 (0–15), 350 (0–15), 2912 (0–27), 115 (0–18), 702
Myositis Damage Index global VAS (0–100)16 (3–34), 14620 (5–38), 8419 (8–34), 8526 (8–36), 5847 (39–62), 2214 (3–40), 1829 (11–41), 820 (6–38), 421
  • *Multiple malignancies were recorded in some cases, therefore total may exceed 100%.

  • †Malignancy diagnosed within 3 years of the idiopathic inflammatory myopathy diagnosis.

  • ‡For cases with >1 visit with any disease severity assessment recorded. Environmental toxin exposure includes exposure to asbestos, silica, fibreglass, solvents or coal dust.

  • HAQ-DI, Health Assessment Questionnaire-Disability Index; VAS, visual analogue scale; MMT-8, manual muscle test-8 score; ULN, upper limit of normal.