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)
Breast 22231716814 19%
Bowel 871214210 10%
Ovarian 1322300 7%
Lung 10714900 9%
Other 526361597286 58%
‘Cancer -associated myositis’† (n=2701)933353132 (5)
Dysphagia (n=1945)43352653503616767 (39)
Disease severity assessments at last visit— median (IQR), n Median (IQR), n
Duration of follow-up in years‡2.6 (1.5–6.1), 186 2.8 (1.3–5.6), 124 3.2 (1.8–6.5), 123 3.8 (2.1–6.6), 91 3.3 (1.5–5.9), 33 2.2 (0.7–5.6), 23 1.3 (0.5–7.1), 16 3.0 (1.5–6.1), 596
MMT-8 score (0–80)75 (64–79), 156 72 (61–78), 89 75 (69–80), 93 71 (59–77), 55 63 (53–70), 21 69 (57–77), 21 78 (70–80), 7 73 (63–79), 442
Physician-completed global disease activity VAS (0–100)10 (1–26), 258 10 (2–26), 156 9 (1–24), 163 8 (1–27), 107 14 (4–29), 39 16 (10–53), 36 10 (0–23), 13 10 (1–26), 772
Patient-completed global disease activity VAS (0–100)30 (5–55), 203 45 (28–55), 103 40 (10–55), 123 47 (22–64), 83 44 (24–67), 32 42 (10–60), 30 3 (1–16), 13 40 (11–57), 587
HAQ-DI (0–3)0.50 (0–1.25), 239 0.88 (0.25–1.50), 132 0.63 (0–1.25), 142 0.88 (0.38–1.50), 98 1.82 (1.38–2.50), 38 0.56 (0.13–2.13), 26 0 (0–0.13), 17 0.75 (0.13–1.50), 692
Creatine kinase (as ratio of ULN)0.44 (0.29–0.97), 63 1.12 (0.45–3.20), 62 0.63 (0.35–2.06), 40 0.66 (0.31–1.90), 19 1.79 (1.17–2.02), 5 1.28 (0.63–2.79), 8 0.56 (0.39–1.85), 11 0.63 (0.37–1.78), 208
Extramuscular disease activity VAS (0–100)7 (0–22), 237 4 (0–15), 141 8 (0–18), 149 6 (0–16), 100 0 (0–15), 35 0 (0–15), 29 12 (0–27), 11 5 (0–18), 702
Myositis Damage Index global VAS (0–100)16 (3–34), 146 20 (5–38), 84 19 (8–34), 85 26 (8–36), 58 47 (39–62), 22 14 (3–40), 18 29 (11–41), 8 20 (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.