Table 2

Outcomes of patients with antisynthetase associated ILD (ASyS)

Outcome of patients with ASySn=76
Follow-up period, years, median (IQR)3.97 (2.56–5.77)
Patients treated with immunomodulatory therapy (n,%)60 (92%), n=65
Number of drugs used, median (IQR), (range)2 (2–3),(0–5)
Immunomodulatory treatments used, (n)
 Mycophenolate mofetil44
 Cyclophosphamide (IV)22
Interval HRCT imaging, n37, median follow-up 1 year (IQR 1–3.2 years)
Improvement (n,%)12 (32%)
Stable (n,%)11 (30%)
Worsened (n,%)14 (35%)
Change in FVC % predicted at 1 year (%) (median, (IQR))3 (-2–14), n=65
Improvement (n,%)20 (31%)
Stable (n,%)39 (60%)
Worsened (n,%)6 (9%)
Change in TLCO % predicted at 1 year (%) (median, (IQR))0.5 (–4–8), n=52
Improvement (n,%)10 (19%)
Stable (n,%)38 (73%)
Worsened (n,%)4 (8%)
Died during follow-up, n(%)3 (4%), median follow-up 1.64 years (IQR 0.88–5.12)
5 year survival97% (n=30)
  • Disease progression and/or treatment response for patients with ASyS at around 1 year was defined on lung function as a decrease or increase in FVC by more or less than 10% predicted (respectively) compared with baseline, and/or decrease or increase in TLCO by more or less than 15% predicted (respectively), as used in systemic sclerosis.10

  • ASyS, antisynthetase syndrome; FVC, forced vital capacity; ILD, interstitial lung disease; TLCO, transfer factor for carbon monoxide.