Outcome of patients with ASyS | n=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) | |
Prednisolone | 58 |
Azathioprine | 10 |
Mycophenolate mofetil | 44 |
Methotrexate | 3 |
Cyclophosphamide (IV) | 22 |
Rituximab | 6 |
Interval HRCT imaging, n | 37, 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 survival | 97% (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.