DomainActivity levelDescription
Pulmonary
Rate as ‘No activity’ stable long-lasting features related to damage, or respiratory involvement not related to the disease (tobacco use, etc)
No=0Absence of currently active pulmonary involvement
Low=5Persistent cough due to bronchial involvement with no radiographic abnormalities on radiography Or radiological or HRCT evidence of interstitial lung disease with: no breathlessness and normal lung function test
Moderate=10Moderately active pulmonary involvement, such as interstitial lung disease shown by HRCT with shortness of breath on exercise (NHYA II) or abnormal lung function tests restricted to: 70% >DLCO ≥40% or 80% >FVC≥60%
High=15Highly active pulmonary involvement, such as interstitial lung disease shown by HRCT with shortness of breath at rest (NHYA III, IV) or with abnormal lung function tests: DLCO <40% or FVC <60%
  • FVC, forced vital capacity; HRCT, high-resolution CT; NYHA, New York Heart Association.