Patient selection: interstitial lung disease associated with scleroderma (systemic sclerosis)
Predictors of progression:
(1) older age
(2) African-American race
(3) low baseline FVC as percent of predicted, or significant decline over 2 years
(4) low baseline DLco as percent of predicted, or significant decline over 2 years
(5) more extensive interstitial fibrosis on HRCT (clearly > 20%)
(6) diffuse cutaneous sclerosis
(7) elevated biomarkers (IL-6, CRP, serum chemokine ligand 18)
(8) high titers of autoantibodies (anti-topoisomerase I, anti-CXCR3, anti-CXCR4)
where:
• Low baseline FVC and DLco may reflect more advanced disease at the time of diagnosis.
