Pulmonology Medical News about systemic sclerosis;interstitial lung disease;disese-modifying therapy

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Current concepts in disease-modifying therapy for systemic sclerosis-associated interstitial lung disease: Lessons from clinical trials
Current Rheumatology Reports, 03/27/09

Au K et al. – This article discusses interstitial lung disease (ILD) in systemic sclerosis (SSc), with a brief review on the pathogenesis and clinical measurements of disease, and it also focuses on clinical trials of potential disease-modifying therapy. SSc-related ILD now appears to be a treatable disease. Oral or intravenous cyclophosphamide is likely to be beneficial. Upcoming therapies include mycophenolatemofetil, tyrosine kinase inhibitors, (imatinib, dasatinib), and anti–IL-13 monoclonal antibody. Pts who should be offered treatment for SSc-related ILD include:

Pts with limited or diffuse SSc with dyspnea
Pts in first 4–6 yrs after onset of signs or symptoms attributable to SSc
Pts with a decline in their predicted FVC% of at least 7% and/or predicted DLCO% of at least 10% in the preceding 3–6 mo
FVC% predicted of ≤70% at time of presentation
Moderate fibrosis on baseline HRCT and ≥grade 2 fibrosis
Debate whether to treat pts with a decline in FVC and/or DLCO if SSc disease duration is >6 yrs and if pts have no dyspnea DLCO—carbon monoxide diffusing exists

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