Scleroderma Interstitial Lung Diseases - What they are and what's new
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Scleroderma lung disease encompasses various respiratory complications, including interstitial fibrosis and alveolar ductitis, which are prevalent in patients. Recent research has identified differential gene expression in lung fibroblasts and new treatment options like antifibrotic and anti-inflammatory drugs. Despite the challenging prognosis, ongoing studies are promising in elucidating the disease mechanisms and potential therapeutic targets.
Scleroderma Interstitial Lung Diseases - What they are and what's new
- 1. Scleroderma interstitial lung diseases - what they are and what’s new Dean Schraufnagel, MD University of Illinois at Chicago March 15, 2014 Nothing financial to disclose.
- 2. Lung disease - leading cause of death Steen and Medsger. Ann Rheum Dis 2007; 66:940–4 Not one, but many diseases
- 3. What is “scleroderma lung?”
- 4. Respiratory diseases of SSc • Vascular • Interstitial fibrosis • Alveolar ductitis (organizing pneumonia) • Quiescent scarring • Aspiration-induced • Drug-induced
- 5. Respiratory diseases of SSc • Neoplastic – 8.6/1000 person-years – Risk ratio 16.5 – ↑ with fibrosis and smoking • Extra pulmonary – Pleural – Chest wall restriction – Deconditioning – Drugs affecting breathing – Depression
- 7. Fibrotic disease • Present in 70-80% at autopsy • Inflammatory changes occur early • Progression variable – Exaggerated TGFß to injury • Prognosis: better than IPF
- 10. Other forms
- 11. Alveolar ductitis - responds to steroids
- 12. Lymphoid follicles Scl-70 responsive T-cell clones
- 13. SSc woman with stable disease for many years
- 14. Aspiration
- 15. Aspiration • Many studies relate to lung disease • SSc: Lower esophageal sphincter • Upper laryngopharyngeal reflexes • Acid vs bland contents • One of our interests – Schraufnagel et al. Am J Roentgenol 2008;191:748-52.
- 17. What new in Ssc-ILD?
- 18. Classification
- 19. New classification scleroderma (2013) – “Skin thickening of the fingers extending proximal to the metacarpophalangeal joints” – If not present, 7 other criteria that include interstitial lung disease or pulmonary hypertension – American College of Rheumatology and European League against Rheumatism. Arthritis Rheum 2013;65:2737-47
- 20. Basic Science
- 21. Basic science • 843 genes differentially expressed in lung fibroblasts from SSc-ILD – These are therapy targets • Lindahl et at., Respir Res. 2013;14:80 – TGFß and related molecules • Many studies (770 in 2013) • Our interests – Endothelial cell rigidity (Levitan and colleagues) – Caveolin-1 (Minshall and colleagues)
- 22. Treatments
- 23. Treatment • Antifibrotic & anti-inflammatory drugs (ILD) – e.g. Pirfenidone, nintedanib, N-acetylcysteine – Lota and Wells. Expert Opin Pharmacother 2013;14:79-89. • Lysophosphatidic acid receptor – Zhao and Natarajan. Biochim Biophys Acta 2013;1831:86-92 • Scleroderma mouse model – Castelino et al. Arthritis Rheum 2011;63:1405-15
- 24. Transplantation • Survival - same nonconnective tissue ILD – Esophageal does not impact outcome – Sottlie et al., Transplantation. 2013;95:975-80 • CRP predicts outcome - useful measure – Liu et al., Arthritis Care Res 2013;65:1375-80.
- 25. Summary
- 26. Summary • Scleroderma lung disease has many forms • Take clues from Hx, Px, radiographs – Biopsy if need be • Although Rx of SSc lung disease is generally poor, many components can be treated. • Exciting research is helping to understand the basis of the disease
- 27. Questions?