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Scleroderma Interstitial Lung Diseases - What they are and what's new
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.
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Scleroderma Interstitial Lung Diseases - What they are and what's new
- 1. Scleroderma interstitial lung diseases - whatthey are and what’s new Dean Schraufnagel, MD University of Illinois at Chicago March 15, 2014 Nothing financial to disclose.
- 2. Lung disease - leadingcause of death Steen and Medsger. Ann Rheum Dis 2007; 66:940–4 Not one, but many diseases
- 3. What is “sclerodermalung?”
- 4. Respiratory diseases ofSSc • Vascular • Interstitial fibrosis • Alveolar ductitis (organizing pneumonia) • Quiescent scarring • Aspiration-induced • Drug-induced
- 5. Respiratory diseases ofSSc • 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
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- 7. Fibrotic disease • Presentin 70-80% at autopsy • Inflammatory changes occur early • Progression variable – Exaggerated TGFß to injury • Prognosis: better than IPF
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- 11. Alveolar ductitis -responds to steroids
- 12. Lymphoid follicles Scl-70responsive T-cell clones
- 13. SSc woman withstable disease for many years
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- 15. Aspiration • Many studiesrelate 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 inSsc-ILD?
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- 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
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- 21. Basic science • 843genes 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)
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- 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.
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- 26. Summary • Scleroderma lungdisease 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
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