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Scleroderma Associated Lung Disease

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Scleroderma Associated Lung Disease is presented by
Jane Dematte MD, MBA, Director, ILD program
Division of Pulmonary and Critical Care, Northwestern Feinberg School of Medicine

Published in: Health & Medicine
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Scleroderma Associated Lung Disease

  1. 1. 2016 Patient Forum N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Jane Dematte MD, MBA Director, ILD program Division of Pulmonary and Critical Care Northwestern Feinberg School of Medicine October 15, 2016 Scleroderma Associated Lung Disease Northwestern Medicine ILD Program
  2. 2. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E How does SSc affect the lungs? • Interstitial Lung Disease • Aspiration Lung Injury • Pulmonary Vascular Disease » Pulmonary arterial hypertension • Pulmonary Edema » Pulmonary hypertension — Pulmonary arterial hypertension — Pulmonary venous hypertension –
  3. 3. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Northwestern Medicine ILD Program What is Interstitial Lung Disease
  4. 4. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E How Common is ILD in SSc? Prevalence ILD Serology RA 19% +RF, +CCP Scleroderma 25-85% +Scl 70 (30%) ACA 40-80% lc SLE 3-33% dsDNA 50-80% Primary Sjogrens 9-75% antiSSa/SSb (ro/la) DM/PM/ (AS syndrome) 20-65% U1RNP Antisynthetase ABs (anti Jo-1) 40-80% MCTD 80% nRNP 100
  5. 5. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Northwestern Medicine ILD Program Pathologic and Radiographic Corrleates
  6. 6. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Interstitial Lung Disease vs Pulmonary Fibrosis Northwestern Medicine ILD Program
  7. 7. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Causes of SSc related deaths Steen et al Annals of the Rheumatic Diseases 2007;66:940-944
  8. 8. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Severity of Disease NSIP Need a PFT in here 2/2012 Result % predicted FVC 2.06 55 FEV1 1.60 62 FEV1/FVC NL NL TLC 3.90 69 FRC 2.36 86 (BMI 32) RV 1.84 84 Dlco 11.9 52 Hgb range (10.1-12)
  9. 9. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E What is the Impact of ILD Northwestern Medicine ILD Program Association between severity of ILD and survival in patients with SSc. FVC, forced vital capacity; PF, pulmonary fibrosis; SSc, systemic sclerosis. Steen et al. Arthritis Rheum 1994 N=890 University of Pittsburg Scleroderma databank
  10. 10. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Steen et al Annals of the Rheumatic Diseases 2007;66:940-944 Survival in Scleroderma
  11. 11. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Limited vs Extensive Ds: SSc ? RA N=215 Northwestern Medicine ILD Program Goh et al Am J Resp Crit Care Med; 177:1248-54 (2008) Ds progression Decline in FVC >10% Dlco >5%
  12. 12. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Fig 1. Survival of total IIP v. CVD-IP Park et al American Journal of Respiratory and Critical Care Medicine Vol 175. pp. 705-711, 2007 Fig 2. Comparison of the survival curves of all subject groups CVD- UIP v. CVD- NSIP; I- NSIP v. UIP N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E N=362, IIP 269, CT associated 93 UIP 203/269, 66/269 i-NSIP/36/93 UIP, 57 NSIP CV :Younger, F, nonsmokers, no diff in baseline PFT Northwestern Medicine ILD Program
  13. 13. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Who is at Risk Khanna et al; Arthritis Rheum Oct 2011; 63(10):3078
  14. 14. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Available Treatments • Mycophenolate vs Cytoxan • Rituxan • Stem Cell transplant • Lung transplant
  15. 15. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Scleroderma Lung Study II: Cytoxan v MMF FVC mRSS
  16. 16. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Rituxin in SSc 2 year fu study Dauossis et al; Clin Exp Rheumatol 2012; 30 (Suppl. 71): S17-S22. 24m median inc 12.8% 24m median inc 19.5% Northwestern Medicine ILD Program
  17. 17. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E ASTIS SCOT N randomized 156 75 Transplant regimen CYC 200 mg/kg, rabbit ATG 7.5 mg/kg, graft manipulation CD4 selection CYC 120 mg/kg, equine ATG 90 mg/kg, TBI 800 cGY lung shield, graft manipulation CD4 selection Control Arm 12x monthly CYC 750 mg/m2 12x monthly CYC 750 mg/m2 Primary endpoint Event free Survival Composite, death & end stage organ failure at 56 months Secondary endpoints TRM/toxicity mRSS OSF/HAQ-DI Deaths in both arms, no unexpected toxicity Current status Published Recruitment completed May 2011 in follow up Prospective Randomized Trials of Stem Cell Txplant in SSc: SCOT and ASTIS Tyndall, A. Nat. Rev. Rheumatol. Adc online pub 13 Sept 2011; doi:10.1038/nrrheum.2011.136
  18. 18. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E ASTIS Autologous Stem cell Transplantation International Scleroderma • 156 pts enrolled between March 2001 and Oct 2009 • Similar protocol and endpoint as SCOT • Major differences are » No TBI » rabbit ATG JAMA 2014;311(24):2490-98
  19. 19. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E HRs: (time varying): 3m 2.40 (p=.14), 6m 1.50 p=.38, 1yr .48 p=.02, 2yr .29 p=.002, thru 10 yr HR .29 p=.002
  20. 20. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Causes of Death
  21. 21. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Lung Transplant 2006 ISHLT endorsed Lung Transplantation for CT associated ILD Respiratory Medicine (2013) 107, 2081-2087 Transplantation 2013; 95: 975-980Northwestern Medicine ILD Program
  22. 22. N O R T H W E S T E R N U N I V E R S I T Y F E I N B E R G S C H O O L O F M E D I C I N E Results: 5 year follow up Northwestern Medicine ILD Program
  23. 23. thank you!

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