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
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Scleroderma Associated Lung Disease
- 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. 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. thank you!