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Sarcoidosis: what’s new?

Sarcoidosis: what’s new?. Dominique Valeyre EA2363, Université Paris 13, Sorbonne Paris Cité AP-HP, site Avicenne, Bobigny. PneumoTrieste 04/17/2018. COI disclosure. No COI with this presentation Member of scientific advisory boards on IPF treatment (Bohringer-Ingelheim, Roche)

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Sarcoidosis: what’s new?

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  1. Sarcoidosis: what’s new? Dominique Valeyre EA2363, Université Paris 13, Sorbonne Paris Cité AP-HP, site Avicenne, Bobigny PneumoTrieste 04/17/2018

  2. COI disclosure • No COI with this presentation • Member of scientific advisory boards on IPF treatment (Bohringer-Ingelheim, Roche) • Support for transportation and acomodation in ATS, ERS and CPLF scientific meetings • Fees for lectures on « sarcoidosis » in « avancées de pneumologie » (Astra) and on ILDs (ISIS France)

  3. Selected themes and articles Epidemiology • Duchemann B et al ERJ 2017; 50 • Rossides M et al. ERJ 2018; 51 Severepulmonarysarcoidosis • Kirkil G et al Chest 2017 • Uzunhan Y et al. ERJ 2017; 49 • Boucly A et al. ERJ 2017; 50 CVD risk in sarcoidosis(Ungraet ERJ 2017) Phenotypes of sarcoidosis • Schupp ERJ 2018 • Mana Medicine 2017 Treatment • Broos CE et al. ERJ 2018; 51 • Khan Respir Med 2017 • Heidelberger JAMA Dermatology 2017 • SchimmelpenninckRespir Med 2018 Réhabilitation • Lingner H et al. Respiration 2018 Etiology/pathogenesis(mTorpathway; Th17.1; miRNA; microbiome) • Linke M, Weicchart T Nat Immunology 2017 • Broos C ERJ 2018 • Ascoli C AJRCMB 2018 • Zimmermann ERJ 2017

  4. Epidemiology of sarcoidosis

  5. ILD (including sarcoidosis) epidemiologyin a multiethnic county of greater Paris B Duchemann ERJ 2017

  6. B Duchemann Eur Respir J 2017

  7. ILD prevalence Duchemann Eur Respir J 2017

  8. ILD incidence B Duchemann Eur Respir J 2017

  9. ILD prevalence according to age and sexe Duchemann Eur Respir J 2017

  10. Impact of geographical origin in ILD epidemiology Table 3: Odd ratio by geographical origin for the main etiological diagnoses (multinomial logistic regression analysis, reviewed cases) Duchemann Eur Respir J 2017

  11. Sarcoidosis epidemiology is variable Arkema ERJ 2016; Baughman Ann ATS 2016; Gribbin Thorax 2006 Morimoto ERJ 2008; Duchemann ERJ 2017

  12. Sarcoidosis mortality in Sweden: a population-based cohort study National patient register in Sweden • cohort study • only 30% with sarcoidosis mention on death certificate • increase of all cause mortality (+62%) • associated to initial treatment • ≠ Ungrasaert Mayo Clinic Proc 2016 Rossides ERJ 2018

  13. Walsh Lancet RM 2014

  14. Walsh Lancet RM 2014

  15. Kirkil Chest 2017

  16. Uzunhan ERJ 2017 Denning ERJ 2016

  17. Uzunhan ERJ 2017; Godet Chest 2016

  18. Uzunhan ERJ 2017

  19. Uzunhan ERJ 2017

  20. Conclusions for CPA-sarcoidosis • Occurs in advanced pulmonary sarcoidosis (often with pulmonary masses) • Association with prior intensive mould occupational exposure • Mortality mainly caused by advanced pulmonary fibrosis (>20% at CT, PH, CPI, Walsh algorithm) more than CPA

  21. Survival at 1, 3, 5 yrs=93, 74, 55% Boucly et al ERJ 2017

  22. IS to consider when compression by active lymphadenopathy or persistance of active vascular lesions (PET +++)

  23. Schupp ERJ 2018

  24. Mana Medicine 2017

  25. Ungrasert ERJ 2017

  26. Daily home spirometry to detect early steroid treatment effects in newly pulmonary sarcoidosis Broos ERJ 2018

  27. GC adverse effects Khan Respir Med 2017 • Retrospective monocentric study of new sarcoidosis cases 105 GC+ vs 49 GC- • Criteria: diabetes, BMI, HTN, hyperlipidemia, bone density, ophtalmologic complications; any complication; new abnomality or worsening of a previous abnormality • Predisposing factors: age, sexe, origin • End-point: time elapsed before any event; each event

  28. Results • Risk of adverse events x 2.35 in group GC+ • Linked with cumulated doses or duration of GC • Predisposing factors: age and preexisting trouble • Most significant effects on: BMI and HTN

  29. Khan Respir Med 2017

  30. Khan Respir Med 2017

  31. Impact of inflammation/DMARDs on CVD in sarcoidosis Ungraert ERJ 2017

  32. Efficacy and safety of infliximab biosimilar inflectra in sarcoidosisSchimmelpenninck Respir Med 2018 • Retrospective study: n=29 (main indication: lung) • Dose 5mg/kg • Duration 6 months • Response +: SUV max; sIL-2R; FVC; HRQOL • 4 infectious episodes (not severe) • No need for interruption of treatment • Conclusion: • Far cheaper • Short-term efficacy and safety of inflectra • Unresolved questions • Long-term immunisation? • Effet of serial change of biosimilars?

  33. Heidelberger JAMA Dermatology 2017

  34. Aknowledgements • Avicenne + P13 (EA2363) • H Nunes; Y Uzunhan; D Bouvry; O Freynet; F Jeny • C Planès; PY Brillet; M Brauner; D Piver; M Kambouchner; JF Bernaudin • Equipes de génétique (A Calender; Y Pacheco); immunologie (M Miyara; G Gorochov); épidémiologie (I Annesi-Maesano; PA Rosental; N Nathan); modèles (C Planès; V Besnard; F Jeny) • Groupe GSF, groupe de travail officiel de la SPLF

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