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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? 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) • 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)
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
ILD (including sarcoidosis) epidemiologyin a multiethnic county of greater Paris B Duchemann ERJ 2017
ILD prevalence Duchemann Eur Respir J 2017
ILD incidence B Duchemann Eur Respir J 2017
ILD prevalence according to age and sexe Duchemann Eur Respir J 2017
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
Sarcoidosis epidemiology is variable Arkema ERJ 2016; Baughman Ann ATS 2016; Gribbin Thorax 2006 Morimoto ERJ 2008; Duchemann ERJ 2017
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
Uzunhan ERJ 2017 Denning ERJ 2016
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
Survival at 1, 3, 5 yrs=93, 74, 55% Boucly et al ERJ 2017
IS to consider when compression by active lymphadenopathy or persistance of active vascular lesions (PET +++)
Daily home spirometry to detect early steroid treatment effects in newly pulmonary sarcoidosis Broos ERJ 2018
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
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
Impact of inflammation/DMARDs on CVD in sarcoidosis Ungraert ERJ 2017
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?
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