DES Pneumologie Ile de France...
Transcript of DES Pneumologie Ile de France...
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Yurdagül Uzunhan
Service de Pneumologie, Pr Nunes
Hôpital Avicenne Université Paris 13
DES Pneumologie Ile de France
09/12/2016
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Observation 1 – Mme P. 53 ans
Motif de Consultation: PID
Mode de Vie
o Secrétaire commerciale
o Pavillon sain – 1 canari
o Non fumeuse
ATCD
o Pas de notion de PID dans la famille ni de connectivite
o 1992: Hémianopsie latérale homonyme droite sous oestro-progestatifs
o 2000: Coelioscopie pour Endométriose
o Canal carpien bilatéral opéré en 1999 à droite et en 2002 à gauche
HDLM
o Asthénie d’installation lentement progressive avec arthralgies des mains, toux sèche
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EFR : CVF 2, 600 L (92 %) CPT 3,660 L (79%), VEMS 2,140 L (86%) DLCO 40%
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30% des SSc diffuses 25-50% des SSc -Myosites
PM-Scl ; Th/To Topoisomérases
70-80% des SSc limitées
Centromères
Gabrielli et al. N Engl J Med 2009
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Diamètre coronal de la lumière ≥ 9 mm sous-aortique
Niveau hydro-aérique
Atteinte oesophagienne
Diapo H. Nunes
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Pneumopathie Interstitielle Diffuse
Connectivite Pneumopathies
Interstitielles idiopathiques
Granulomatose: sarcoïdose
Formes particulières : HPCL, LAM,
protéinose alvéolaire, PCIE…
Chroniques fibrosantes Aiguës, subaiguës
- PINS - PINS PO - PIC
- PO - PIA
- DIP - LIP - PPFE
Œdème pulmonaire Infections (miliaire, pneumocystose…) Prolifération tumorale (lymphangite carcinomateuse, lymphome)
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• Résultats variables en fonction du territoire lavé.
• Données du LBA non corrélées au pattern histologique de la PID-SSc.
• Résultats corrélés à la sévérité de la PID, mais impact pronostique propre controversé.
Goh et al. Arthritis Rheum 2007 Strange et al. Am J Respir Crit Care Med 2008
Kowal-Bielecka et al. Semin Arthritis Rheum 2010
• Pas de valeur prédictive d’une progression de la maladie ou d’une réponse au traitement.
Goh et al. Arthritis Rheum 2007 Strange et al. Am J Respir Crit Care Med 2008
LBA
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Traitement
Qui ?
Quand ?
Comment ?
Combien de temps?
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3 essais thérapeutiques avec le Cyclophosphamide Amélioration modeste voire non significative
Tashkin et al. N Engl J Med. 2006 Hoyles et al. Arthritis Rheum. 2006
Bénéfice sur les formes aggravatives
Berezne et al. J Rheumatol. 2008
Mais attendre 6 mois = retard au traitement
Important de déterminer des facteurs pronostiques au diagnostic de la PID
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215 patients with SSc-ILD
2 radiologists, 2 physicians with > 18 mo of experience in ILD and 4 trainees (2 radiologists and 2 physicians, with 6 mo experience in ILD).
Analysis required 1.49 ± 0.93 minutes/scan.
Interobserver agreement was good for practitioners (k = 0.64), and fair to moderate for trainees (k = 0.41).
Algorithme pronostique
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Moore et al. Rheumatology 2013
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Figure Legend:
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CYP
CS
MMF
O2
AZT
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Observation 2 – Mme R. née en 1983
Motif de Consultation: adressée par le service de dermatologie pour sclérodermie avec PID
Mode de Vie
o Née en Guyane, secrétaire, tabac 0, 6 canaris
ATCD
o Pas de notion de PID dans la famille ni de connectivite
HDLM
o 2007 Sclérodermie systémique cutanée diffuse
o Atteinte cutanée diffuse
o Raynaud
o Atteinte oesophagienne
o Atteinte musculaire prouvée
o AAN 1/160ème moucheté type centromères
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EFR 2007: CVF 1670 ml (49 %), CPT 2470 ml (54% ), TLCO à 44%
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TM6 : distance parcourue 472 m désaturation de 97 à 92 % EFR : stabilité des volumes pulmonaires, amélioration de la DLCO CVF à 1100 ml (31 % Theo) CPT à 1720 ml (38 %) CVF à 1080 ml (34 %) DLCO à 29 % Echographie cardiaque: normale, notamment PAPs estimée à 21 mmHg
Traitement : - Cellcept : 2 g par jour - Cortancyl : 16 mg par jour - Bitildiem 120 : 1 cp x 2 par jour. - Fonzilane 300 : 1 cp x 2/jour - Triatec 1,25 : 1 cp par jour - Cacit D3 : 1 sachet par jour - Inexium 40 : 1 cp par jour - Motilium : 1 cp x 3 par jour. consultation dans un centre de transplantation pulmonaire
05 / 2008
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Crise rénale sclérodermique 5 % des patients ayant une Sclérodermie systémique Anticorps anti-RNApolymerase III (10% des SSc ; 15% SSc cutanées diffuses)
CS > 15 mg/j dans les 3 mois
Mouthon et al. J Rheumatol. 2015
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Scleroderma renal crisis (SRC): HTA maligne & I rénale aiguë oligo-anurique Plus fréquent chez les patients sous CS Déclenché par nephrotoxiques ou depletion vasculaire PBR non indispensable mais élément pronostique Pronostic (5-year survival 65%) amélioré avec introduction
des IEC
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PID: cause directe de 40% des DC et facteur contributif majeur dans 27% des autres cas
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CYP
CS
MMF O2
KT droit 12/2014 PAPm 35 mmHg
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10/2016 : CVF=1.03L (29%), VEMS=980mL (33%); GDS sous 2L/min: pH 7.37, PaOC2= 49, PaO2= 74 ; TM6: 210m (29%) Nadir 78%.
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Khan et al. Respir Med 2013
Transplantation Pulmonaire au cours de la Sclérodermie
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Khan et al. Respir Med 2013
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Launay et al. PressMed 2014
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Daoussis et al. Semin Arthr Rheum 2016
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p=0,013
Daoussis et al. Semin Arthr Rheum 2016
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PHRCN 2015 : acronyme "EvER-ILD" coordonné par le Pr Sylvain-Marchand Adam (Equipe 2 du CEPR/Inserm U1100). Evaluation de l'Efficacité et de la tolérance du Rituximab en association avec le mycophénolate mofetil comparé au mycophénolate mofetil seul chez les patients ayant une pneumopathie interstitielle et non répondeurs à une première ligne de traitement immunosuppresseur : essai randomisé en double-insu contre placebo.
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Inclusion Criteria: 1.Diagnosis of systemic sclerosis−related (SSc) confirmed by the American College of Rheumatology classification criteria of systemic sclerosis (Masi 1980); duration of diagnosis less than (<) 7 years 2.Diagnosis of SSc-ILD based on an high-resolution computed tomography (HRCT) scan 3.Screening forced vital capacity (FVC) greater than equal to (>=) 50 percent (%) of the predicted value, and screening carbon monoxide diffusing capacity (DLCO) >=40% of the predicted value 4.At study entry, the participant either was not taking SSc-ILD medication or was taking cyclophosphamide or mycophenolate Exclusion Criteria: 1.Clinically significant pulmonary hypertension 2.Known underlying liver disease 3.Clinical evidence of significant aspiration or uncontrolled gastroesophageal reflux 4.History of clinically significant asthma or chronic obstructive pulmonary disease 5.Active infection 6.Diagnosis of another connective tissue disorder 7.Evidence of a malignancy that is likely to result in significant disability or require significant medical or surgical intervention 8.History of unstable or deteriorating cardiac or pulmonary disease (other than SSc-ILD) 9.Pregnancy or lactation 10.Creatinine clearance <40 milliliters per minute (mL/min) 11.Prior use of pirfenidone 12.Unsuitable for enrollment or unlikely to comply with study requirements
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Khanna et al. Rheumatol. 2016
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Khanna et al. Rheumatol. 2016
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Khanna et al. Rheumatol. 2016
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PNN ≥ 3%
et/ou
PNE ≥ 2%
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Lien significatif en analyse multivariée avec la mortalité précoce HR=8.40 (95% IC: 1.91–36.95), p=0.005
Pas de lien significatif avec la mortalité tardive ou le déclin fonctionnel
Disparition de l’association avec la mortalité globale après ajustement sur la sévérité de la PID