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Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension

Athénaïs Boucly, Vincent Cottin, Hilario Nunes, Xavier Jaïs, Abdelatif Tazi, Grégoire Prévôt, Martine Reynaud-Gaubert, Claire Dromer, Catherine Viacroze, Delphine Horeau-Langlard, Christophe Pison, Emmanuel Bergot, Julie Traclet, Jason Weatherald, Gérald Simonneau, Dominique Valeyre, David Montani, Marc Humbert, Olivier Sitbon, Laurent Savale
European Respiratory Journal 2017 50: 1700465; DOI: 10.1183/13993003.00465-2017
Athénaïs Boucly
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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  • For correspondence: athenais.boucly@aphp.fr
Vincent Cottin
4Université Lyon-1, HCL, Service de Pneumologie, Centre de Référence des Maladies Pulmonaires Rares, Hôpital Louis Pradel, Lyon, France
15These authors contributed equally to this work
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Hilario Nunes
5Service de Pneumologie, AP-HP, Hôpital Avicenne, Université Paris 13, Bobigny, France
15These authors contributed equally to this work
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Xavier Jaïs
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Abdelatif Tazi
6Service de Pneumologie, AP-HP, Hôpital Saint-Louis, Université Paris-Diderot, Paris, France
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Grégoire Prévôt
7Service de Pneumologie, CHU de Toulouse, Hôpital Larrey, Toulouse, France
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Martine Reynaud-Gaubert
8Service de Pneumologie, CHU Nord, Aix-Marseille Université, Marseille, France
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Claire Dromer
9Service de Maladies Respiratoires, CHU de Bordeaux, Hôpital du Haut Lévêque, Université de Bordeaux, Pessac, France
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Catherine Viacroze
10Service de Pneumologie, CHU de Rouen, Hôpital Bois-Guillaume, Rouen, France
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Delphine Horeau-Langlard
11Service de Pneumologie, CHU de Nantes, Hôpital Laënnec, Nantes, France
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Christophe Pison
12Clinique Universitaire de Pneumologie, CHU de Grenoble-Alpes, Pôle Thorax et Vaisseaux, INSERM U1055, Université Grenoble-Alpes, Grenoble, France
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Emmanuel Bergot
13Service de Pneumologie, CHRU de Caen, Hôpital Côte de Nacre, Université de Caen-Normandie, Caen, France
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Julie Traclet
4Université Lyon-1, HCL, Service de Pneumologie, Centre de Référence des Maladies Pulmonaires Rares, Hôpital Louis Pradel, Lyon, France
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Jason Weatherald
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
14Dept of Medicine, Division of Respirology, University of Calgary, Calgary, AB, Canada
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Gérald Simonneau
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Dominique Valeyre
5Service de Pneumologie, AP-HP, Hôpital Avicenne, Université Paris 13, Bobigny, France
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David Montani
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Marc Humbert
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
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Olivier Sitbon
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
16These authors contributed equally to this work
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Laurent Savale
1Université Paris-Sud, Faculté de Médecine, Université Paris-Saclay, Le Kremlin-Bicêtre, France
2Service de Pneumologie, AP-HP, Centre de Référence de l'Hypertension Pulmonaire, Hôpital Bicêtre, Le Kremlin-Bicêtre, France
3INSERM UMR_S 999, Hôpital Marie Lannelongue, Le Plessis Robinson, France
16These authors contributed equally to this work
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  • FIGURE 1
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    FIGURE 1

    Patient disposition and initial therapy. RHC: right heart catheterisation; PAH: pulmonary arterial hypertension; ERA: endothelin receptor antagonist; PDE-5i: phosphodiesterase type 5 inhibitor.

  • FIGURE 2
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    FIGURE 2

    Box plots of change in 6-min walk distance (6MWD) and pulmonary vascular resistance (PVR) according to a, b) radiological stage of sarcoidosis or c, d) severity of restrictive physiology (forced vital capacity (FVC)). a) Change in 6MWD according to radiological stage of sarcoidosis (stage IV versus others): +5±49% versus +21±49%; p=0.11. b) Change in PVR according to radiological stage of sarcoidosis (stage IV versus others): –24±29% versus –22±32%; p=0.73. c) Change in 6MWD according to FVC (≤50% versus >50% predicted): –1±56% versus +14±45%; p=0.10. d) Change in PVR according to FVC (≤50% versus >50% predicted): –22±32% versus –27±25%; p=0.57. Box plots indicate the mean, standard deviation and extreme values.

  • FIGURE 3
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    FIGURE 3

    Kaplan–Meier analysis of the overall survival in patients with severe sarcoidosis-associated pulmonary hypertension. Survival at 1, 3 and 5 years was 93%, 74% and 55%, respectively.

  • FIGURE 4
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    FIGURE 4

    Proposed algorithm for the management of sarcoidosis-associated pulmonary hypertension. This algorithm must be read with caution because it relies on retrospective and open-label data, and must therefore be confirmed by future randomised controlled trials. HRCT: high-resolution computed tomography; V/Q: ventilation/perfusion; 18F-FDG: 18F-2-fluoro-2-deoxy-d-glucose; PET: positron emission tomography; mPAP: mean pulmonary artery pressure; CI: cardiac index; PAH: pulmonary arterial hypertension.

Tables

  • Figures
  • Supplementary Materials
  • TABLE 1

    Demographics and baseline characteristics of patients with newly diagnosed severe sarcoidosis-associated pulmonary hypertension

    Patients126
    Male/female65 (52)/61 (48)
    Age years57.5±10.6
    BMI kg·m−225.5±6.6
    Tobacco exposure42 (33)
    Time between sarcoidosis and pulmonary hypertension diagnosis months204 (59–313)
    Radiological stage
     I6 (5)
     II22 (17)
     III7 (6)
     IV91 (72)
    WHO/NYHA functional class
     I–II21 (17)
     III79 (63)
     IV26 (20)
    6MWD m319±143
    Borg dyspnoea scale4.9±2.3
    Haemodynamics
     RAP mmHg7±5
     mPAP mmHg46±10
     PAWP mmHg9±4
     Cardiac index L·min−1·m−22.6±0.8
     PVR Wood units8.8±4.3
     SvO2 %63.4±8.9
    Lung function tests
     FVC % pred64±21
     FEV1 % pred55±22
     KCO % pred54±23
    Long-term oxygen therapy68 (54)

    Data are expressed as n, n (%), mean±sd or median (interquartile range). BMI: body mass index; WHO: World Health Organization; NYHA: New York Heart Association; 6MWD: 6-min walk distance; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PAWP: pulmonary arterial wedge pressure; PVR: pulmonary vascular resistance; SvO2: mixed venous oxygen saturation; FVC: forced vital capacity; FEV1: forced expiratory volume in 1 s; KCO: transfer coefficient of the lung for carbon monoxide.

    • TABLE 2

      Effects of pulmonary arterial hypertension-targeted therapy on World Health Organization (WHO)/New York Heart Association (NYHA) functional class, exercise capacity and haemodynamics at first follow-up visit in patients with severe sarcoidosis-associated pulmonary hypertension#

      BaselineFirst follow-up visit¶Differencep-value
      WHO/NYHA functional class I–II/III/IV11/52/1826/45/100.01
      6MWD m311±127324±138+13 m0.33
      RAP mmHg7±46±4−14%0.007
      mPAP mmHg48±942±11−13%<0.00001
      Cardiac index L·min−1·m−12.6±0.82.9±0.8+12%<0.00001
      PVR Wood units9.7±4.46.9±3.0−29%<0.00001

      Data are expressed as n or mean±sd, unless otherwise stated. 6MWD: 6-min walk distance; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance. #: n=81; ¶: median (interquartile range) 4.5 (4.0–6.7) months.

      • TABLE 3

        Evolution at 6 months of individual and haemodynamic parameters in patients treated with initiation or escalation of immunosuppressive therapy alone

        PatientSexAge
        years
        Extrinsic
        pulmonary
        artery
        compression
        Increased
        18F-FDG
        uptake in
        PET scan
        Radiological
        stage
        FVC %WHO/NYHA
        functional
        class
        6MWD mmPAP
        mmHg
        Cardiac
        index
        L·min−1·m−2
        PVR Wood
        units
        Immunosuppressive
        therapy
        T0T6T0T6T0T6T0T6T0T6
        1Male66Yes (lymph nodes)Yes196IIIII49054038293.13.73.21.9Methotrexate
        2Female40Yes (lymph nodes)Yes1100IIIIIND42051292.12.211.74.2Prednisone 60 mg·day−1
        3Female55Yes (fibrosing mediastinitis)Yes142IIIII19019546303.32.67.46.5Prednisone 60 mg·day−1
        4Male67Yes (fibrosing mediastinitis)No457IIII55053042462.81.96.310.6Prednisone 30 mg·day−1
        5Female58Yes (fibrosing mediastinitis)ND440IIIIII39036748392.82.97.38.4Prednisone 30 mg·day−1
        6Female45NoND477IIIIII44946239282.82.96.54.8Prednisone 60 mg·day−1
        7Male41NoND452IIIV52527039452.3ND7.7NDPrednisone 60 mg·day−1
        8Male64NoND263IIIIII38837742304.32.64.94.7Prednisone 60 mg·day−1
        9Female68NoND465IIIIII242ND44253.34.07.03.6Azathioprine 100 mg·day−1
        10Female65NoND4108IIIII41031048384.13.36.56.6Prednisone 40 mg·day−1
        11Male79NoND448IIIIII509ND56462.32.49.39.5Prednisone 60 mg·day−1

        18F-FDG: 18F-2-fluoro-2-deoxy-d-glucose; PET: positron emission tomography; FVC: forced vital capacity; WHO: World Health Organization; NYHA: New York Heart Association; 6MWD: 6-min walk distance; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance; T0: baseline visit; T6: 6-month visit; ND: no data available.

        • TABLE 4

          Univariate analysis relating survival time to selected baseline variables

          Hazard ratio (95% CI)p-value
          Sex (female)1.017 (0.573–1.806)0.954
          Age1.004 (0.977–1.031)0.794
          WHO/NYHA functional class IV2.010 (1.052–3.840)0.034
          6MWD0.995 (0.993–0.997)<0.001
          RAP0.930 (0.861–1.005)0.066
          mPAP0.997 (0.968–1.027)0.834
          Cardiac index1.401 (0.994–1.976)0.054
          PVR0.966 (0.898–1.039)0.350
          FVC0.984 (0.969–0.999)0.042
          KCO0.982 (0.968–0.996)0.013
          Radiological stage IV1.845 (0.954–3.559)0.069
          PAH-targeted therapy0.845 (0.526–2.650)0.684

          WHO: World Health Organization; NYHA: New York Heart Association; 6MWD: 6-min walk distance; RAP: right atrial pressure; mPAP: mean pulmonary artery pressure; PVR: pulmonary vascular resistance; FVC: forced vital capacity; KCO: transfer coefficient of the lung for carbon monoxide; PAH: pulmonary arterial hypertension.

          Supplementary Materials

          • Figures
          • Tables
          • Supplementary Material

            Please note: supplementary material is not edited by the Editorial Office, and is uploaded as it has been supplied by the author.

            Supplementary tables S1 and S2 ERJ-00465-2017_Tables

            Supplementary figure S1 ERJ-00465-2017_Figure

          • Supplementary Material

            E. Bergot ERJ-00465-2017_Bergot

            A. Boucly ERJ-00465-2017_Boucly

            V. Cottin ERJ-00465-2017_Cottin

            M. Humbert ERJ-00465-2017_Humbert

            Xavier Jaïs ERJ-00465-2017_Jais

            D. Montani ERJ-00465-2017_Montani

            H. Nunes ERJ-00465-2017_Nunes

            C. Pison ERJ-00465-2017_Pison

            Grégoire Prévôt ERJ-00465-2017_Prevot

            L. Savale ERJ-00465-2017_Savale

            G. Simonneau ERJ-00465-2017_Simonneau

            O. Sitbon ERJ-00465-2017_Sitbon

            A. Tazi ERJ-00465-2017_Tazi

            J. Traclet ERJ-00465-2017_Traclet

            D. Valeyre ERJ-00465-2017_Valeyre

            J. Weatherald ERJ-00465-2017_Weatherald

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          Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension
          Athénaïs Boucly, Vincent Cottin, Hilario Nunes, Xavier Jaïs, Abdelatif Tazi, Grégoire Prévôt, Martine Reynaud-Gaubert, Claire Dromer, Catherine Viacroze, Delphine Horeau-Langlard, Christophe Pison, Emmanuel Bergot, Julie Traclet, Jason Weatherald, Gérald Simonneau, Dominique Valeyre, David Montani, Marc Humbert, Olivier Sitbon, Laurent Savale
          European Respiratory Journal Oct 2017, 50 (4) 1700465; DOI: 10.1183/13993003.00465-2017

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          Management and long-term outcomes of sarcoidosis-associated pulmonary hypertension
          Athénaïs Boucly, Vincent Cottin, Hilario Nunes, Xavier Jaïs, Abdelatif Tazi, Grégoire Prévôt, Martine Reynaud-Gaubert, Claire Dromer, Catherine Viacroze, Delphine Horeau-Langlard, Christophe Pison, Emmanuel Bergot, Julie Traclet, Jason Weatherald, Gérald Simonneau, Dominique Valeyre, David Montani, Marc Humbert, Olivier Sitbon, Laurent Savale
          European Respiratory Journal Oct 2017, 50 (4) 1700465; DOI: 10.1183/13993003.00465-2017
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