Figures
- 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
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
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
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
- TABLE 1
Demographics and baseline characteristics of patients with newly diagnosed severe sarcoidosis-associated pulmonary hypertension
Patients 126 Male/female 65 (52)/61 (48) Age years 57.5±10.6 BMI kg·m−2 25.5±6.6 Tobacco exposure 42 (33) Time between sarcoidosis and pulmonary hypertension diagnosis months 204 (59–313) Radiological stage I 6 (5) II 22 (17) III 7 (6) IV 91 (72) WHO/NYHA functional class I–II 21 (17) III 79 (63) IV 26 (20) 6MWD m 319±143 Borg dyspnoea scale 4.9±2.3 Haemodynamics RAP mmHg 7±5 mPAP mmHg 46±10 PAWP mmHg 9±4 Cardiac index L·min−1·m−2 2.6±0.8 PVR Wood units 8.8±4.3 SvO2 % 63.4±8.9 Lung function tests FVC % pred 64±21 FEV1 % pred 55±22 KCO % pred 54±23 Long-term oxygen therapy 68 (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#
Baseline First follow-up visit¶ Difference p-value WHO/NYHA functional class I–II/III/IV 11/52/18 26/45/10 0.01 6MWD m 311±127 324±138 +13 m 0.33 RAP mmHg 7±4 6±4 −14% 0.007 mPAP mmHg 48±9 42±11 −13% <0.00001 Cardiac index L·min−1·m−1 2.6±0.8 2.9±0.8 +12% <0.00001 PVR Wood units 9.7±4.4 6.9±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
Patient Sex Age
yearsExtrinsic
pulmonary
artery
compressionIncreased
18F-FDG
uptake in
PET scanRadiological
stageFVC % WHO/NYHA
functional
class6MWD m mPAP
mmHgCardiac
index
L·min−1·m−2PVR Wood
unitsImmunosuppressive
therapyT0 T6 T0 T6 T0 T6 T0 T6 T0 T6 1 Male 66 Yes (lymph nodes) Yes 1 96 III II 490 540 38 29 3.1 3.7 3.2 1.9 Methotrexate 2 Female 40 Yes (lymph nodes) Yes 1 100 III II ND 420 51 29 2.1 2.2 11.7 4.2 Prednisone 60 mg·day−1 3 Female 55 Yes (fibrosing mediastinitis) Yes 1 42 III II 190 195 46 30 3.3 2.6 7.4 6.5 Prednisone 60 mg·day−1 4 Male 67 Yes (fibrosing mediastinitis) No 4 57 II II 550 530 42 46 2.8 1.9 6.3 10.6 Prednisone 30 mg·day−1 5 Female 58 Yes (fibrosing mediastinitis) ND 4 40 III III 390 367 48 39 2.8 2.9 7.3 8.4 Prednisone 30 mg·day−1 6 Female 45 No ND 4 77 III III 449 462 39 28 2.8 2.9 6.5 4.8 Prednisone 60 mg·day−1 7 Male 41 No ND 4 52 II IV 525 270 39 45 2.3 ND 7.7 ND Prednisone 60 mg·day−1 8 Male 64 No ND 2 63 III III 388 377 42 30 4.3 2.6 4.9 4.7 Prednisone 60 mg·day−1 9 Female 68 No ND 4 65 III III 242 ND 44 25 3.3 4.0 7.0 3.6 Azathioprine 100 mg·day−1 10 Female 65 No ND 4 108 II III 410 310 48 38 4.1 3.3 6.5 6.6 Prednisone 40 mg·day−1 11 Male 79 No ND 4 48 III III 509 ND 56 46 2.3 2.4 9.3 9.5 Prednisone 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 Age 1.004 (0.977–1.031) 0.794 WHO/NYHA functional class IV 2.010 (1.052–3.840) 0.034 6MWD 0.995 (0.993–0.997) <0.001 RAP 0.930 (0.861–1.005) 0.066 mPAP 0.997 (0.968–1.027) 0.834 Cardiac index 1.401 (0.994–1.976) 0.054 PVR 0.966 (0.898–1.039) 0.350 FVC 0.984 (0.969–0.999) 0.042 KCO 0.982 (0.968–0.996) 0.013 Radiological stage IV 1.845 (0.954–3.559) 0.069 PAH-targeted therapy 0.845 (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 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
