SclerodermaSpectrum Disorders Dinesh Khanna MD Professor of Medicine Division of Rheumatology Department of Medicine Director Scleroderma Program University of Michigan Medical Center Ann Arbor MI ID: 934351
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Slide1
How to Screen for PAH inScleroderma-Spectrum Disorders
Dinesh Khanna, MDProfessor of Medicine,Division of Rheumatology,Department of MedicineDirector, Scleroderma ProgramUniversity of Michigan Medical CenterAnn Arbor, MI
Slide2Twelve individual studies reported PAH prevalence ranging from 3.6% to 32% in patients with
SScThe pooled prevalence estimate of PAH in patients with SSc was 13%In patients with DLCO < 60%, the prevalence is 19%PAH is a Frequent Complication of SSc
Figure
adapted
from Yang X, Markedian J, Sanders KN, et al. Prevalence of pulmonary arterial hypertension in patients with connective tissue diseases: a systematic review of the literature. Clin Rheumatol. 2013; 32:1519-1531. Coghlan JG, Denton CP, Grünig E, et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73:1340-1349.
60
40
20
Hachulla
et al.
Hachulla
et al.
Avouac
et al.
Vonk
et al.
Phung
et al.
de Azevedo
et al.
Coral-Alvarado
et al.
Kumar
et al.
Pope
et al.
Gindzienska
et al.
Chang et al.
Yamane et al.
Combined
0.0
Prevalence, %
(95% confidence interval)
Study prevalence estimate (relative size of box equals relative weight)
Pooled prevalence estimate
Slide3Patients With PAH-CTD Have a Poorer Survival Than IPAH Patients
100
80
60
40
20
0
0
1
2
3
4
5
6
7
Time from diagnosis (years)
Survival (%)
IPAH (
n
= 1201)
PAH-CTD (
n
= 742)
415
257
553
342
600
344
609
329
538
276
415
203
268
138
143
67
No. at risk:
IPAH
PAH-CTD
p
< 0.001
IPAH
PAH-CTD
Yr
1
88%
80%
Yr
3
74%
57%
Yr
5
64%
44%
Yr
7
57%
36%
Data from patients enrolled in the REVEAL Registry cohort
Benza RL, Miller DP, Barst RJ,
et al.
An
evaluation
of long-
term
survival
from
time of
diagnosis
in
pulmonary
arterial
hypertension
from
the REVEAL
Registry
.
Chest
2012; 142:448-56.
Slide4Screening of Scleroderma-Spectrum Disorders
Khanna D, Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201.Hoeper M, Bogaard
HJ,
Condliffe
R, et al. Definitions and diagnosis of pulmonary hypertension.
J Am Coll Cardiol. 2013;62(suppl 25):D42-D50. Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J. 2015;46:903-975. Galiè N, Humbert M,
Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37:67-119.
HOW OFTEN
Annually
HOW TO SCREEN
Resting echocardiography, PFT, and NT-proBNP or DETECT algorithm
(if DL
CO
< 60%)
IF ABNORMAL
Send for RHC
DL
CO
= diffusing capacity of the lung for carbon monoxide, RHC = right heart catheterization
PFT = pulmonary function tests; NT-
proBNP
– n-terminal pro-brain natriuretic peptide
Slide52013 Recommendations for CTD-PAH
2013 WSPH Nice symposium2015 ESC/ERS GuidelinesGuidelines Recommend Annual Screening with Resting Echocardiography for Asymptomatic Patients
Khanna D,
Gladue
H,
Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201.Hoeper M, Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl 25):D42-D50.Galiè N, Humbert M, Vachiery JL,
et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT).
Eur Respir J. 2015;46:903-975. Galiè N, Humbert M, Vachiery J-L, et al.
2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J. 2016;37:67-119.
Slide62013 Recommendations for CTD-PAH
2013 WSPH Nice symposium2015 ESC/ERS GuidelinesGuidelines Recommend Annual Screening for Asymptomatic Patients with DLCO < 60% and Disease Duration > 3 years with DETECT Algorithm DL
CO
= diffusing capacity of the lung for carbon monoxide
Khanna D,
Gladue H, Channick R, et al. Recommendations for screening and detection of connective-tissue disease associated pulmonary arterial hypertension. Arthritis Rheum. 2013;65(12):3194-3201.Hoeper M,
Bogaard HJ, Condliffe R, et al. Definitions and diagnosis of pulmonary hypertension. J Am Coll
Cardiol. 2013;62(suppl 25):D42-D50.
Galiè N, Humbert M, Vachiery JL, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: The Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European
Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur
Respir J. 2015;46:903-975. Galiè N, Humbert M,
Vachiery J-L, et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Heart J.
2016;37:67-119.
Slide7DETECT: Two-step Decision Tree for Screening SSc Patients
Step 1Step 2
RHC
for diagnosis
of presence / absence
of PAH
No referral for RHC
Step 1 total risk score plus 2 echo variables:
Right atrium area
TR velocity
No referral for echo
NO
6 non-echo variables:
FVC % pred./DLCO % pred.
Anti-centromere antibodies
Telangiectasias
NT-proBNP
ECG: Right axis deviation
Serum urate (uric acid)
Is total risk score > 300?
NO
Is total risk score > 35?
YES
YES
DETECT: Evidence-based screening algorithm for PAH in SSc patients
Coghlan JG, Denton CP, Gr
ünig
E,
et al. Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: the DETECT study. Ann Rheum Dis. 2014;73:1340-1349.
Slide8Conclusions
PAH is a frequent complication of SSc and is associated with a particularly poor prognosisScreening of SSc patients facilitates early detection of PAH and improves long term outcomesEvidence-based screening algorithms have been published that improves the detection of PAH-SSc and is recommended by different guidelines