How to Screen for PAH in Scleroderma-Spectrum Disorders

Slides:

Advertisements

Similar presentations

Managing Multiple Medications in Patients with PAH ADAANI FROST, MD Director, Pulmonary Hypertension Center Professor of Medicine Baylor College of Medicine.

Advertisements

Coinvolgimento polmonare nella sclerosi sistemica Marco Matucci Cerinic Departments of Rheumatology AVC BioMedicine & Division of Rheumatology AOUC Medicine,

Doppler Echocardiography Overestimates Pulmonary Hypertension in Patients with Scleroderma Interstitial Lung Disease Kevin M Chan 1, Elena Tishkowski 2,

Prevalence of PAH in SSc ReferenceMethodologyDiagnosisPAH prevalence Mukerjee; 2003, UK n = 722, single center Prospective RHC12%

Early detection of pulmonary involvement in scleroderma patients By Mohamed Mostafa Metwally, MD, FCCP Assistant professor of chest diseases Assiut University.

Ipertensione polmonare Roberto Cassandro U.O. di Pneumologia e UTIR Servizio di Emodinamica e Fisiopatologia Respiratoria Ospedale San Giuseppe - Milano.

Pulmonary hypertension Goal directed therapy Pulmonary Hypertension Programme University of Toronto FMD.

VASCULAR COMPLICATIONS OF SYSTEMIC SCLEROSIS Early Diagnosis of PAH in Systemic Sclerosis: How Do We Recognize the Warning Signs? Joseph C. Shanahan, MD.

Marius M Hoeper Therapeutic goals and algorithms.

Screening for CAD Nuclear Medicine prospective S.R.Zakavi,MD,IBNM Nuclear Medicine Research Center Mashhad University of Medical Sciences.

Date of download: 7/9/2016 Copyright © The American College of Cardiology. All rights reserved. From: Pulmonary Arterial Hypertension J Am Coll Cardiol.

Scleroderma Lung Disease Robert Schilz DO, PhD University Hospitals Case Medical Center Cleveland, OH.

Figure 1 Diagnostic flowchart for patients with suspected heart failure—showing alternative ‘echocardiography first’ (blue) or ‘natriuretic peptide first’

Pulmonary response in sarcoidosis

Figure 1. Suggested algorithm for locoregional lymph node staging in patients with non-metastatic NSCLC. CT, computed tomography; EBUS, endoscopic bronchial.

REVEAL Registry PAH Risk Score Calculator

Pulmonary arterial hypertension due to pulmonary veno-occlusive disease in systemic sclerosis: Importance of early diagnosis and cautious use of pulmonary.

Volume 141, Issue 2, Pages (February 2012)

Risk Stratification in PAH: What Do the Latest Data Suggest?

UNDERSTANDING RISK STRATIFICATION IN PAH:

by Victor R. Gordeuk, Oswaldo L. Castro, and Roberto F. Machado

Are Direct Oral Anticoagulants Appropriate for PAH?

Baseline and Serial Brain Natriuretic Peptide Level Predicts 5-Year Overall Survival in Patients With Pulmonary Arterial Hypertension: Data From the REVEAL.

The following slides highlight an educational report from a Satellite Session at the 2009 Congress of the European Society of Cardiology in Barcelona,

Physiological changes in pulmonary arterial hypertension (PAH) patients which occur in response to pregnancy. Physiological changes in pulmonary arterial.

Chronic Obstructive Pulmonary Disease: An Evidence-Based Approach to Treatment With a Focus on Anticholinergic Bronchodilation Nicholas J. Gross, MD,

The following slides highlight an educational report from a Satellite Session at the 2010 Congress of the European Society of Cardiology in Stockholm,

Systemic sclerosis (SSc; also known as scleroderma) is a complex connective tissue disease (CTD) of unknown etiology. It is characterized by fibrotic.

ECG: electrocardiogram; PFT: pulmonary function testing; Dlco: diffusion capacity of the lung for carbon monoxide; BGA: blood gas analysis; HRCT: high-resolution.

High-resolution computed tomography (HRCT) features of interstitial lung abnormalities. a) HRCT of a 56-year-old patient whose mother died of idiopathic.

Diagnostic algorithm for pulmonary arterial hypertension (PAH).

Cohort identification and exclusion.

Volume 126, Issue 1, Pages 14S-34S (July 2004)

Pulmonary Arterial Hypertension and Connective Tissue Disease

Volume 146, Issue 6, Pages (December 2014)

Familial history of hereditary haemorrhagic telangiectasis (HHT) of the 29-yr-old patient (•) with HHT and severe pulmonary arterial hypertension (PAH).

Algorithm for the diagnosis and treatment of portopulmonary hypertension (POPH). Algorithm for the diagnosis and treatment of portopulmonary hypertension.

REVEAL pulmonary arterial hypertension (PAH) risk score.

Cox proportional hazard estimates for multivariate model of survival, limited to terms included in the final stepwise model. Cox proportional hazard estimates.

Inclusion characteristics of a) previously diagnosed and b) newly diagnosed pulmonary arterial hypertension (PAH) patients enrolled in REVEAL. PVR: pulmonary.

An algorithm for the early diagnosis of pulmonary arterial hypertension in systemic sclerosis. An algorithm for the early diagnosis of pulmonary arterial.

Extent of interstitial lung disease (ILD) in patients with systemic sclerosis-associated ILD. A simple stratification that utilises pulmonary function.

An expert proposal for a treat-to-target checklist for pulmonary arterial hypertension (PAH). An expert proposal for a treat-to-target checklist for pulmonary.

Risk assessment and treat-to-target approach for pulmonary arterial hypertension. Risk assessment and treat-to-target approach for pulmonary arterial hypertension.

Patient case study 2—fibrotic uILD in the setting of IPAF

Micha T. Maeder, MD, PhD, Niklas F. Ehl, MD

3-year survival of lung cancer patients in the general population and in those with a prior diagnosis of chronic obstructive pulmonary disease (COPD).

Simplified REVEAL (Registry to Evaluate Early And Long-term PAH Disease Management) risk score. Simplified REVEAL (Registry to Evaluate Early And Long-term.

Proposed screening algorithm for identification of pulmonary arterial hypertension (PAH) associated with connective tissue disease. Proposed screening.

Predicting Survival in Patients With Pulmonary Arterial Hypertension

Cumulative count of the different primary pulmonary function test (PFT) outcomes in longitudinal studies of systemic sclerosis-associated interstitial.

Prevalence of different aetiologies of pulmonary hypertension (PH) in the paediatric population (results from national registries and paediatric cohorts).

A) Cumulative count and b) percent cumulative use of the different pulmonary function test (PFT) measures as longitudinal outcomes for systemic sclerosis-associated.

Survival in patients with pulmonary arterial hypertension based on aetiology. •: congenital heart disease; ▪: collagen vascular disease; ▵: HIV-related;

Proportion of patients in each World Health Organization functional class (WHO-FC) at the time of pulmonary arterial hypertension-associated systemic sclerosis.

Pulmonary arterial hypertension specific medication use at enrolment among previously diagnosed patients. 184 (7%) of patients were not on a prostaglandin,

Distribution of mutations in sporadic and familial pulmonary arterial hypertension (PAH) and pulmonary veno-occlusive disease and/or pulmonary capillary.

Forest plot of the a) sensitivity and b) specificity of different pulmonary function test screening algorithms for the presence of systemic sclerosis-associated.

Change in physiological variables from baseline values a) at rest and b) during exercise after saline infusion and exposition to different β-blocker agents.

Results of classification and regression tree analysis in patients with pulmonary hypertension-sickle cell disease. Results of classification and regression.

Survival of idiopathic pulmonary arterial hypertension (IPAH) patients in World Health Organization functional class (FC) at baseline IV is extremely poor.

A) Pulmonary arterial hypertension (PAH) aetiological breakdown of REVEAL patients at enrolment. b) Breakdown of associated PAH subgroup. a) Pulmonary.

Survival rates in older (>65 years) compared with younger (18–65 years) patients with idiopathic pulmonary arterial hypertension. a) Expected ( )

Distribution of diffusing capacity of the lung for carbon monoxide (DLCO) among 243 scleroderma patients who underwent right heart catheterisation (RHC)

Progression in the patient’s pulmonary function tests from 2010 to a) Forced vital capacity (FVC); b) total lung capacity (TLC); c) diffusing capacity.

Module 2: evaluation of the REVEAL risk score in the FPHN validation cohort. Module 2: evaluation of the REVEAL risk score in the FPHN validation cohort.

Treatment algorithm for chronic thromboembolic pulmonary hypertension.

Real-life pulmonary arterial hypertension (PAH) patient cases to reflect the importance of a collaborative approach to patient engagement. Real-life pulmonary.

Schematic diagram of the DETECT study [56].

Effect of placebo (n=88) and bosentan (n=80) on the co-primary end-point pulmonary vascular resistance (PVR) in the EARLY (Endothelial Antagonist Trial.

Presentation transcript:

How to Screen for PAH in Scleroderma-Spectrum Disorders Dinesh Khanna, MD Professor of Medicine, Division of Rheumatology, Department of Medicine Director, Scleroderma Program University of Michigan Medical Center Ann Arbor, MI

PAH is a Frequent Complication of SSc 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 Twelve individual studies reported PAH prevalence ranging from 3.6% to 32% in patients with SSc The pooled prevalence estimate of PAH in patients with SSc was 13% In patients with DLCO < 60%, the prevalence is 19% 0.0 20 40 60 Prevalence, % (95% confidence interval) Study prevalence estimate (relative size of box equals relative weight) Pooled prevalence estimate 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.

Patients With PAH-CTD Have a Poorer Survival Than IPAH Patients Data from patients enrolled in the REVEAL Registry cohort 100 p < 0.001 80 IPAH PAH-CTD Yr 1 88% 80% Yr 3 74% 57% Yr 5 64% 44% Yr 7 36% 60 Survival (%) 40 IPAH (n = 1201) 20 PAH-CTD (n = 742) 1 2 3 4 5 6 7 Time from diagnosis (years) No. at risk: IPAH PAH-CTD 415 257 553 342 600 344 609 329 538 276 415 203 268 138 143 67 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.

Screening of Scleroderma-Spectrum Disorders HOW OFTEN Annually HOW TO SCREEN Resting echocardiography, PFT, and NT-proBNP or DETECT algorithm (if DLCO < 60%) IF ABNORMAL Send for RHC DLCO = diffusing capacity of the lung for carbon monoxide, RHC = right heart catheterization PFT = pulmonary function tests; NT-proBNP – n-terminal pro-brain natriuretic peptide 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.

Guidelines Recommend Annual Screening with Resting Echocardiography for Asymptomatic Patients 2013 Recommendations for CTD-PAH 2013 WSPH Nice symposium 2015 ESC/ERS Guidelines 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.

Guidelines Recommend Annual Screening for Asymptomatic Patients with DLCO < 60% and Disease Duration > 3 years with DETECT Algorithm 2013 Recommendations for CTD-PAH 2013 WSPH Nice symposium 2015 ESC/ERS Guidelines DLCO = 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.

DETECT: Two-step Decision Tree for Screening SSc Patients DETECT: Evidence-based screening algorithm for PAH in SSc patients Step 1 Step 2 RHC for diagnosis of presence / absence of PAH 6 non-echo variables: FVC % pred./DLCO % pred. Anti-centromere antibodies Telangiectasias NT-proBNP ECG: Right axis deviation Serum urate (uric acid) Step 1 total risk score plus 2 echo variables: Right atrium area TR velocity YES YES Is total risk score > 300? Is total risk score > 35? NO NO No referral for echo No referral for RHC 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.

Conclusions PAH is a frequent complication of SSc and is associated with a particularly poor prognosis Screening of SSc patients facilitates early detection of PAH and improves long term outcomes Evidence-based screening algorithms have been published that improves the detection of PAH-SSc and is recommended by different guidelines