SCREENING FOR PH-ILD

You can uncover clues for PH early with routine ILD tests^2,3

Routine ILD assessments, including regular monitoring of D_LCO trends and review of HRCT reports, help identify PH-ILD.

Routine ILD tests	PH watchouts
PFTs (D_LCO)*	PH watchouts Low D_LCO (<40% predicted)^2-7 Disproportionate decline in D_LCO vs FVC^2,3,5†
CT/HRCT scan^2,3,8-11	PH watchouts RV enlargement (eg, RV:LV ratio >1) PA enlargement (eg, PA >32 mm) Flattening of the septum Enlarged pulmonary arteries in the lung periphery
Exercise capacity	PH watchouts Poor results during exercise testing^3-5,12,13‡ Marked or worsening desaturation or dyspnea Severely reduced or worsened distance, particularly with stable PFTs Impaired heart rate recovery
Oxygen needs	PH watchouts Any need for supplemental oxygen³
BNP and NT-proBNP	PH watchouts Elevated or increasing levels of BNP or NT-proBNP^14-16 Levels correlate to the severity of cardiac stress¹⁷ Levels increase before symptoms of severe PH and heart failure (eg, peripheral edema, weight gain) are present¹⁷

Preliminary findings from the PHINDER study suggest trends in D_LCO and PA enlargement are helpful in detecting PH early.² Learn more about PHINDER study

*Declining FVC and D_LCO are both measures of ILD disease progression and severity. In ILD, the decline in D_LCO is thought to be due to alveolar destruction and thickening/compression of pulmonary capillaries. PH can cause damage to the microvasculature, resulting in an additional decline in D_LCO that does not always correlate with FVC impairment.^3-5,18-21

^†CPFE is commonly associated with the combination of preserved lung volumes and extremely low D_LCO and also carries a high risk for PH. WSPH recommends using an FVC/D_LCO ratio >1.6 as a threshold for suspecting PH in patients with ILD.^5,22

^‡Based on studies using the 6MWT.^3-5

Echo alone is not sensitive enough to rule out PH-ILD^23-25

In a cohort of patients with ILD clinically suspected of having PH-ILD^§ (N=265)

40%

of patients with an echo showing a low likelihood of PH were confirmed to have PH-ILD by RHC²⁴§^||

Echo probability of PH^\|\|	Patients, n	Patients with PH, n (%) (confirmed by RHC)
Low	43	17 (40%)
Intermediate	60	42 (70%)
High	162	135 (83%)

Low probability of PH was defined as TRV ≤2.8 m/s or unmeasurable, with no other echo signs of PH.²⁴

Why does echo have limitations for identifying PH early?

Echo findings alone do not always detect early-stage PH.^26-28

Signs of RV dysfunction may appear in later-stage PH
Some echo findings that are suggestive of PH require expert echo assessment and may not be routinely evaluated

RVSP estimates of mPAP derived from echo are frequently inaccurate or not reported.^4,23,25-27

In fact, in about 50% of patients with ILD, TR jet velocity (used to calculate RVSP) may:

Frequently over- or underestimate RVSP, causing echo estimates to differ from RHC values by >10 mm Hg^||
Be difficult to visualize, preventing RVSP from being calculated or reported

Echo is just one piece of the puzzle for PH-ILD detection.^23-25

Interpret echo results in conjunction with other clinical findings to help determine the need for a right heart catheterization.

Interpret echo results in conjunction with other clinical findings to help determine the need for RHC.^3¶

§Clinical suspicion was determined following integrated review of all relevant information (ie, physical exam, echo, PFTs, and other tests where available) by an expert PH physician.³

^||Probability was determined based on modified 2015 ESC/ERS screening recommendations (ie, using peak TRV thresholds plus the presence of RV dilatation and/or dysfunction).
PH was defined as mPAP ≥25 mm Hg. Of the 194 patients with confirmed PH, 140 (72%) had precapillary PH (PCWP ≤15 mm Hg). For the 17 patients misclassified as having a “low probability” of PH, mean mPAP was 35 mm Hg, mean PCWP was 11.4 mm Hg, and mean PVR was 4.9 WU.³

^¶Echo-estimated systolic PAP vs RHC-measured systolic PAP.^4,6

Are your patients' ILD symptoms getting worse, or is it PH?^4,23,29,30

Overlapping symptoms such as reduced exercise ability or inability to quickly recover, fatigue and dyspnea in your ILD patients may be an early indicator of pulmonary hypertension.

Ask your patients about any recent deterioration in symptoms.^4,23,29,30

PH is often not diagnosed until significant right heart dysfunction has developed^4,23,29

Early PH symptoms overlap with symptoms of ILD progression^4,23,29:

Increased shortness of breath

Fatigue

Reduced ability to be active

Later, more distinct PH signs indicate significant right heart dysfunction^29,30:

Jugular vein distention

Peripheral edema

Early discussions with your patients about PH may help facilitate earlier detection.^3,4 Start the conversation today.

To catch PH early, talk to your patients about any changes they experience^3,4

Questions to consider asking your patients:

What has changed since your last appointment?

Are you able to walk as far as you used to without resting?

What does a typical day look like for you? How is your routine different since our last appointment?

Are you not doing some things you used to?

For example:

– Do you walk your dog? How far are you able to walk together? Has that become a more difficult activity?

– Can you walk to a store from the parking lot? Or do you need to be dropped off at the front?

6MWT=6-minute walk test; BNP=B-type natriuretic peptide; CPFE=combined pulmonary fibrosis and emphysema; CT=computed tomography; D_LCO=diffusing capacity of the lung for carbon monoxide; ERS=European Respiratory Society; ESC=European Society of Cardiology; FVC=forced vital capacity; HRCT=high-resolution computed tomography; ILD=interstitial lung disease; LV=left ventricular; mPAP=mean pulmonary arterial pressure; NT-proBNP=N-terminal pro–B-type natriuretic peptide; PA=pulmonary artery; PAH=pulmonary arterial hypertension; PAP=pulmonary arterial pressure; PCWP=pulmonary capillary wedge pressure; PFT=pulmonary function test; PH=pulmonary hypertension; PVR=pulmonary vascular resistance; RHC=right heart catheterization; RV=right ventricular/right ventricle; RVSP=right ventricular systolic pressure; TR=tricuspid regurgitation; TRV=tricuspid regurgitation velocity; WHO=World Health Organization; WSPH=World Symposium on Pulmonary Hypertension; WU=Wood units.

LEARN MORE ABOUT DIAGNOSING PH-ILD

SCREENING FOR PH-ILD

You can uncover clues for PH early with routine ILD tests^2,3

Echo alone is not sensitive enough to rule out PH-ILD^23-25

Why does echo have limitations for identifying PH early?

Are your patients' ILD symptoms getting worse, or is it PH?^4,23,29,30

PH is often not diagnosed until significant right heart dysfunction has developed^4,23,29

To catch PH early, talk to your patients about any changes they experience^3,4

IMPORTANT SAFETY
INFORMATION

WARNINGS AND PRECAUTIONS

IMPORTANT SAFETY
INFORMATION

WARNINGS AND PRECAUTIONS

DRUG INTERACTIONS/SPECIFIC POPULATIONS

ADVERSE REACTIONS

SCREENING FOR PH-ILD

You can uncover clues for PH early with routine ILD tests2,3

Echo alone is not sensitive enough to rule out PH-ILD23-25

Why does echo have limitations for identifying PH early?

Are your patients' ILD symptoms getting worse, or is it PH?4,23,29,30

PH is often not diagnosed until significant right heart dysfunction has developed4,23,29

To catch PH early, talk to your patients about any changes they experience3,4

You can uncover clues for PH early with routine ILD tests^2,3

Echo alone is not sensitive enough to rule out PH-ILD^23-25

Are your patients' ILD symptoms getting worse, or is it PH?^4,23,29,30

PH is often not diagnosed until significant right heart dysfunction has developed^4,23,29

To catch PH early, talk to your patients about any changes they experience^3,4