Approach to Scleroderma-Associated Pulmonary Arterial Hypertension - Flowchart
Approach to Scleroderma-Associated Pulmonary Arterial Hypertension - Flowchart Scleroderma (Systemic Sclerosis) Scleroderma (Systemic Sclerosis)
«Flowchart»

ECHO Doppler clinical assessment

ECHO Doppler clinical assessment

ECHO Doppler clinical assessment

ECHO Doppler clinical assessment


eRVSP <45 mm Hg
DLCO >55% predicted
No unexplained dyspnea
No cardiac disease


eRVSP <45 mm Hg
DLCO >55% predicted
No unexplained dyspnea
No cardiac disease


eRVSP <45 mm Hg
DLCO >55% predicted
No unexplained dyspnea
No cardiac disease eRVSP <45 mm Hg


eRVSP >45 mm Hg or eRVSP 34–40 mm Hg and unexplained dyspnea
DLCO <55% with FVC and TLC >80%
Declining DLCO (>20%) with dyspnea


eRVSP >45 mm Hg or eRVSP 34–40 mm Hg and unexplained dyspnea
DLCO <55% with FVC and TLC >80%
Declining DLCO (>20%) with dyspnea


eRVSP >45 mm Hg or eRVSP 34–40 mm Hg and unexplained dyspnea
DLCO <55% with FVC and TLC >80%
Declining DLCO (>20%) with dyspnea eRVSP >45 mm Hg or eRVSP 34–40 mm Hg and unexplained dyspnea

FOLLOW

FOLLOW

FOLLOW

End

End

End

PAH confirmed
Resting mean PAP ≥25 mm Hg or exercise
PAP ≥30 mm Hg and PCW ≤15 mm Hg

PAH confirmed
Resting mean PAP ≥25 mm Hg or exercise
PAP ≥30 mm Hg and PCW ≤15 mm Hg

PAH confirmed
Resting mean PAP ≥25 mm Hg or exercise
PAP ≥30 mm Hg and PCW ≤15 mm Hg

PAH confirmed
≥
≥ ≤

Right heart catheterization
± exercise, ± vasodilator challenge

Right heart catheterization
± exercise, ± vasodilator challenge

Right heart catheterization
Right heart catheterization

Exclude other causes of PH


Thromboembolic disease
Pulmonary venous hypertension (left heart disease)
Hypoxemic pulmonary disease (COPD, sleep disorder, ILD)

Exclude other causes of PH

Exclude other causes of PH


Thromboembolic disease
Pulmonary venous hypertension (left heart disease)
Hypoxemic pulmonary disease (COPD, sleep disorder, ILD)


Thromboembolic disease
Pulmonary venous hypertension (left heart disease)
Hypoxemic pulmonary disease (COPD, sleep disorder, ILD) Exclude other causes of PH

THERAPY

THERAPY

THERAPY

Unchanged or failure

PDE5i + ERA

Unchanged or failure

PDE5i + ERA

Unchanged or failure

Unchanged or failure

PDE5i + ERA

PDE5i + ERA

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA PDE5i or ERA; PDE5i + ERA

Functional class IV

Functional class IV

Functional class IV

PDE5i + ERA + prostacyclin (inhaled) or PRA

PDE5i + ERA + prostacyclin (inhaled) or PRA

PDE5i + ERA + prostacyclin (inhaled) or PRA

PDE5i + ERA + prostacyclin (inhaled) or PRA

Lung transplant referral

Lung transplant referral

Lung transplant referral

Lung transplant referral

IV or SC prostacyclin

IV or SC prostacyclin

IV or SC prostacyclin

IV or SC prostacyclin

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA PDE5i or ERA; PDE5i + ERA

Lung transplant referral

Lung transplant referral

Lung transplant referral Lung transplant referral

Functional class I/II

Functional class I/II

Functional class I/II

Functional class III

Functional class III

Functional class III

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA

PDE5i or ERA; PDE5i + ERA PDE5i or ERA; PDE5i + ERA

Routine baseline and annual assessment

Routine baseline and annual assessment

Routine baseline and annual assessment

Routine baseline and annual assessment

Advanced assessment

Advanced assessment

Advanced assessment

Advanced assessment

Risk stratification


WHO functional class
Submaximal exercise testing (6-minute walk)
Hemodynamic assessment (peak PA pressure, CI, PVR)

Risk stratification


WHO functional class
Submaximal exercise testing (6-minute walk)
Hemodynamic assessment (peak PA pressure, CI, PVR)

Risk stratification

Risk stratification


WHO functional class
Submaximal exercise testing (6-minute walk)
Hemodynamic assessment (peak PA pressure, CI, PVR)


WHO functional class
Submaximal exercise testing (6-minute walk)
Hemodynamic assessment (peak PA pressure, CI, PVR)