HYPID-2 study is an extension of HYPID study (NCT01443598) :

HYPID-2 is also an observational and prospective study of patients with interstitial lung disease and pre capillary hypertension diagnosed by right heart sided catheterization.

It concerns only incident patients (i.e patients included within 6 months after PH diagnosis) whereas HYPID concerned prevalent and incident cases.

The primary aim is the same than HYPID : identify prognostic factors

Pre capillary pulmonary hypertension (PH) may be present in patients with diffuse interstitial lung disease.

In this context, PH represents an important factor of morbidity and mortality for these patients.

As in HYPID, the main purpose of HYPID-2 is to determine predictive factors of mortality within this cohort of incident patients (i.e patients included within 6 months after PH diagnosis).

In order to reach that aim,the study includes an evaluation based on exams conducted for the routine follow-up of incident patients.

Each incident patient will be followed during 2 years at least.

Inclusion Criteria:

Patients newly diagnosed with:

• Pre capillary pulmonary hypertension at right heart sided catheterization with: mPAP > or = 25 mmHg, PCWP < or = 15 mmHg
• Interstitial lung disease with diffuse infiltrative opacities on chest CT scan

Exclusion Criteria:

• Pulmonary hypertension related to a thromboembolic disease
• Respiratory disease other than diffuse interstitial lung disease
• Any etiological factor of pulmonary arterial hypertension based on NICE 2013 classification other than diffuse interstitial lung disease
• Any progressive disease associated to a life expectancy less than 6 months other than pulmonary hypertension, diffuse interstitial lung disease and respiratory insufficiency

Incident patients with pre capillary pulmonary hypertension and interstitial lung disease at HRCT