PH-ILD patient characteristics and burden of disease in Europe: a clinician survey

David Montani, Stefano Ghio, Federica Meloni, John Cannon, Hilario Nunes, Jose Cifrian-Martinez, Raquel Rojo, Andreas Günther, Luke Howard, Karl Pehk, Gabriela Jeanneret

Pulmonary hypertension (PH) associated with interstitial lung disease (PH-ILD) is a rare, serious, progressive disease and there is a lack of patient characterisation within this group.

A retrospective online survey was conducted to elicit clinician insights on current disease management and clinical outcomes of PH-ILD in France, Germany, Italy, Spain, and the UK. The analysis of patient characteristics data is reported here.

Responses were submitted by 55 clinicians each managing 294 (± 500), 146 (± 214) and 38 (± 37) ILD, PH and PH-ILD patients respectively. Most patients were ≥60 y old (73%). 68% (± 20) of PH-ILD patients had their ILD diagnosed ≥2 y ago and 41% (± 24) had PH diagnosed >1 y ago. At PH diagnosis, 72% (± 21) of PH-ILD patients were at World Health Organization functional class 3—4, and 46% (± 5) had a predicted forced vital capacity of 50–70%. The most common underlying ILD was scleroderma-ILD-PH (30% ± 24 of patients), followed by idiopathic pulmonary fibrosis (29% ± 19) and combined pulmonary fibrosis and emphysema (15% ± 12). Symptoms such as dyspnoea, fatigue, and cough had a high frequency (present in >50% of patients) (100%, 80% and 58% of clinicians respectively). Reasons given for hospitalisation were right heart failure (31% ± 15), acute ILD exacerbation (27% ±17), symptom burden (21% ±16) and comorbidities (16% ±9). Yearly average inpatient admissions and emergency visits were 2.8 (±3.6) and 1.9 (±3.7) respectively (data provided by 29/55 clinicians).

Our physician survey captures current PH-ILD patient characteristics in Europe. Most have severe disease when their PH is diagnosed, suggesting a high disease burden with high rates of right heart failure and lung exacerbation.