Pulmonary hypertension in patients with moderate to severe hypersensitivity pneumonitis.
Pulmonary hypertension is common in severe hypersensitivity pneumonitis with interstitial lung disease.
Pulmonary hypertension in patients with moderate to severe hypersensitivity pneumonitis.
Hypersensitivity pneumonitis (hypersensitivity pneumonitis) is characterized by a complex immunological reaction of the lung that may lead to chronic interstitial lung disease (interstitial lung disease).
We aimed to assess the prevalence of pulmonary hypertension in two independent cohorts including patients with moderate and severe hypersensitivity pneumonitis.
We retrospectively analyzed the data of hypersensitivity pneumonitis patients with severe interstitial lung disease undergoing lung transplantation (cohort-1) and invited hypersensitivity pneumonitis patients with mild disease participating at pulmonary rehabilitation for a prospective screening for pulmonary hypertension (cohort-2).
In cohort 1 (n = 55, median age 57 [95% CI 52-60] years, 59% male, forced vital capacity [FVC] 37 [35-45] % predicted), echocardiographic systolic pulmonary arterial pressure (sPAP) was available in 31 patients and showed elevated values (53 [45-63] mmHg).
Sixteen patients (29%) had a high probability of pulmonary hypertension (pulmonary hypertension; sPAP > 50 mmHg).
In cohort 2 (n = 96, median age 62 [60-65] years, 66% male, FVC 98 [95-104] % predicted), echocardiographic screening was available in 88 patients and showed upper-normal sPAP values (29 [28-31] mmHg).
One patient (1%) had a high probability of pulmonary hypertension.
Pulmonary hypertension is a frequent complication in patients with hypersensitivity pneumonitis and severe interstitial lung disease.
In hypersensitivity pneumonitis patients with mild disease, pulmonary hypertension is uncommon and alternative causes should be considered.