Pulmonary hypertension in chronic interstitial lung diseases

Abstract

Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. The degree of PH in ILDs is typically mild-to-moderate. However, some of these patients may develop a disproportionate increase in PH that cannot be justified solely by hypoxia and parenchymal injury: this condition has been termed “out-of-proportion” PH. The pathogenesis of PH in these diseases is various, incompletely understood and may be multifactorial. The clinical suspicion (i.e. increased dyspnoea, low diffusion capacity) and echocardiographic assessment are the first steps towards proper diagnosis of PH; however, right heart catheterisation remains the current gold standard for diagnosis of PH. At present, no specific therapies have been approved for the treatment of PH in patients with ILDs

Physiology of the lung in idiopathic pulmonary fibrosis

“…Right heart catheterisation is the gold standard for the diagnosis of PH, which is defined as a mean pulmonary artery pressure (mPAP) ⩾25 mmHg at rest [86].…”

A Clinical and Radiological Profile of Patients of Interstitial Lung Diseases, Attending the Chest Hospital of Medical College From North India

“…5 A study from Denmark reported pulmonary hypertension in 14% with mixed types of ILD and who were screened for PH by echocardiography and concluded that 8% of them had mild PH (Mean PAP >25 mmHg, but <35 mmHg) and 6% of them had severe PH (Mean PAP >35 mmHg). [23][24] Hamada et al observed that the prevalence of PH in this patient population was as low as 8.1%. 25 Abeles AM et al reported that C-Reactive Protein was raised in 45.2% cases and was the most consistent finding followed by serum ANA positivity in 17.8% cases (Maximum in IPF: 27.6%) and also found that Serum DsDNA was positive in 6.9% cases with IPF and 20% in CTD associated ILDs.…”

A new definition: Combined pulmonary fibrosis and emphysema syndrome

“…Pulmoner hipertansiyon (PAH) KOAH ve IPF'nin en önem-li komplikasyonudur ve kötü prognozla ile ilişkilendirilir (1,11). KPFA sendromunda da PAH, KOAH ve IPF'ye göre daha sık görülür ve daha ciddi seyreder (1,3,11,12). PAH prevelansı KOAH'ta %50, , IPF'de %31-46 oranında iken, KPFA' da PAH gelişimi %47-90 hastada bildirilmiştir (1,3,11,12).…”

“…KPFA sendromunda da PAH, KOAH ve IPF'ye göre daha sık görülür ve daha ciddi seyreder (1,3,11,12). PAH prevelansı KOAH'ta %50, , IPF'de %31-46 oranında iken, KPFA' da PAH gelişimi %47-90 hastada bildirilmiştir (1,3,11,12). Sağ kalp kateterizasyonu ile doğrulanan PAH gelişen 40 KPFA'lı hastanın bir yıllık sağkalım oranı %60 bulunmuştur (13).…”