Complications
Pulmonary hypertension in patients with interstitial lung disease (PH-ILD) is classified as WHO Group 3.[140] Mild pulmonary hypertension is common in idiopathic pulmonary fibrosis (IPF), with a mean pulmonary artery pressure ≥25 mmHg (normal, 8-20 mmHg) identified in 8% to 15% of patients at presentation, increasing to 30% to 50% with advanced disease and >60% with end-stage disease.[141] Its presence has a strong negative impact on prognosis.[142][143][144]
The 2011 ATS/ERS/JRS/ALAT guidelines weakly recommended against the use of systemic treatments for PH in IPF, the 2015 guidelines deferred reassessment, and a 2022 update offered no new recommendations.[3][4][5] Therefore, a trial of vasomodulatory therapy may be reasonable in a select minority of patients only.[3]
Add-on sildenafil to either nintedanib or pirfenidone has been shown to provide no symptomatic benefit compared with either treatment alone.[145][146][147]
One network meta-analysis reported that sildenafil probably reduces acute exacerbations, hospitalizations, and mortality, while the combination of nintedanib plus sildenafil probably reduces the decline in overall forced vital capacity.[79]
Treprostinil is used for the treatment of pulmonary hypertension in patients with ILD (PH-ILD; WHO Group 3) to improve exercise ability. Although the inhaled route is thought to mitigate systemic adverse effects, the risks and benefits associated with long-term use merit further investigation.[148][149]
Patients with idiopathic pulmonary fibrosis (IPF) seem to have an increased risk of bronchogenic carcinoma.[150][151] The frequency of lung cancer in patients with IPF in studies ranges from 4% to 48%. The mechanism underlying the association is unknown.[152]
Antifibrotic therapy is possibly associated with a reduced risk of lung cancer in patients with IPF and may confer a survival benefit in patients with comorbid lung cancer.[153]
Gastroesophageal reflux is prevalent in patients with idiopathic pulmonary fibrosis.
The 2022 ATS/ERS/JRS/ALAT guidelines conditionally recommended against the medical or surgical treatment of asymptomatic gastroesophageal reflux for the purpose of improving respiratory outcomes.[5][99][Evidence C]
Symptomatic reflux should be treated as usual
Worse survival rates in patients with viral or bacterial pulmonary infection indicates that infection may drive idiopathic pulmonary fibrosis progression.[34][35][154] Coinfection with viruses and bacteria has been associated with significantly higher mortality and worse respiratory outcomes compared with either infection alone.[34]
Compared with several other forms of interstitial lung disease, pneumothorax is an unusual complication.
However, because of the decreased compliance and architectural distortion of the lung, pneumothorax may be harder to treat in patients with idiopathic pulmonary fibrosis than in other patients.
Can be a cause of an acute deterioration in a patient with idiopathic pulmonary fibrosis (IPF). May account for 3% to 7% of deaths in IPF patients.[155]
It has been suggested that patients with idiopathic pulmonary fibrosis have an increased risk of adverse cardiovascular events, including DVT.[156]
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