Idiopathic pulmonary fibrosis: current diagnosis and treatment

Abstract

Idiopathic pulmonary fibrosis (IPF) is a devastating chronic lung disease without a clear recognizable cause. IPF has been at the forefront of new diagnostic algorithms and treatment developments that led to a shift in patients' care in the past decade, indeed influencing the management of fibrotic interstitial lung diseases other than IPF itself. Clinical presentation, pathophysiology, and diagnostic criteria are briefly addressed in this review article. Additionally, evidence regarding the use of antifibrotics beyond the settings of clinical trials, impact of comorbidities, and therapeutic approaches other than pharmacological treatments are discussed in further detail.

Figure 1. HRCT patterns in relation to usual interstitial pneumonia (UIP). In A, UIP pattern, showing exuberant honeycombing. In B, probable UIP pattern, with traction bronchiectasis and typical subpleural inferior distribution. In C, indeterminate for UIP, showing mild reticular and ground-glass opacities.

Figure 2. Probability of idiopathic pulmonary fibrosis based on clinical and radiological features. ENA: extractable nuclear antigen; UIP: usual interstitial pneumonia; NSIP: nonspecific interstitial pneumonia.

Figure 3. Main comorbidities in idiopathic pulmonary fibrosis.

Figure 4. Mechanical ventilation parameters in a patient with acute exacerbation of idiopathic pulmonary fibrosis. Mechanical ventilation in this setting can be very challenging: note the high concentrations of oxygen (Fio₂ = 75%) and low static lung compliance (estimated on 11 mL/cmH₂O), with high driving pressure swings (22 cmH₂O) even with high respiratory rates (42 breaths/min) to prevent severe respiratory acidosis.

Figure 5. Management of patients with idiopathic pulmonary fibrosis.