What is the management approach for chronic interstitial prominence in lungs?

From the Guidelines

The management of chronic interstitial prominence in lungs should prioritize the use of antifibrotic medications, such as nintedanib, to slow disease progression, as supported by the most recent and highest quality study 1.

Key Considerations

• The treatment approach should focus on addressing the underlying cause of the interstitial lung disease (ILD) while providing supportive care to improve quality of life and reduce morbidity and mortality.
• According to the 2023 study in Nature Reviews Rheumatology 1, medications like nintedanib and pirfenidone have been shown to slow the progression of fibrosis in ILD, making them a crucial part of the management plan.
• The use of immunosuppressants, such as mycophenolate mofetil, may also be considered in certain cases, particularly in steroid-resistant patients or to reduce steroid dependence.

Treatment Recommendations

• Nintedanib (150 mg twice daily) is recommended as a first-line treatment for chronic interstitial prominence in lungs to slow disease progression 1.
• Pirfenidone (801 mg three times daily) may also be considered as an alternative antifibrotic medication.
• Supplemental oxygen should be provided to maintain oxygen saturation above 88% to support respiratory function.
• Pulmonary rehabilitation and treatment of comorbidities, such as gastroesophageal reflux and sleep-disordered breathing, are also essential components of the management plan.

Rationale

• The 2023 study in Nature Reviews Rheumatology 1 provides the most recent and highest quality evidence for the management of chronic interstitial prominence in lungs, prioritizing the use of antifibrotic medications to slow disease progression.
• The American Thoracic Society, European Respiratory Society, Japanese Respiratory Society, and Asociacion Latinoamericana de Torax guideline update in 2022 1 also supports the use of antifibrotic medications, such as nintedanib, in the treatment of idiopathic pulmonary fibrosis and progressive pulmonary fibrosis.

From the FDA Drug Label

The efficacy of pirfenidone was evaluated in patients with IPF in three phase 3, randomized, double-blind, placebo-controlled, multicenter trials (Studies 1,2, and 3) Study 1 was a 52-week trial comparing pirfenidone 2,403 mg/day (n=278) versus placebo (n=277) in patients with IPF. Eligible patients were to have %FVC greater than or equal to 50% at baseline and a percent predicted diffusing capacity of the lungs for carbon monoxide (%DLCO) greater than or equal to 30% (Study 1) or 35% (Studies 2 and 3) at baseline.

The management approach for chronic interstitial prominence in lungs (also known as Idiopathic Pulmonary Fibrosis (IPF)) includes the use of pirfenidone.

• Key points:
  • Pirfenidone is administered three times daily with food.
  • The recommended dose is 2,403 mg/day.
  • Patients should have a %FVC greater than or equal to 50% at baseline and a %DLCO greater than or equal to 30% (Study 1) or 35% (Studies 2 and 3) at baseline.
  • The primary endpoint is the change in percent predicted forced vital capacity (%FVC) from baseline to study end.
  • Pirfenidone has been shown to reduce the decline in FVC volume compared to placebo in some studies 2.

From the Research

Management Approach for Chronic Interstitial Prominence in Lungs

The management of chronic interstitial prominence in lungs, also known as interstitial lung disease (ILD), involves a multidisciplinary approach to diagnosis and treatment.

• A holistic approach to patient care is essential, as patients with ILD often have a high symptom burden and considerable palliative care needs 3.
• The use of high-resolution computed tomography (HRCT) imaging is crucial in the assessment of ILD, and computer-aided CT analysis can provide objective and reproducible methods for examining ILDs 4.
• Patients with chronic obstructive pulmonary disease (COPD) and ILD may coexist, and the presence of interstitial lung abnormalities (ILA) is notable among patients with COPD, associated with poor prognosis 5.
• Early recognition of patients with ILD remains challenging, yet critically important, and expert consensus can inform early recognition and referral 6.
• Imaging surveillance is important for patients with ILD, those with ILA, and those at risk of developing ILD, although the role of imaging surveillance remains unclear in some cases 7.

Key Considerations

• A multidisciplinary approach to diagnosis, including key history and examination features, blood panel, pulmonary function tests, HRCT imaging, and when required bronchoalveolar lavage and lung biopsy results, is the gold standard for ILD diagnosis 3.
• The development of disease-modifying anti-fibrotic medications, such as nintedanib and pirfenidone, continues to shape the future management of ILD 3.
• Patients with ILA should be referred to a pulmonologist, and high-resolution CT imaging and full pulmonary function tests should be ordered if certain CT imaging findings are observed 6.
• Guidance has been established for identifying clinically relevant ILA, subsequent referral, and follow-up, laying the foundation for developing practical guidance on managing patients with ILA 6.