What is the management and treatment approach for a patient with Interstitial Lung Disease (ILD)?

Management and Treatment Approach for Interstitial Lung Disease (ILD)

The management of Interstitial Lung Disease requires a multidisciplinary approach with treatment tailored to the specific ILD subtype, focusing on slowing disease progression, improving quality of life, and reducing mortality.

Diagnostic Evaluation

• Conduct thorough symptom assessment focusing on cough and dyspnea on exertion, which are key symptoms of ILD 1, 2
• Perform lung auscultation to detect inspiratory crackles, which can aid in early identification of ILD 1, 2
• Obtain High-Resolution CT (HRCT) scan as the gold standard for diagnosing ILD, with approximately 91% sensitivity and 71% specificity for diagnosing ILD subtypes 1, 3
• Complete pulmonary function tests (PFTs) including FVC, TLC, and DLCO measurements to establish baseline lung function and provide prognostic information 1, 2
• Consider 6-minute walk test (6MWT) to evaluate exercise capacity and detect exercise-induced oxygen desaturation 2, 3

Classification and Risk Assessment

• Identify the specific ILD pattern on HRCT (UIP/probable UIP, fibrotic hypersensitivity pneumonitis, or fibrotic NSIP) 1
• Assess for risk factors of progression including male gender, smoking history, longer disease duration, and high autoantibody titers in connective tissue disease-associated ILD 4
• A 5% decline in forced vital capacity (FVC) over 12 months is associated with an approximately 2-fold increase in mortality 3
• Lung biopsy is generally not recommended for baseline evaluation of ILD unless specific indications exist 1

Treatment Approaches by ILD Subtype

Idiopathic Pulmonary Fibrosis (IPF)

• Antifibrotic therapy with nintedanib or pirfenidone is strongly recommended as first-line treatment for IPF, as these medications slow annual FVC decline by approximately 44% to 57% 5, 3
• Pirfenidone has demonstrated efficacy in reducing FVC decline compared to placebo in multiple clinical trials, with a mean treatment difference of 193 mL at Week 52 5
• Avoid long-term glucocorticoids in IPF as they provide no benefit and may increase mortality 1

Connective Tissue Disease-Associated ILD (CTD-ILD)

• For CTD-ILD, immunomodulatory therapy is the cornerstone of treatment 1
• Mycophenolate mofetil is the preferred first-line agent for most CTD-ILD subtypes 1, 4
• Rituximab is particularly beneficial for patients with active inflammatory arthritis alongside ILD 4
• Azathioprine is another recommended first-line option for CTD-ILD 1, 4
• Cyclophosphamide can be considered for severe or rapidly progressive CTD-ILD 1, 4
• Short-term glucocorticoids (≤3 months) may be used as part of initial therapy 1, 4

Inflammatory Myopathy-Associated ILD (IIM-ILD)

• Calcineurin inhibitors (CNIs), particularly tacrolimus, are recommended as first-line treatment options for IIM-ILD 1
• For other SARD-ILD subtypes, CNIs are not recommended as first-line therapy 1

Progressive-Fibrosing ILD (PF-ILD)

• For progressive ILD of any cause, nintedanib is conditionally recommended 4, 6
• Pirfenidone can be added as a treatment option for progressive disease 4, 5
• Tocilizumab is conditionally recommended for CTD-ILD that progresses despite first-line treatment 4

Monitoring and Follow-up

• Schedule short-term PFTs within 3 months of initial evaluation to determine rate of progression 1, 2
• Consider repeat HRCT within 6 months of initial evaluation to assess for radiological progression 1, 2
• For mild ILD (FVC ≥70% and <20% fibrosis extent on HRCT), schedule PFTs every 6 months for the first 1-2 years 1
• For moderate-to-severe ILD or progressive disease, increase PFT frequency to every 3-6 months 1

Supportive Care

• Provide supplemental oxygen for patients who desaturate below 88% on exertion, as it reduces symptoms and improves quality of life 3
• Implement structured exercise therapy to reduce symptoms and improve 6-minute walk test distance 3
• Consider pulmonary rehabilitation for symptomatic patients 3
• Evaluate for pulmonary hypertension, which develops in up to 85% of patients with end-stage fibrotic ILD 3

Advanced Disease Management

• Consider early referral for lung transplantation evaluation for patients with advanced or rapidly progressive disease 1, 3
• After lung transplant, patients with ILD have a median survival of 5.2 to 6.7 years compared with a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant 3
• Implement early palliative care for symptom management in advanced disease 7

Multidisciplinary Approach

• Establish a multidisciplinary team involving pulmonologists, radiologists, rheumatologists, and pathologists for optimal management 1, 4
• The multidisciplinary team should integrate HRCT findings, PFT results, and clinical symptoms to determine disease severity and treatment approach 1, 2