Current Management Approaches for Interstitial Lung Disease (ILD)

Multidisciplinary Diagnosis and Classification

The European Respiratory Society recommends a multidisciplinary approach as the cornerstone of effective ILD management, with treatment strategies tailored to specific ILD subtypes and disease behavior patterns 1, 2
Multidisciplinary discussion (MDD) involving pulmonologists, rheumatologists, radiologists, and pathologists is essential for accurate diagnosis and improved patient outcomes 1
Cases that are "unclassifiable" often prove to be related to connective tissue disease or drug-induced rather than idiopathic on MDD 3
Virtual organization of multidisciplinary meetings has enabled broader access to expert consultation for complex cases 1

ILD management should be based on disease behavior patterns, which can be categorized as reversible and self-limited, reversible with risk of progression, stable with residual disease, progressive, irreversible with potential for stabilization, and progressive, irreversible despite therapy 3
The American Thoracic Society recommends treatment goals and monitoring strategies for each disease behavior pattern, including removal of possible cause, achievement of response, maintenance of status, stabilization, and slowing of progression 3

The European League Against Rheumatism recommends mycophenolate as the preferred first-line therapy across all SARD-ILD subtypes 5, 4
Rituximab, azathioprine, and cyclophosphamide are conditionally recommended as first-line options for many SARD-ILD subtypes 5, 4
Tocilizumab and nintedanib are conditionally recommended for SSc-ILD, while JAK inhibitors and calcineurin inhibitors are conditionally recommended for IIM-ILD 5, 4

The American Thoracic Society recommends short-term PFTs and HRCT to determine the rate of progression, with follow-up intervals depending on disease severity and progression rate 2
Patients with mild ILD should have PFTs scheduled every 6 months for the first 1-2 years, while those with moderate-to-severe ILD or progressive disease require more frequent monitoring 2

High-risk features for ILD progression include demographic and clinical factors, genetic factors, imaging features, and physiologic factors, such as family history of pulmonary fibrosis, older age, smoking history, and abnormal lung function tests 7

Several biomarkers in blood and bronchoalveolar lavage have been associated with poor survival in ILD, including SP-A, SP-D, KL-6, and oxidative stress levels 3