Management of Increased Interstitial Lung Markings
Initial Evaluation and Monitoring
- The American Thoracic Society recommends obtaining high-resolution CT chest to confirm findings, performing baseline pulmonary function testing, evaluating for underlying causes, and establishing serial monitoring with repeat CT in 2-3 years (or 12 months if high-risk features present) for patients with increased interstitial lung markings 1, 2
- The American College of Chest Physicians suggests assessing for dyspnea on exertion and non-productive cough that may be attributable to the lung findings, as any amount of dyspnea and/or cough attributed to ILD by the clinician upgrades the diagnosis from ILA to ILD 3, 4
- Baseline spirometry with FVC, total lung capacity (TLC), and diffusion capacity (DLCO) should be obtained, as any value below the lower limit of normal that is attributed to ILD upgrades the diagnosis from ILA to ILD 1, 3, 4
Risk Stratification and Prevention
- The American Thoracic Society recommends counseling on smoking cessation, implementing exposure remediation for identified environmental or occupational triggers, and ensuring age-appropriate vaccinations to reduce the risk of progression 2
- High-risk features for progression include definite fibrosis on CT, subpleural fibrotic or subpleural nonfibrotic subtypes, greater extent of abnormalities, abnormal or borderline FVC, TLC, or DLCO, family history of pulmonary fibrosis, older age, and smoking history 2
Diagnosis and Treatment
- The American College of Rheumatology suggests initiating mycophenolate mofetil as first-line immunosuppressive therapy for CTD-associated ILD, and considering rituximab or tocilizumab as alternative immunomodulatory options 6, 5
- For idiopathic pulmonary fibrosis pattern, the American Thoracic Society recommends initiating antifibrotic therapy with nintedanib or pirfenidone, as these agents slow annual FVC decline by 44-57% 6
- The American College of Chest Physicians advises against using immunosuppressive therapy for IPF, as it is not effective and may be harmful 6