Early and accurate diagnosis of all ILDs is essential in formulating and instituting a timely management plan. Inova adopts a multidisciplinary, multiprong approach that includes diagnosis confirmation, consideration of medical therapies, clinical trials, other ancillary interventions, and, when all else fails, the option of lung transplantation.
Following are guidelines to help you identify interstitial lung disease in your patients. We also invite you to refer patients to us for diagnosis.
STEP 1: RECOGNIZE ILD
Typical presentation includes subacute or chronic cough and exertional dyspnea (breathlessness).
1. While performing an H&P
- Listen carefully for crackles, especially at the lung bases
- Look for evidence of autoimmune disease based on a comprehensive history and physical exam
- Walk your patient informally in the hallway to assess for desaturation (>3% drop)
2. Order initial testing based on your H&P and consider the following:
- Spirometry (normal or low FVC, often with a normal FEV1/FVC ratio in ILD)
- Lung Volumes (normal or low TLC)
- DLCO (frequently reduced in ILD)
- Chest X-ray (CXR) (unexplained, persistent markings)
- Please note that CXR and lung function tests can often appear normal in the context of mild ILD
3. If you suspect ILD, perform a high-resolution CT chest scan. HRCT should include the following:
- Inspiratory and expiratory supine imaging
- High-spatial frequency algorithm reconstruction at 0.625 to 2.5mm thickness
- No IV contrast required
- Consider prone imaging when mild dependent ground-glass attenuation is present
STEP 2: ATTEMPT TO IDENTIFY THE CAUSE OF ILD
Step 2A: Perform a Detailed History and Physical
Below is a list of diseases and questions/findings to consider
- Connective Tissue
Joints, skin, Raynaud’s, reflux, dry eyes/mouth, muscle weakness Disease-related ILD or pain - Chronic Hypersensitivity Pneumonitis
Exposure to mold sources (forced air heating, hot tubs, humidifiers, water damage, or visible mold), Exposure to birds, down bedding, farming, or agriculture - Drug/Radiation-induced ILD
Chemotherapy, amiodarone, nitrofurantoin, other drugs Check PneumoTox.com. Radiation therapy to the chest - Familial ILD
Family history of ILD, Sarcoidosis, home oxygen use, autoimmune disease - Pneumoconioses
Occupational history - Vasculitis
Sinus disease, hoarseness, hematuria, hemoptysis
Step 2B: Order Relevant Blood Tests
- Eosinophilic pneumonia: CBC with differential • Chronic Hypersensitivity Pneumonitis: HP panel (controversial)
- CTD-ILD: ESR, CRP
- Idiopathic inflammatory myositis: ANA, Jo-1, CK, myoglobin, aldolase, consider myositis panel
- Rheumatoid Arthritis: RF, CCP
- Sarcoidosis: Serum calcium
- Scleroderma/MCTD: ANA, Scl70, centromere, U1RNP
- Sjogren’s: ANA, Ro/SSA, La/SSB
- Vasculitis: Anti-PR3 and MPO (ANCA), creatinine
Step 2C: Consider Surgical Lung Biopsy
Contact our multidisciplinary Interstitial Lung Disease team at 703-776-7939 for a consultation, during which a decision will be made regarding the need for a surgical lung biopsy. We will take multiple appropriately sized biopsies from 2–3 lobes (not just the lingula or middle lobe). Our pulmonary pathologist with expertise in ILD will review the biopsy.
ILD Management Checklist
- Recommend smoking cessation
- Advise patients to purchase a pulse oximeter to monitor oxygen levels immediately after activity: stairs, hills, level ground, exercise, showering
- Prescribe different oxygen prescriptions at rest, with routine activities, with exercise, and with sleep to maintain oxygen levels over 90% 24 hours per day
- Consider nocturnal oximetry or polysomnography
- Prescribe pulmonary rehabilitation
- Advise patients to achieve a healthy weight
- Administer influenza, pneumococcal and COVID-19 vaccinations, and COVID-19 boosters
- Provide information about your local PFF support group
- Consider lung transplant evaluation
- Consider clinical trial enrollment
- Consider pharmacological treatment after a diagnosis is confirmed
This content was adapted from the Pulmonary Fibrosis Foundation ILD Pocket Guide