Interstitial Lung Disease (ILD)

There are a number of different conditions that can cause interstitial lung disease with fibrosis or diffuse lung inflammation (alveolitis):

• Idiopathic Pulmonary Fibrosis (IPF)
• Hypersensitivity Pneumonitis (organic dust exposure eg moulds, birds, hay. Many cases are idiopathic, and no obvious cause is identified)
• Connective tissue disease (eg Rheumatoid arthritis, Scleroderma, SLE, Sjogren’s Disease, Mixed Connective Tissue Disease)
• Drug reactions (eg nitrofurantoin, methotrexate, statin therapy)
• Smoking related ILD
• Sarcoidosis

Patients with ILD usually present with:

• Breathlessness on exertion
• Dry cough
• Non-specific fatigue
• Bilateral inspiratory crackles on auscultation
• Clubbing of the fingers suggests IPF
• Normal spirometry or a restrictive pattern but sometimes with an obstructive pattern as IPF may coexists with emphysema/COPD.
• Consider heart failure, bronchiectasis in differential diagnosis

Assess everyone with suspected ILD by taking a detailed history including drug, occupational and smoking history, carrying out a clinical examination and performing initial investigations to help exclude alternative diagnoses.

• Urinalysis, FBC, U&Es, Calcium, LFTs
• ECG
• Chest X-ray
• Finger probe oxygen saturations (SpO2)
• Spirometry if available – will help risk stratify but spirometry and lung diffusion (DLCO) will automatically be booked by the secondary care team
• High Resolution CT (GPs can request HRCT directly via ICE if patients have symptoms suggestive of an interstitial lung disease and a chest X-ray or spirometry (restrictive defect) suggestive of an ILD).

• Known Interstitial Lung disease/fibrosis with worsening symptoms
• Initial assessment supported by abnormal imaging suggesting new ILD/fibrosis.

SCI Gateway/Respiratory Medicine/Interstitial Lung Disease.

Non-urgent advice through SCI Gateway. Non-urgent advice through SCI Gateway.