11th January 2024

Management

ILD management is led by respiratory physicians, but it is important to understand general principles of management and to familiarise yourself with the most commonly used medications. The general principle of management is that inflammatory ILDs are mainly treated with immunosuppressive drugs, while fibrotic ones are treated with fibrinolytics; a combination of the two can be used when both elements are present.

Conservative measures

Supportive care plays a crucial role and should be considered in all patients at the time of diagnosis.

• Pulmonary rehabilitation – every patient diagnosed with ILD should be assessed for the need for pulmonary rehabilitation. It should be offered to those whose quality of life is significantly affected by the disease, e.g. those with low exercise tolerance. Sessions include information on the condition, nutritional advice, breathing exercises, psychological support, and social network opportunities.
• Long-term oxygen therapy (LTOT) – many patients with ILD may require supplemental oxygen if/as the disease progresses; this is particularly relevant for IPF patients who almost universally develop hypoxia. It is important to recognise the point where it needs to be initiated in order to preserve patient’s quality of life, as well as avoid or minimise complications of chronic hypoxia, such as pulmonary hypertension and polycythaemia.
• Smoking cessation – should be advised to all patients with ILD
• Minimising risk of chest infections – annual flu vaccination and pneumococcal vaccination should be offered to all patients. Those with frequent exacerbations can be offered prophylactic antibiotics. Whenever possible, antibiotic choice should be guided by sensitivities of patient’s sputum culture.

Idiopathic pulmonary fibrosis

Fibrinolytics

• Pirfenidone and Nintedanib are the only two drugs approved for treatment of ILD in the UK.
• Pirfenidone has both anti-inflammatory and anti-fibrotic properties. It inhibits the production of transforming growth factor-β1 (TGF-β1), a cytokine involved in differentiation of human lung fibroblasts into myofibroblasts, thereby attenuating excess collagen and extracellular matrix deposition. Its anti-inflammatory properties come from its inhibition of several pro-inflammatory cytokines, including TNF-α, interleukin-1 (IL-1) and interferon-gamma (IFN-γ).
• Nintedanib is a tyrosine kinase inhibitor which inhibits multiple growth factors. Consequently, it prevents the proliferation and activation of fibroblasts and attenuates angiogenesis within the lungs. The result is a slowing down of fibrosis as well as associated complication of pulmonary hypertension.
• Both drugs were shown to slow down deterioration in FVC and DLCO when compared to non-treated cohort with IPF2. Patients with FVC of 50% to 80% are offered one of the medications. Treatment is stopped if FVC declines by >10% in 12 months while on the drug.
• They are generally well tolerated by patients; main side effect is gastrointestinal upset, which settles after a couple of months in most patients.
• Both medications are contraindicated in pregnancy and severe liver impairment.

Lung transplantation

• Patients with IPF should be referred for consideration of lung transplant soon after the diagnosis if there are no absolute contraindications and the patient is deemed to be fit enough for the procedure.

Hypersensitivity pneumonitis 

• Avoidance of the causative antigen – for many patients, avoiding the culprit antigen is enough to alleviate symptoms, especially if the disease is caught early in acute stage. The time between antigen avoidance and symptom improvement is antigen and patient-dependant; re-evaluation should be done at 8-12 weeks.
• Corticosteroids – typically given during the acute HP flare for a short period of time. In chronic HP, trial of long-term steroids is recommended but is more likely to be successful in patients with inflammatory features, such as characteristic ground-glass opacities on CT or lymphocytosis on BAL.
• Lung transplantation

Sarcoidosis

About half of patients with sarcoidosis require treatment, which depends on stage of the condition.

• Asymptomatic, stage 1: usually only observation is required
• Symptomatic, stage 1: oral corticosteroids. Consider gastric protection and osteoporosis prophylaxis if steroids are used long-term. However, vitamin D and calcium supplementation are often avoided due to risk of hypercalcaemia in sarcoidosis
• Symptomatic, stage 2 and above: oral corticosteroids are a mainstay of treatment. Cytotoxic medications such as methotrexate or azathioprine may be considered, but this should always be discussed with a respiratory consultant. If symptoms persist, or there is evidence of progression, biological therapies can also be given, e.g. infliximab.
• Lung transplantation

References

• Idiopathic pulmonary fibrosis in adults: diagnosis and management. NICE clinical guideline. www.nice.org.uk/guidance/cg163, ISBN: 978-1-4731-0179-1
• Stefania Cerri et al., Real-life comparison of pirfenidone and nintedanib in patients with idiopathic pulmonary fibrosis: A 24-month assessment. Respiratory Medicine, Volume 159, 2019, 105803, ISSN 0954-6111
• Talmadge E King, Hypersensitivity pneumonitis (extrinsic allergic alveolitis): Treatment, prognosis, and prevention. Uptodate.com, accessed on 17/01/2023.
• Leslie Grammer. Hypersensitivity pneumonitis. BMJ Best Practice, last updated on 01/11/2022, accessed on 17/01/2023.
• Dr Colin Tidy, Sarcoidosis. Edited on 15/08/2022, accessed on 01/01/2023. https://patient.info/doctor/sarcoidosis-pro