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Exploring Treatment Options for ILD

May 13th, 2025

Interstitial lung disease (ILD) refers to a group of lung disorders that cause inflammation and scarring of lung tissue, leading to progressive breathing difficulties. While there is currently no cure for ILD, treatment focuses on controlling symptoms, slowing disease progression, and improving quality of life. If left untreated, ILD can lead to serious complications and early mortality. Management strategies typically include both pharmacological and non-pharmacological approaches, tailored to the specific ILD subtype and disease severity.


There currently is no cure for ILD and therefore management of the disease is mainly focused on controlling symptoms and slowing disease progression. Improperly treated ILD is associated with a poorer quality of life, an increased risk of life-threatening complications and early mortality [1]. The treatment of ILD usually involves a combination of both pharmacological and non-pharmacological approaches but this can vary based on the ILD subtype and disease severity.


Pharmacological

Those available pharmacological drug treatments for ILD can broadly be divided into three main categories; anti-inflammatory medications, immunosuppressants and antifibrotic medications. The treatment of ILD initially involves corticosteroids, to reduce inflammation and immunosuppressants to inhibit or reduce the immune response. Immunosuppressants are often given to reduce the amount of steroids the patient is taking particularly when the side effects are not well tolerated and consequently they are sometimes referred to as “steroid-sparing” medications. Additionally, anti-fibrotic medications are commonly given to ILD patients early in the disease course to slow the development of lung fibrosis.

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Non-pharmacological

A variety of different non-pharmacological options also exist for ILD including smoking cessation, pulmonary rehabilitation, oxygen therapy, and lung transplantation.


Smoking Cessation

Smoking is a known causative or risk factor in several subtypes of ILD. For example, a recent study reported that current or former smokers were almost 60% more likely than lifelong nonsmokers to develop IPF [2]. However, limited evidence exists for the effects of smoking cessation on ILD outcomes although a few small studies have reported radiological improvements and the stabilization of ILD symptoms [3–6]. Regardless, ILD patients who continue to smoke put themselves at risk of developing other lung conditions such as COPD and lung cancer. Therefore, it is recommended that ILD patients undertake smoking cessation treatment in an effort to stop smoking.


Pulmonary Rehabilitation

Pulmonary rehabilitation describes a program of exercise training and disease education designed especially for those with chronic lung disease who suffer from persistent breathlessness. Evidence suggests that pulmonary rehabilitation can provide both short and long term improvements in functional exercise capacity, dyspnoea and quality of life in those with ILD [7]. However, pulmonary rehabilitation is not always widely available and may not be suited for all ILD patients i.e. those with unstable cardiac disease or suffering from neuromuscular conditions.


Oxygen Therapy

Oxygen therapy is often prescribed in ILD patients who experience hypoxia at rest or on exertion. In fact estimates suggest that as many as 40% of ILD patients at some point require supplemental oxygen [8]. However limited evidence exists for the benefits of oxygen therapy on ILD outcomes although a few studies have reported that it can improve quality of life and exercise parameters in ILD patients for which hypoxia is a limiting factor [8]. The main limitation of oxygen therapy is the requirement for bulky gas cylinders which may limit its utility in improving exercise capacity.


Lung Transplant

Lung transplantation of either one of both lungs may be considered in ILD patients with severe disease who display a poor response to pharmacological treatments. Some common criteria for lung transplantation include an age below 70 and demonstrate a >10% reduction in FVC over 6 months and a <89% SaO2 or the need for oxygen at rest [9]. Evidence suggests that lung transplantation can improve survival in carefully selected ILD patients [10]. However, there is a shortage of donor organs meaning one in six patients may die before a suitable donor is identified [9]. It is important to note that lung transplantation is a complex procedure that carries a significant risk of perioperative morbidity and mortality.


Managing ILD requires a multifaceted approach, combining medications, lifestyle changes, and in some cases, advanced interventions like lung transplantation. While there is no cure, ongoing research continues to enhance available therapies, offering hope for improved outcomes. If you or a loved one are facing ILD, seeking early and specialised medical care can help optimise treatment and enhance quality of life.

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References

[1] Singh P, Ali SN, Zaheer S, et al. Cellular mechanisms in the pathogenesis of interstitial lung diseases. Pathol Res Pract [Internet]. 2023;248:154691. Available from: https://www.sciencedirect.com/science/article/pii/S0344033823003916.

[2] Baumgartner KB, Samet JM, Stidley CA, et al. Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 1997;155:242–248.

[3] Mogulkoc N, Veral A, Bishop PW, et al. Pulmonary Langerhans’ Cell Histiocytosis. Chest. 1999;115:1452–1455.

[4] Portnoy J, Veraldi KL, Schwarz MI, et al. Respiratory Bronchiolitis-Interstitial Lung Disease. Chest. 2007;131:664–671.

[5] Shinohara T, Kadota N, Hino H, et al. Improvement in idiopathic nonspecific interstitial pneumonia after smoking cessation. Respir Med Case Rep. 2015;14:7–9.

[6] Von Essen S, West W, Sitorius M, et al. Complete Resolution of Roentgenographic Changes in a Patient with Pulmonary Histiocytosis X. Chest [Internet]. 1990;98:765–767. Available from: https://www.sciencedirect.com/science/article/pii/S0012369216321237.

[7] Dowman L, Hill CJ, May A, et al. Pulmonary rehabilitation for interstitial lung disease. Cochrane Database of Systematic Reviews. 2021;2021.

[8] Clark KP, Degenholtz HB, Lindell KO, et al. Supplemental Oxygen Therapy in Interstitial Lung Disease: A Narrative Review. Ann Am Thorac Soc. 2023;20:1541–1549.

[9] Wallis A, Spinks K. The diagnosis and management of interstitial lung diseases. BMJ. 2015;350:h2072–h2072.

[10] Chambers DC, Cherikh WS, Harhay MO, et al. The International Thoracic Organ Transplant Registry of the International Society for Heart and Lung Transplantation: Thirty-sixth adult lung and heart–lung transplantation Report—2019; Focus theme: Donor and recipient size match. The Journal of Heart and Lung Transplantation. 2019;38:1042–1055.

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