From the Guidelines
For people with chronic interstitial lung disease (ILD), the treatment approach should be individualized based on the underlying cause and disease severity, with a focus on reducing inflammation, preventing further scarring, and supporting respiratory function. The most recent and highest quality study, 1, provides guidance on the treatment of ILD in people with systemic autoimmune rheumatic diseases. According to this study,
- for people with SARD-ILD other than SSc-ILD, glucocorticoids are conditionally recommended as a first-line ILD treatment,
- while for people with SSc-ILD, glucocorticoids are strongly recommended against as a first-line ILD treatment. Some key points to consider in the treatment of ILD include:
- The use of mycophenolate, azathioprine, rituximab, and cyclophosphamide as first-line ILD treatment options for people with SARD-ILD, as conditionally recommended by 1.
- The conditional recommendation against leflunomide, methotrexate, TNFi, and abatacept as first-line ILD treatment options for people with SARD-ILD, as stated in 1.
- The use of nintedanib as a first-line ILD treatment option for people with SSc-ILD, as conditionally recommended by 1, but not for people with SjD-ILD, IIM-ILD, and MCTD-ILD.
- The conditional recommendation against pirfenidone as a first-line ILD treatment option for people with SARD-ILD, as stated in 1.
- The importance of optimal medical management, including supportive care and treatment of underlying conditions, as emphasized in 1.
- The consideration of lung transplantation for advanced disease unresponsive to medical therapy, as mentioned in 1. Overall, the treatment of chronic ILD requires a comprehensive approach that takes into account the individual patient's needs and disease characteristics, with a focus on improving morbidity, mortality, and quality of life.
From the Research
Treatment Options for Chronic Interstitial Lung Disease
- Antifibrotic therapy with nintedanib or pirfenidone slows annual forced vital capacity (FVC) decline by approximately 44% to 57% in individuals with idiopathic pulmonary fibrosis (IPF), scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause 2
- Immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up in patients with connective tissue disease-associated ILD 2
- Structured exercise therapy reduces symptoms and improves 6-minute walk test distance in individuals with dyspnea 2
- Oxygen reduces symptoms and improves quality of life in individuals with ILD who desaturate below 88% on a 6-minute walk test 2
- Lung transplant may improve symptoms and resolve respiratory failure in patients with end-stage ILD, with a median survival of 5.2 to 6.7 years compared to a median survival of less than 2 years in patients with advanced ILD who do not undergo lung transplant 2
Antifibrotic Medications
- Pirfenidone and nintedanib are antifibrotic medications approved for IPF treatment and have been shown to reduce the rate of decline in FVC and the risk of acute exacerbation among patients with IPF 3
- Nintedanib has demonstrated efficacy in slowing disease progression in patients with interstitial lung diseases other than IPF 3
- Pirfenidone and nintedanib have side effects, including gastrointestinal symptoms, photosensitivity, and skin rashes, which can be severe and require treatment discontinuation 4
Management of Interstitial Lung Disease
- The management of connective tissue disease-associated interstitial lung disease (CTD-ILD) is complex and often based on experience rather than evidence 5
- Pharmacologic intervention with immunosuppression is usually the mainstay of therapy and is reserved for those with clinically significant and/or progressive ILD 5
- Non-pharmacologic management strategies, such as supplemental oxygen and cardiopulmonary rehabilitation, are important components of a comprehensive treatment plan in CTD-ILD 5
- Antifibrotic therapies, such as nintedanib and pirfenidone, may be useful as adjuvant to traditional immunosuppression in the management of IIM-associated ILD 6