From the Guidelines
People with systemic autoimmune rheumatic diseases (SARDs) should be screened for interstitial lung disease (ILD) according to the 2023 American College of Rheumatology (ACR)/American College of Chest Physicians (CHEST) guideline. This guideline is crucial in identifying individuals with SARDs who are at high risk of developing ILD, particularly those with rheumatoid arthritis (RA), systemic sclerosis (SSc), idiopathic inflammatory myopathies (IIM), mixed connective tissue disease (MCTD), and Sjögren disease (SjD) 1. The screening process should take into account demographics, disease manifestations, and antibody profile, as outlined in the guideline.
Some key points to consider in the screening and monitoring of ILD in people with SARDs include:
- Identifying risk factors for the development of ILD, such as demographics and disease manifestations
- Using pulmonary function tests and imaging to monitor disease progression and treatment response
- Collaborative management between rheumatologists and pulmonologists to optimize care for individuals with SARD-associated ILD
- Recognizing that ILD can be a significant cause of morbidity and mortality in people with SARDs, and that early detection and treatment can improve outcomes 1.
In terms of treatment, the approach will vary depending on the specific type and severity of ILD, as well as the underlying SARD. However, early detection and treatment of ILD can significantly improve morbidity, mortality, and quality of life outcomes. It is essential to follow the guidelines and work collaboratively with healthcare professionals to ensure the best possible outcomes for individuals with SARDs and ILD 1.
From the Research
Interstitial Lung Disease (ILD) Overview
- ILD consists of a group of pulmonary disorders characterized by inflammation and/or fibrosis of the lung parenchyma associated with progressive dyspnea that frequently results in end-stage respiratory failure 2.
- The most common forms of ILD are idiopathic pulmonary fibrosis (IPF), hypersensitivity pneumonitis, and connective tissue disease (CTD) 2.
Treatment Options for ILD
- Antifibrotic therapy with nintedanib or pirfenidone slows annual FVC decline by approximately 44% to 57% in individuals with IPF, scleroderma associated ILD, and in those with progressive pulmonary fibrosis of any cause 2.
- For connective tissue disease-associated ILD, immunomodulatory therapy, such as tocilizumab, rituximab, and mycophenolate mofetil, may slow decline or even improve FVC at 12-month follow-up 2.
- Pirfenidone has been shown to slow disease progression in patients with fibrotic ILDs other than IPF who deteriorate despite conventional therapy 3.
Efficacy of Pirfenidone and Nintedanib in ILD
- Recent data suggest that different interstitial lung diseases with a progressive pulmonary fibrosis phenotype can share similar pathogenetic and biological pathways and could be amenable to antifibrotic therapies 4.
- Nintedanib has been shown to be effective in slowing disease progression in patients with interstitial lung diseases other than idiopathic pulmonary fibrosis 4.
- Results on the use of pirfenidone in interstitial lung diseases other than idiopathic pulmonary fibrosis should be interpreted with more caution due to trial limitations 4.