Abstract
Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases and a leading cause of death in these patients. A proportion of patients with autoimmune ILDs develop a progressive fibrosing form of ILD, characterized by increasing fibrosis on high-resolution computed tomography, worsening of lung function, and early mortality. Autoimmune disease–related ILDs have a variable clinical course and not all patients will require treatment, but all patients should be monitored for signs of progression. Apart from systemic sclerosis–associated ILD, the limited evidence to support the efficacy of immunosuppression as a treatment for ILDs is based mainly on small retrospective series and expert opinion. Non-clinical data suggest that there are commonalities in the mechanisms that drive progressive fibrosis in ILDs with an immunological trigger as in other forms of progressive fibrosing ILD. This suggests that nintedanib and pirfenidone, drugs known to slow disease progression in patients with idiopathic pulmonary fibrosis, may also slow the progression of ILD associated with systemic autoimmune diseases. In the SENSCIS® trial, nintedanib reduced the rate of ILD progression in patients with systemic sclerosis–associated ILD. The results of other large clinical trials will provide further insights into the role of anti-fibrotic therapies in the treatment of autoimmune disease–related ILDs.
Highlights
Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases including systemic sclerosis (SSc) [1], rheumatoid arthritis (RA) [2], mixed connective tissue disease (MCTD) [3], polymyositis/dermatomyositis [4], and Sjögren’s syndrome [5]
In an analysis of 167 patients with RA-ILD referred to a tertiary care center, the proportion of patients with forced vital capacity (FVC) < 50% predicted increased from 14% at diagnosis to 22% after 5 years, while the proportion of patients with DLco < 40% predicted increased from 29% at diagnosis to 40% after 5 years; patients with a usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) were more likely to progress to DLco < 40% predicted than those with non-specific interstitial pneumonia (NSIP) [8]
ILD is a major cause of morbidity and mortality in patients with systemic autoimmune diseases
Summary
Interstitial lung disease (ILD) is a common manifestation of systemic autoimmune diseases ( referred to as connective tissue diseases) including systemic sclerosis (SSc) [1], rheumatoid arthritis (RA) [2], mixed connective tissue disease (MCTD) [3], polymyositis/dermatomyositis [4], and Sjögren’s syndrome [5]. Beyond SSc-ILD, more robust trial data are needed to determine the role of immunosuppressant therapy for autoimmune-associated ILDs. CYC, MMF, rituximab, and azathioprine are commonly used in the treatment of RA-ILD, the evidence to support their use comes solely from retrospective or observational studies [59,60,61,62]. At the latest time point with evaluable data, 30 patients (68%) did not meet the criteria for ILD progression (i.e., did not have a decline in FVC of > 10% predicted or DLco of > 15% predicted, worsening of ILD score on HRCT, or death from progressive lung disease) [62]. Based on their metabolism [98, 99], drug-drug interactions between anti-fibrotic therapies and immunosuppressant therapies are not expected, but further data are needed
Sign-in/Register to view highlights & summary Sign-in/Register to access full text options 
Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
