The American College of Rheumatology (ACR) released 2 clinical practice guidelines for the screening, monitoring, and treatment of interstitial lung disease (ILD) in patients with systemic autoimmune rheumatic diseases (SARDs). These conditions include rheumatoid arthritis, systemic sclerosis (SSc), Mixed Connective Tissue Disease (MCTD), Sjögren's Disease (SjD), and idiopathic inflammatory myopathies (IIM), all of which are associated with the greatest risk of developing ILD. Recommendations aim to help the identification of ILD among this patient population and to aid in improving the co-management of patients with SARDs-ILD by pulmonologists and rheumatologists.

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“Interstitial lung disease is a major cause of morbidity and mortality across several systemic autoimmune rheumatic diseases,” said Sindhu R Johnson, MD, PhD, lead author on the guidelines and director of the University of Toronto’s clinical epidemiology & healthcare research program, in a statement. “Guidance was needed for which tests to use for screening and monitoring this particular disease.”

Highlights

The following recommendations are for the screening of SARD-ILD:

• It is conditionally recommended to use pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) of the chest to screen for ILD in patients with SARDs at an increased risk of developing ILD
• It is conditionally recommended against screening with 6-minute walk distance (6MWD), chest radiography, bronchoscopy, and ambulatory desaturation testing for patients with SARDs at an increased risk for developing ILD
• It is strongly recommended against screening with surgical lung biopsy for patients with SARDs at an increased risk of developing ILD

The following recommendations are for the monitoring of ILD progression:

• It is conditionally recommended to monitor with PFTs, HRCT, and ambulatory desaturation therapy in patients with SARD-ILD
• It is conditionally recommended to monitor with PFTs and HRCT chest over PFTs alone
• It is conditionally recommended against monitoring with 6MWD, bronchoscopy, and chest radiography in patients with SARD-ILD
• It is suggested patients with IIM-ILD and SSc-ILD receive PFTs every 3-6 months for the first year, then less frequently once stable
• It is suggested patients with RA-ILD, MCTD-ILD, and SjD-ILD receive PFTs every 3-12 months for the first year, then less frequently once stable
• No guidance was provided regarding the frequency of routine HRCT chest for monitoring ILD in patients with SARD-ILD, although HRCT is suggested when clinically indicated
• It is suggested patients with SARD-ILD receive an ambulatory desaturation assessment every 3-12 months

The following recommendations are for the first-line ILD treatment in patients with SARDs-ILD:

• Glucocorticoids are conditionally recommended as a first-line treatment for patients with SARD-ILD, except for SSc-ILD
• It is strongly recommended against prescribing glucocorticoids as a first-line ILD treatment for patients with SSc-ILD and instead tocilizumab is conditionally recommended for these patients as well as those with MCTD-ILD
• It is conditionally recommended against using tumor necrosis factor inhibitors, methotrexate, abatacept, and leflunomide as first-line ILD treatment options
• It is conditionally recommended against pirfenidone as a first-line ILD treatment option
• It is conditionally recommended patients with IIM-ILD receive Janus kinase (JAK) inhibitors, although it is conditionally recommended all other patients with SARD-ILD do not receive JAK inhibitors as first-line treatment
• Similarly, it is conditionally recommended patients with IIM-ILD can receive calcineurin inhibitors (CNI) as a first-line ILD treatment, although CNI is conditionally recommended against for all other patients with SARD-ILD
• Optimal medical management over referral for stem cell or lung transplant as first-line ILD treatment is conditionally recommended

The following are recommendations for the management of SARD-ILD despite first-line ILD treatment:

• Long-term glucocorticoid use is strongly recommended against for patients with SSc-ILD progression and conditionally recommended against for other patients with SARD-ILD
• Mycophenolate, nintedanib, rituximab, and cyclophosphamide are conditionally recommended for this patient population
• Pirfenidone is conditionally recommended against for patients with SARD-ILD, except for those with RA-ILD progression, in which it is conditionally recommended
• CNIs are conditionally recommended against in patients with SARD-ILD, except for those with IIM-ILD, in which it is conditionally recommended
• It is conditionally recommended against using plasma exchange as a treatment option
• Stem cell transplantation and/or lung transplantation was conditionally recommended for patients with SSc-ILD progression despite first ILD treatment

“We know that early detection and hastened referral to care, in collaboration with pulmonology, is critical for the best patient outcomes,” stated Sonye K Danoff, MD, PhD, guideline author, pulmonologist and director of the Interstitial Lung Disease/ Pulmonary Fibrosis program at Johns Hopkins University School of Medicine. “Because symptoms of ILD (cough, shortness of breath, fatigue) can be subtle or result from other common diseases, the diagnosis of ILD can be delayed. Increasing awareness of the groups at highest risk for developing ILD and implementing appropriate screening and treatment practices should have long-term benefits.”