ACR New Guidelines for Interstitial Lung Disease management

The American College of Rheumatology (ACR) has provided an overview of forthcoming recommendations regarding the screening, surveillance, and management of interstitial lung disease (ILD) in individuals diagnosed with systemic autoimmune rheumatic diseases.

Interstitial lung disease includes a wide array of conditions, individually relatively uncommon, but collectively found in approximately 50 people per lakh of the population. There are over 200 specific diffuse lung diseases including those characterized by fibrosing and granulomatous histopathology.

Systemic autoimmune rheumatic diseases (SARDs) encompass a range of medical conditions where the immune system mistakenly targets healthy tissues throughout the body, resulting in systemic inflammation and various rheumatic symptoms.

Major highlights from the guidelines providing evidence-based recommendations for the screening and treatment include:

Providers can screen patients at higher risk for ILD with pulmonary function tests (PFTs) and high-resolution computed tomography (HRCT) of the chest.

PFTs, chest HRCT, and ambulatory desaturation testing are conditionally recommended for monitoring ILD progression.

It is conditionally recommended that providers do not use 6-minute walk test distance, chest radiography, or bronchoscopy for screening or monitoring disease.

Guideline suggests that patients with IIM-ILD and SSc-ILD receive PFTs for monitoring every 3-6 months during the first year, then less frequently once stable.

Guideline suggests that patients with RA-ILD, SjD-ILD, and MCTD-ILD receive PFTs every 3-12 months for the first year, then less frequently once stable.

The guidelines strongly recommend against using glucocorticoids for first-line ILD treatment in patients with SSc-ILD.

Short-term glucocorticoids are conditionally recommended as a first-line ILD treatment for patients with systemic autoimmune rheumatic disease–related ILD (SARD-ILD), excluding SSc-ILD.

Mycophenolate, azathioprine, rituximab, and cyclophosphamide are all potential first-line ILD treatment options for patients with SARD-ILD.

It is conditionally recommended that patients with SARD-ILD do not receive leflunomide, methotrexate, tumor necrosis factor inhibitors, or abatacept as first-line ILD treatment.

If SARD-ILD progresses despite first-line therapy; mycophenolate, rituximab, cyclophosphamide, and nintedanib are potential secondary treatment options.

If RA-ILD progresses following initial therapy, pirfenidone is a treatment option.

The guidelines conditionally recommend against pirfenidone as a secondary treatment option for SARD-ILD other than RA-ILD.

“We know that early detection and hastened referral to care, in collaboration with pulmonology, is critical for the best patient outcomes,” said Sonye K. Danoff, MD, Ph.D., 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.”