Management of Interstitial Lung Disease

Classification and Initial Approach

The American College of Rheumatology recommends micofenolato as the first-line treatment for most interstitial lung diseases associated with systemic autoimmune diseases, while antifibrotic therapy with nintedanib or pirfenidona is indicated for idiopathic pulmonary fibrosis and progressive fibrosing interstitial lung disease 1, 2.

The American College of Rheumatology recommends micofenolato, rituximab, and azatioprina as first-line treatment options for interstitial lung disease associated with rheumatoid arthritis 1, 2, 4.
The American Thoracic Society recommends antifibrotic therapy with nintedanib or pirfenidona for idiopathic pulmonary fibrosis, which reduces the annual decline in forced vital capacity by 44-57% 7.

The American College of Rheumatology conditionally recommends nintedanib and pirfenidona as treatment options for progressive interstitial lung disease associated with rheumatoid arthritis 3.
The American Thoracic Society recommends against the use of long-term glucocorticoids in patients with idiopathic pulmonary fibrosis, as they are associated with increased mortality 7.

The American College of Rheumatology recommends methylprednisolone pulses and initial combination therapy as first-line treatment for rapidly progressive interstitial lung disease 5, 6.
The American Thoracic Society recommends considering referral for lung transplantation in patients with rapidly progressive interstitial lung disease, with a conditional recommendation for early referral over late referral after progression with optimal medical management 5, 6.

The American Thoracic Society recommends an algorithmic approach to managing cough in interstitial lung disease, including determining if the interstitial lung disease is the likely cause, investigating other causes, and considering treatment complications 7.

The American College of Rheumatology emphasizes the importance of co-management with pulmonologists for the initiation of interstitial lung disease treatment, particularly for determining the need for treatment in asymptomatic patients with stable and mild interstitial lung disease 1, 4.
The American Thoracic Society recommends against the use of methotrexate, leflunomide, tumor necrosis factor inhibitors, and abatacept for the treatment of interstitial lung disease associated with autoimmune diseases, although they may be appropriate for extrapulmonary manifestations 1, 4.