Fast Five Quiz: Progressive Fibrosing Interstitial Lung Disease

Zab Mosenifar, MD, FACP, FCCP


April 12, 2022

Antifibrotic therapy with nintedanib or pirfenidone slows lung function decline and is the backbone of treatment for IPF with an expanded indication of PF-ILD for nintedanib. Nintedanib is an oral intracellular tyrosine kinase inhibitor that blocks cell signaling pathways involved in fibrosis progression. Pirfenidone is another oral antifibrotic agent that reduces fibrotic progression by inhibition of collagen synthesis and fibroblast proliferation. Tocilizumab, an inhibitor of interleukin-6 signaling, also has antifibrotic effects and was recently shown to preserve FVC in systemic sclerosis-related ILD as a secondary end point.

Gastroesophageal reflux is common in IPF and CTD-ILDs and may contribute to disease progression. Current guidelines recommend antacid therapy in IPF, though quality of evidence is low. In retrospective studies, antacids reduced exacerbations and lung function decline and improved survival in IPF. However, a large post hoc analysis from 2016 had shown no difference in mortality or FVC and more pulmonary infections with antacid use.

Pulmonary hypertension (PH) occurs frequently with ILD and is associated with worse symptoms and exercise tolerance, earlier mortality, and increased need for supplemental oxygen. Elevated right ventricular systolic pressure or right heart dysfunction on echocardiography should prompt investigation into comorbid hypoxemia or obstructive sleep apnea. Pulmonary vasodilators have historically shown mixed results in PH, and current IPF guidelines recommend against use of sildenafil or endothelin receptor agonists. Riociguat increases mortality and should be avoided.

Immunosuppression, while used for CTD and fibrotic hypersensitivity pneumonitis (fHP), is harmful in IPF and should be avoided. Along with antigen avoidance, systemic oral corticosteroids are effective in nonfibrotic HP and are recommended in fHP. More recent data, however, reveal that corticosteroids may not improve mortality or lung function in fHP. In individuals with fHP on corticosteroids, transition to azathioprine or mycophenolate mofetil can improve side effects and stabilize lung function. Leflunamide has recently been shown to modestly improve lung function and allow for corticosteroid cessation in chronic HP, but side effects were frequent and response in fibrotic disease was less robust.

Learn more about treatments for IPF.


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