Managing comorbidities in idiopathic pulmonary fibrosis

Figures & data

Table 1 Selected diagnostic and management considerations for common comorbidities of IPF

Comorbidity	Selected screening and diagnostic considerations	Selected management considerations
Pulmonary comorbidities
Emphysema (CPFE)	• Most common in male former smokers • Characterized by disproportionately reduced DLCO with relatively preserved flow rates and lung volumes • Frequently associated with pulmonary hypertension and lung cancer	• Similar management approach to isolated COPD • Unclear role of bronchodilators in patients without flow limitation • Unclear role of inhaled corticosteroids • Typically treat acute exacerbation with systemic corticosteroids and antibiotics • Rapidly taper systemic corticosteroids after an acute exacerbation
Lung cancer	• Frequently presents with nonspecific symptoms or as an incidental finding on chest imaging • Low-dose CT scan screening for lung cancer may have less benefit in IPF (compared to mortality benefit observed in the National Lung Screening Trial)	• Increased mortality and acute exacerbation of IPF following surgical resection, chemotherapy, or radiation • Decrease risk of postoperative acute exacerbation of IPF by reducing intravenous fluid replacement, avoiding high ventilatory pressures, and minimizing hyperoxia • Consider palliative therapies
Pulmonary hypertension	• Consider echocardiography for high-risk patients • Consider right heart catheterization to confirm diagnosis • Screen for other causes of pulmonary hypertension in confirmed cases	• Multiple studies show no benefit of endothelin receptor antagonists in IPF with or without pulmonary hypertension • Possible role for sildenafil in advanced IPF with pulmonary hypertension
Venous thromboembolism	• Consider contrast CT in patients with acute respiratory worsening • Isolated subsegmental filling defects are of uncertain significance in severe fibrosis	• Similar approach to management and prophylaxis compared to non-IPF patients • No role for anticoagulation in IPF patients that lack a conventional indication
Sleep-disordered breathing	• Consider screening for OSA with overnight oximetry or polysomnography in all IPF patients	• Similar management approach compared to non-IPF patients • CPAP may improve quality of life and sleep quality in patients that do not endorse sleepiness
Extra-pulmonary comorbidities
GERD	• Asymptomatic reflux is common in IPF • Unclear role of screening for acid reflux in IPF	• Antiacid medication is recommended for most patients with IPF, including those without symptoms of reflux
Coronary artery disease	• Coronary artery calcification on CT may be useful as a screening tool	• Similar medication approach to non-IPF patients • Caution with coronary artery stenting and commitment to irreversible antiplatelet agents in candidates for lung transplantation
Depression and anxiety	• Depression is more common in patients with dyspnea and severe fibrosis • Screen all patients for symptoms of depression and anxiety	• Consider cognitive behavioral therapy, antidepressants, and antianxiety treatments in patients with significant and persistent symptoms • Possible benefit of pulmonary rehabilitation
Deconditioning	• Screen all patients for reduced physical activity levels and deconditioning	• Consider pulmonary rehabilitation in IPF patients with exertional dyspnea or functional limitation

Abbreviations: COPD, chronic obstructive pulmonary disease; CPAP, continuous positive airway pressure; CPFE, combined pulmonary fibrosis and emphysema; CT, computed tomography; DLCO, diffusing capacity of the lung for carbon monoxide; GERD, gastroesophageal reflux disease; IPF, idiopathic pulmonary fibrosis; OSA, obstructive sleep apnea.