Diffuse Alveolar Hemorrhage Following Pirfenidone Initiation.

INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is bleeding into the alveolar space of the lungs. Pirfenidone is an antifibrotic agent that is approved for the treatment of idiopathic pulmonary fibrosis (IPF). The most commonly reported side effects include gastrointestinal and skin-related events. We present 3 cases of hemoptysis and DAH among patients on pirfenidone therapy for IPF. CASE SUMMARIES: An 88-year-old female, a 75-year-old male, and a 73-year-old male all with IPF on pirfenidone presented with hemoptysis and chest computed tomography (CT) findings of usual interstitial pneumonia (UIP) with superimposed opacities. In 2 patients, DAH was confirmed with bronchoscopy. Corticosteroids were initiated and pirfenidone discontinued in all patients, and 2 patients improved while the third continued to deteriorate. Nintedanib was initiated in the remaining 2 patients at follow-up visit with no further issues. DISCUSSION: IPF is a chronic, progressive, fibrotic interstitial lung disease (ILD) which appears to be increasing in the United States and has a relatively short survival. Nintedanib and pirfenidone were the first Food and Drug Administration (FDA)-approved agents for the treatment of IPF in October 2014. We present 3 cases of DAH in patients with IPF receiving pirfenidone. Symptoms occurred within 2 months of pirfenidone initiation and resolved with discontinuation of pirfenidone and initiation of systemic corticosteroids in 2 patients; however, one case was complicated by concomitant discontinuation of aspirin. The mechanism by which DAH occurred in our patients remains unclear. CONCLUSION: We report the first cases of possible pirfenidone-induced DAH. Further studies are warranted to explore this reaction, but prescribers should be cognizant of this potential issue when choosing to prescribe pirfenidone.

Management of Non-Cystic Fibrosis Bronchiectasis.

OBJECTIVE: The purpose of this report is to describe the case of a 43-year-old male with asthma who was hospitalized for an exacerbation of non-cystic fibrosis bronchiectasis (NCFB), a chronic lung disease that is characterized by dilation of the airways, persistent cough, chronic sputum production, and recurrent respiratory infections. He was treated with oral and inhaled antibiotics, inhaled bronchodilators, and aggressive airway-clearance techniques including nebulized 7% sodium chloride, flutter valve, and high-frequency chest wall oscillation. SETTINGS: Community pharmacy, nursing facility pharmacy, consultant pharmacy practice. PRACTICE CONSIDERATIONS: As the number of patients diagnosed with NCFB continues to increase, it is crucial to recognize that specific guidance for management of NCFB is warranted, as treatment responses differ from cystic fibrosis bronchiectasis or chronic obstructive pulmonary disease. CONCLUSION: It is important for pharmacists to understand the pharmacologic and nonpharmacologic treatments for NCFB to better assist physicians and patients and improve therapeutic outcomes.