Cystic fibrosis pulmonary exacerbation is an episode of worsening respiratory symptoms in patients with chronic CF lung disease, driven by increased proliferation of established airway pathogens (especially Pseudomonas aeruginosa, Staphylococcus aureus, Burkholderia cepacia complex, nontuberculous mycobacteria, Aspergillus species) and the resulting inflammatory response. Diagnosis is clinical, based on increased cough, change in sputum quantity, color, or consistency, decreased forced expiratory volume in 1 second (FEV1) by at least 10%, fever, weight loss, increased respiratory rate, and chest examination changes. Each exacerbation accelerates lung function decline, and recovery to baseline is incomplete in 25% of episodes.
Risk factors for exacerbation include chronic Pseudomonas colonization, FEV1 less than 40% predicted, frequent prior exacerbations, CF-related diabetes, malnutrition, allergic bronchopulmonary aspergillosis, sinus disease, and gastroesophageal reflux. Sputum culture and susceptibility testing guide antimicrobial selection; established airway flora pattern from recent samples is often relied upon when culture is pending. Imaging with chest radiograph or CT may demonstrate new infiltrates, mucus plugging, or worsening bronchiectasis.
Management combines intensive intravenous antibiotic therapy with two antipseudomonal agents (typically a beta-lactam such as ceftazidime, cefepime, or piperacillin-tazobactam plus an aminoglycoside such as tobramycin) for 14-21 days, intensified airway clearance with high-frequency chest wall oscillation, hypertonic saline, and dornase alfa, optimized inhaled antibiotics (tobramycin, aztreonam) between exacerbations, nutritional support with high-calorie enteral feeds and pancreatic enzyme adjustment, continued CFTR modulator therapy (elexacaftor-tezacaftor-ivacaftor for eligible patients), and treatment of comorbidities. FEV1 response is monitored regularly to assess recovery. Failure to return to baseline triggers re-evaluation for resistant organisms, ABPA, NTM, or non-adherence. Multidisciplinary care from CF centers, including respiratory therapists, dietitians, social workers, and psychologists, optimizes outcomes.