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Pulmonary Medicine›Condition·Updated Jul 11, 2026·v1

Cystic Fibrosis

Cystic fibrosis is an autosomal recessive disorder caused by CFTR mutations, leading to defective chloride/bicarbonate transport, chronic lung disease with bronchiectasis, pancreatic insufficiency, and multisystem complications. Diagnosis relies on elevated sweat chloride (≥60 mmol/L) and genetic confirmation. Management is transformed by CFTR modulators (especially elexacaftor/tezacaftor/ivacaftor), supplemented by airway clearance, hypertonic saline, dornase alfa, azithromycin, and short-course IV antibiotics for exacerbations. Newborn screening and multidisciplinary care have extended median survival beyond 36 years. Key surveillance includes annual OGTT, bone density, and colorectal cancer screening from age 40.

High Evidence754 references·10,507 words·43 min read·v1
Cystic FibrosisCFTRPulmonary MedicineGenetic DiseaseBronchiectasisPancreatic InsufficiencyCFTR ModulatorsPseudomonas aeruginosaPediatricsNewborn Screening

Quick Reference

RxDrug of choiceElexacaftor/tezacaftor/ivacaftor (ETI) for eligible patients ≥6 years with at least one F508del allele.
AltAlternativesIvacaftor for gating mutations; tezacaftor/ivacaftor or lumacaftor/ivacaftor for F508del homozygotes not on ETI.
⊘AvoidAvoid non-dihydropyridine CCBs (diltiazem, verapamil); avoid routine systemic corticosteroids during exacerbations; avoid concomitant azithromycin + IV tobramycin during exacerbation treatment.
DxTest of choiceSweat chloride test (≥60 mmol/L diagnostic); HRCT chest for bronchiectasis detection.
ScKey scoreFEV1 percent predicted (severity staging: mild >70%, moderate 40-70%, severe <40%); Lung clearance index (LCI) for early small-airway disease.
→When to referLung transplant evaluation when FEV1 <30% predicted, especially with hypercapnia (PaCO2 ≥50 mmHg), frequent exacerbations (≥2/year), or supplemental oxygen/NIV need.
CF is a multisystem genetic disease dramatically improved by early diagnosis and CFTR modulator therapy. Core management includes airway clearance, infection control, and aggressive treatment of exacerbations with short-course IV antibiotics.
Cystic fibrosis (CF) is the most common life-limiting autosomal recessive disease in white populations, caused by biallelic CFTR mutations that disrupt epithelial ion transport. The clinical hallmarks, chronic progressive lung disease with bronchiectasis, pancreatic exocrine insufficiency, and elevated sweat chloride, begin in infancy, but early diagnosis through newborn screening and the revolutionary introduction of CFTR modulator therapy have transformed survival from early childhood to a median of >36 years. Management now centers on genotype-directed modulator therapy (elexacaftor/tezacaftor/ivacaftor as first-line), combined with airway clearance, aggressive treatment of pulmonary exacerbations, and surveillance for multisystem complications including CF-related diabetes and bone disease.

Overview and Recommendations

Background

  • •Cystic fibrosis (CF) is an autosomal recessive disorder caused by biallelic pathogenic variants of the gene, leading to defective chloride and bicarbonate transport across epithelial surfaces. This results in progressive obstructive lung disease, pancreatic exocrine insufficiency, and multisystem complications, making it the most common life-limiting autosomal recessive disease in white populations.
  • •Incidence is 1 in 2500-3500 live births in Europe and North America, with median predicted survival now exceeding 36 years due to modern therapies. Over 1900 CFTR mutations have been identified; functional classes I-VI predict disease severity and guide modulator therapy. The F508del mutation (class II) accounts for approximately 70% of CF alleles in populations of European descent.
  • •The pathophysiology begins with CFTR dysfunction abolishing anion secretion, leading to airway surface liquid (ASL) depletion, mucus hyperconcentration, and impaired mucociliary clearance. This creates a cycle of chronic infection (especially and ), neutrophilic inflammation, and progressive bronchiectasis visible on CT as early as 3 months of age.
  • • has shifted diagnosis to early infancy, altering natural history and allowing early intervention. However, in low-resource settings, delayed diagnosis still leads to advanced bronchiectasis at presentation. The distinction between classic CF (sweat Cl >60 mmol/L) and non-classic CF (borderline sweat) determines eligibility for .

Evaluation

  • •Suspect CF in any infant with (15-20% of newborns), persistent cough, poor growth, or chronic diarrhea. Also suspect in older children or adults with recurrent sinopulmonary infections, , or unexplained bronchiectasis.
  • •Perform as the diagnostic gold standard; a value ≥60 mmol/L on two separate occasions is diagnostic. For borderline values (30-59 mmol/L), proceed to CFTR genotyping to identify two confirmed CF-causing mutations.
  • •Order as the cornerstone of lung function monitoring. Measure FEV1 percent predicted; an obstructive pattern (FEV1/FVC < lower limit of normal) is typical. FEV1 declines an average of 1-3% predicted per year without modulator therapy.
  • •Obtain to detect bronchiectasis (airway-to-artery ratio >1), air trapping, and mucus plugging. Bronchiectasis is present in 29% of infants at 3 months and 62% by 3 years in newborn-screened populations. Use low-dose protocols to minimize radiation.
  • •Collect sputum for routine bacterial culture, including nontuberculous mycobacteria (NTM) and fungal studies. In non-expectorating children, is superior to cough swab for pathogen detection. Reserve for unresolved cases or suspected NTM.
  • •Assess pancreatic function by measuring fecal elastase-1; levels <200 μg/g indicate , present in ~85% of classic CF patients. Also screen for with annual oral glucose tolerance test starting at age 10.
  • •Evaluate bone health with dual-energy X-ray absorptiometry (DXA) scan every 1-2 years in adults; low bone mineral density occurs in 25-35% of adults. Check vitamin D levels and supplement as needed.
  • •Consider from multiple-breath washout as a more sensitive marker of early lung disease, especially in children under 6 years who cannot perform spirometry. LCI predicts future exacerbations and bronchiectasis.

Management

  • •Initiate as first-line for all eligible patients. For those ≥6 years with at least one F508del allele, start (ETI). Dosing: for patients <30 kg, give ELX 100 mg once daily, TEZ 50 mg once daily, IVA 75 mg every 12 hours; for ≥30 kg, give adult dose (ELX 150 mg, TEZ 75 mg, IVA 150 mg), two tablets in the morning, one tablet in the evening.
  • •For patients with gating mutations (e.g., G551D) not eligible for ETI, start 150 mg every 12 hours. Ivacaftor improves FEV1 by 10.6 percentage points and reduces exacerbations by 55%.
  • •Add adjunctive chronic therapies: 7% 4 mL inhaled twice daily to improve mucociliary clearance; 2.5 mg inhaled once daily; and 250-500 mg (or 10 mg/kg) three times weekly for patients with chronic Pseudomonas infection to reduce exacerbations.
  • •For acute (defined by worsening symptoms and ≥10% decline in FEV1), admit for IV antibiotics. Use dual antipseudomonal coverage: 3 g IV thrice daily plus 5-10 mg/kg IV once daily. For ceftazidime allergy, substitute 2 g IV thrice daily.
  • •Assess response at days 7-10. If early responder (FEV1 improvement ≥10 percentage points from nadir), complete 10 total days. If non-early responder, continue to 14 days. Do not extend beyond 14 days; STOP2 trial showed no benefit from longer courses.
  • •Continue during exacerbation: encourage twice-daily sessions using oscillatory devices or inhalation. Do not routinely add systemic corticosteroids; they do not improve FEV1 recovery and may cause hyperglycemia.
  • •For hypoxemia, titrate oxygen to target SpO2 ≥92%. For hypercapnia (PaCO2 >45 mmHg) or nocturnal desaturation, consider (NIV). NIV during sleep improves event-free survival.
  • •Refer for evaluation when FEV1 falls below 30% predicted, especially with hypercapnia, frequent exacerbations (≥2/year), or need for oxygen/NIV. ETI can often suspend the need for transplant but reassess at 3-6 months.
  • •Manage with insulin therapy; oral hypoglycemics are not recommended. Monitor renal function and avoid nephrotoxic drugs in those with CKD. Screen for colorectal cancer starting at age 40; earlier post-transplant.
  • •In children, avoid concomitant use of and intravenous during exacerbation treatment; this combination is associated with worse FEV1 recovery and shorter time to next exacerbation. Use weight-based ETI dosing carefully.

Board Review — High Yield

  • •F508del, Most common CFTR mutation in European descent; class II (defective trafficking). ETI is first-line for patients with at least one copy.
  • •Sweat chloride ≥60 mmol/L, Diagnostic threshold for classic CF; borderline (30-59) requires genetic confirmation.
  • •Lung clearance index, More sensitive than FEV1 for early airway disease; can be measured in preschoolers via multiple-breath washout.
  • •STOP2 trial, For CF exacerbations, 10 days IV antibiotics is non-inferior to 14 days in early responders; no benefit beyond 14 days.
  • •Elexacaftor/tezacaftor/ivacaftor, Improves FEV1 by 13.8 points, reduces exacerbations by 63%, lowers sweat chloride by 42 mmol/L (phase 3 trial).
  • •SIMPLIFY trial, In stable patients on ETI with FEV1 ≥70%, discontinuing hypertonic saline and dornase alfa is non-inferior to continuing.
  • •Pseudomonas aeruginosa, Dominant pathogen; chronic infection accelerates FEV1 decline. Azithromycin 3 times weekly reduces exacerbation risk by 44%.
  • •Meconium ileus, Present in 15-20% of newborns; predicts bronchiectasis by age 3 (OR 3.17).
  • •CF-related diabetes, Affects 31% of adults; annual OGTT from age 10. Associated with worse lung function and mortality.
  • •Avoid systemic corticosteroids during exacerbations, No improvement in FEV1 recovery or time to next exacerbation; may cause hyperglycemia.

Deep Dive — Evidence Details

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