Abstract
BackgroundLung disease in patients with cystic fibrosis is thought to develop as a result of airway inflammation, infection, and obstruction. Pulmonary therapies for cystic fibrosis that reduce airway inflammation include corticosteroids, rhDNase, antibiotics, and high-dose ibuprofen. Despite evidence that high-dose ibuprofen slows the progression of lung disease in patients with cystic fibrosis, many clinicians have chosen not to use this therapy because of concerns regarding potential side effects, especially gastrointestinal bleeding. However, studies have shown a low incidence of gastrointestinal ulceration and bleeding in patients with cystic fibrosis who have been treated with high-dose ibuprofen.Case presentationThe described case illustrates a life-threatening upper gastrointestinal bleed that may have resulted from high-dose ibuprofen therapy in a patient with CF who had undergone a pneumonectomy. Mediastinal shift post-pneumonectomy distorted the patient's esophageal anatomy and may have caused decreased esophageal motility, which led to prolonged contact of the ibuprofen with the esophagus. The concentrated effect of the ibuprofen, as well as its systemic effects, probably contributed to the occurrence of the bleed in this patient.ConclusionsThis report demonstrates that gastrointestinal tract anatomical abnormalities or dysmotility may be contraindications for therapy with high-dose ibuprofen in patients with cystic fibrosis.
Highlights
Lung disease in patients with cystic fibrosis is thought to develop as a result of airway inflammation, infection, and obstruction
This report demonstrates that gastrointestinal tract anatomical abnormalities or dysmotility may be contraindications for therapy with high-dose ibuprofen in patients with cystic fibrosis
Airway inflammation is thought to cause much of the lung damage in patients with cystic fibrosis (CF) [1,2]
Summary
Airway inflammation is thought to cause much of the lung damage in patients with cystic fibrosis (CF) [1,2]. A week after initiation of ibuprofen at a dose of 1,000 mg b.i.d. the patient developed severe abdominal pain, hematemesis and bright red blood per rectum He became hemodynamically compromised and an emergency endoscopy revealed bleeding esophageal ulcerations in the distal 12 cm of his esophagus. The initial randomized, double-blind, placebo control study of high-dose ibuprofen in CF did not demonstrate serious side effects during 4 years of therapy with ibuprofen sufficient to achieve peak plasma concentrations of 50–100 mcg/L [8]. In a published case report, a 12-year-old patient with CF on high-dose ibuprofen developed emesis and feeding intolerance [21] She was found to have pyloric channel stricture as a result of healing antral and pyloric ulcers, which may have been caused by ibuprofen
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