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Mucus plugs in CF patients may resemble plastic bronchitis

Symptoms of Scedosporium apiospermum colonization in patients with cystic fibrosis may resemble symptoms of plastic bronchitis, according to research.

“Cystic fibrosis patients may show acute deterioration of their lung disease due to mucus plugs with clinical symptoms resembling plastic bronchitis following airways colonization by [Scedosporium] apiospermum,” Rita Padoan, MD, from the Regional Support Centre for Cystic Fibrosis at Spedali Civili, in Brescia, Italy, and colleagues wrote in their study. “Bronchoscopy and bronchoalveolar lavage with dornase alpha may be helpful in removing bronchial casts and lead to identification of bacteria or fungi. Further data on clinical features due to Scedosporium colonization are needed in order to clarify its role in worsening lung disease in CF patients.”

Padoan and colleagues described the case report of a patient with cystic fibrosis since birth who had normal lung function with an intermittent Pseudomonas aeruginosa infection treated with ciprofloxacin and tobramycin, according to the abstract. At 11 years, the patient showed normal respiratory lung function, which declined the following month.

“In February 2011 the child was admitted for respiratory distress, characterized by dyspnea, tachypnea and tachycardia (94 b/min), persistent dry cough and slight desaturation (SaO2 91%) not responding to oral anti-Pseudomonas antibiotic and intensified bronchodilator therapy and chest physiotherapy,” Padoan and colleagues wrote. “He also reported substantially reduced tolerance to physical effort.”

After prescribing ceftazidime, tobramycin, steroid therapy with methylprednisone and supportive oxygen therapy, there was no clinical improvement after 48 hours. The researchers started intravenous voriconazole after a sputum culture showed growth of PA and fungal hyphae of S. apiospermum.

“Despite anti-Pseudomonas, antifungal and steroid therapies, the respiratory distress worsened with an abrupt fall in [forced expiratory volume in 1 second] FEV₁ (1.33L, 74%),” the researchers wrote. “Patient’s general condition deteriorated progressively with increased need for O2 supplementation and the occurrence of severe side effects of therapy with voriconazole (dizziness, headache) that forced suspension of the treatment.”

During a bronchoscopy, the researchers discovered thick bronchial casts obstructing the bronchial lumen and aspirated them with repeated dornase alpha.

“After endoscopic procedures, inhalation therapy with dornase alpha and 7% hypertonic saline solution was carried on,” the researchers wrote. “Clinical condition of the child gradually improved until complete remission of symptoms.” – by Jeff Craven

Disclosure: The researchers report no relevant financial disclosures.