Bronchiolitis obliterans na stevens-johnsonsyndroom
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Casuïstiek
11-04-2012
Eline A.M. Zijtregtop, Mariëlle W. Pijnenburg, Bart L. Rottier en Liesbeth Duijts

Bronchiolitis obliterans after Stevens-Johnson syndrome

Background

Bronchiolitis obliterans is a non-reversible lung disease in which the inflammatory process ultimately leads to obstruction of the bronchioles. This condition often occurs after a lung or bone marrow transplantation, and sometimes respiratory tract infection. Clinical presentation is that of persistent and increasing airway obstruction. The gold standard for diagnosis is open lung biopsy. Treatment is symptomatic and aimed at preventing further lung damage.

Case description

An 8-year-old girl was treated for a suspected pneumonia. Two weeks later she developed Stevens-Johnson syndrome followed by severe dyspnoea. A CT scan of the chest revealed findings consistent with bronchiolitis obliterans. Methylprednisone pulse therapy was ineffective. Due to respiratory insufficiency she underwent a lung transplantation, which to date has been successful.

Conclusion

Bronchiolitis obliterans after Stevens-Johnson syndrome should be considered in patients with recurrent and progressive respiratory symptoms with typical findings on a CT scan. Lung transplantation is often the only therapeutic option.

Conflict of interest: none declared. Financial support: none declared.