Abstract Ref Number = APCP1217
ONE-YEAR RESULTS OF BRONCHIOLITIS OBLITERANS FOLLOWING STEVENS-JOHNSON SYNDROME IN A 10-YEARS-OLD MALE PATIENT
Siska Permanasari Sinardja,Ayu Setyorini Mestika Mayangsari,Siadi Purniti,Ida Bagus Subanada
Residency Program Department of Child Health of Udayana University Medical School Sanglah Hospital Department of Child Health of Udayana University Medical School Udayana University Hospital Department Department of Child Health of Udayana University Medical School Sanglah Hospital
Background : Bronchiolitis Obliterans (BO) is obstructive small airway disease characterized by fibrosis of terminal and distal bronchioles. The incidence in children is not defined. It is associated with clinical conditions like viral infections, drugs, collagen-vascular diseases, organ transplantation, and rarely, Stevens-Johnson Syndrome (SJS).
Case Presentation Summary : A 10-years-old boy was admitted to the hospital with severe respiratory distress that persists for one month prior to admission. The symptom began after he was admitted with SJS one month before, with severe skin rash and swelling on both eyes after oral amoxicillin. He was previously healthy with no history pulmonary disease before. Physical examination revealed generalized wheezing and desquamated skin. Laboratory results revealed slight leukocytosis, Staphylococcus epidermidis on blood culture, Streptococcus viridans on sputum culture, and an elevated serum levels of IgE (1929 iU/ml). Chest radiographs showed attenuation of vascular and reticular marking, and chest CT imaging showed bronchial wall thickening and patch areas of hypoattenuation. He was diagnosed as BO, hence high dose pulse intravenous methylprednisolone every month, azithromycin 10 mg/kg/day for 3 days every week, and fluticasone furoate inhalation twice daily were administered for 6 months. After that, the patient continued azithromycin twice a week for another 6 months. Clinical improvement was seen after 3 months of therapy marked by improved respiratory distress, lesser until no need of rescue doses of short-acting ?-agonist, and decreased dosage of controller inhalation. Spirometry value showed improvement from the FVC 57%predicted, FEV1 39%predicted, and FEV1/FVC 68.4% for the first time that become 92.1%predicted, 89.3%predicted, and 94.2%predicted respectively after 6 months therapy.
Learning Points/Discussion : Bronchiolitis obliterans following SJS is an extremely rare case. The prognosis is poor with a variable clinical course. In our case, the patient responded very well with no respiratory symptoms and no further medication after one year.
Keywords: Bronchiolitis obliterans Stevens-Johnson syndrome Obstructive lung disease