Pediatrics |

Successful Therapy of Rapidly Progressing Bronchiolitis Obliterans in an Adolescent FREE TO VIEW

Megan Tzeng, MD; Michael Pins, MD; Michael Tsifansky, MD
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Advocate Children's Hospital - Park Ridge, Park Ridge, IL

Chest. 2014;146(4_MeetingAbstracts):691A. doi:10.1378/chest.1988330
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SESSION TITLE: Pediatric Student/Resident Case Report Posters

SESSION TYPE: Medical Student/Resident Case Report

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: Bronchiolitis obliterans (BO) is uncommon in children, occurring most frequently after lung transplantation or infection. Its therapy is poorly defined and prognosis often guarded. We describe an adolescent with rapidly progressive BO in the context of hypersensitivity pneumonitis (HP) who responded to a combination of high-dose steroids, hydroxychloroquine, azithromycin, fluticasone-salmetrol, and tiotropium.

CASE PRESENTATION: A previously healthy 15-year-old boy presented in respiratory distress with bilateral spontaneous pneumothoraces and pneumomediastinum. He had had two days of progressive dyspnea, chest pain, low-grade fever, and malaise following three months of fatigue and decreased appetite. Over the preceding few years, he had raked leaves for extra income and smoked marijuana. A right tube thoracostomy initially relieved his symptoms, but within hours his dyspnea and hypoxemia worsened. Computerized tomography (CT) of his chest demonstrated diffuse bilateral lung consolidations and ground glass opacities (GGOs), without apical blebs [Figure, a]. His initial workup revealed elevated C-reactive protein, erythrocyte sedimentation rate, immunoglobulin E, and mildly positive precipitins to the fungus Phoma betae; the remaining HP panel was unremarkable, as were his sputum studies and vasculitis workup [Table]. Echocardiography documented mild pulmonary hypertension (PHTN). He worsened despite sildenafil, low-dose steroids, and antibiotics. Water-sealing his thoracostomy tube repeatedly failed, and repeat chest CT showed progression of GGOs [Figure, b]. He ultimately underwent bilateral thoracoscopic lung biopsies demonstrating extensive BO against the background of chronic HP. Indeed, the HP panel repeated at a different laboratory revealed high titers to multiple soil fungi [Table]. Treatment with high-dose steroids, hydroxychloroquine, azithromycin, fluticasone-salmetrol, and tiotropium resulted in dramatic clinical and radiographic improvement, including resolution of PHTN [Figure, c].

DISCUSSION: BO is sparsely described in the pediatric population outside of the post-transplant or post-infectious contexts, and no standard therapy exists for pediatric BO. Two aspects of this report are unique: the progression of HP to BO in a pediatric patient and his response to the regimen of high-dose systemic steroids, hydroxychloroquine, azithromycin, fluticasone-salmeterol, and tiotropium. Lastly, the discordant results from the two hypersensitivity panels underscore the importance of repeating laboratory values that do not fit an otherwise suggestive clinical picture.

CONCLUSIONS: Components of this regimen have been used in pediatric BO but with inconsistent outcomes. This is the first report of using this combination in its entirety in an adolescent patient with HP-associated BO, and its short-term effects appear promising.

Reference #1: J Korean Med Sci. 2013;28(6):915-23

Reference #2: Chest. 2013 Sep;144(3):974-80.

Reference #3: Ann Am Thorac Soc. 2014 Jan;11(1):87-91.

DISCLOSURE: The following authors have nothing to disclose: Megan Tzeng, Michael Pins, Michael Tsifansky

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