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A NOVEL CLINICAL PRESENTATION OF BRONCHIOLITIS OBLITERANS

Baylor College of Medicine, Houston, TX

PURPOSE: To describe the significance of a unique clinical presentation of chronic rejection which resulted in rapidly declining lung function in lung transplant recipients.

METHODS: We prospectively reviewed patients who underwent lung transplantation in our institute from 2001 to 2005 and identified patients with a upper lobe predominant ground glass infiltrate and rapid decline in lung function with Bronchiolitis Obliterans.We also analyzed complications and outcomes of this unique clinical presentation of chronic rejection.

RESULTS: We reviewed 59 patients from 2001 to 2005. Demographics of this control group consisted 37(63%) males and 22(37%) females,IPF:26(44%),COPD: 18(30%),CF: 9(15%),PPH:5(9%) and sarcoidosis:1(2%). Among them 42(71%) had double and 17(29%) had single lung transplantation. Further analysis revealed 7 patients who developed organizing pneumonia especially in the upper lobes with rapidly declining lung function. All of them had double lung transplantation with a male predominance of 71% to 28%. The median age at diagnosis was 35 years, which is significantly younger age group compared to the control group. 72% (5/7) had a primary diagnosis of cystic fibrosis and 28%(2/7)had pulmonary fibrosis. Outcome analysis showed 4 among 7 died despite aggressive anti rejection treatment, 2 patients who underwent a re transplantation had steadily improving graft function and 1 patient lost to follow up. Of these patients most of them had eosinophilia in broncho alveolar lavage with CT evidence of a unique pattern of upper lobe pulmonary fibrosis with a relative sparing of the basal segments (Fig:1),autopsy on 4 patients revealed bronchiolitis obliterans and pulmonary function tests demonstrated a rapid decline in FEV1 among all the patients.

CONCLUSION: The novel presentation of chronic rejection with upper lobe fibrosis and rapid decline in graft function leading towards a poor outcome was noted in a younger age group of male Cystic Fibrosis patients who underwent double lung transplantation.

CLINICAL IMPLICATIONS: In view of rapidly progressive decline in lung function in this specific group of patients despite aggressive medical treatment, timely relisting and re transplantation should be considered.

DISCLOSURE: Sivagini Ganesh, No Financial Disclosure Information; No Product/Research Disclosure Information