A precious experience of lobar transplantation in a teenager with end-stage chronic hypersensitivity pneumonia: case report.

Pulmonary fibrosis inevitably develops in patients with chronic hypersensitivity pneumonia (CHP). Lobar transplantation may be a viable option for pediatric and small adult patients with end-stage CHP and life-threatening respiratory decompensation. We describe a 16-year-old girl experiencing end-stage pigeon breeder's hypersensitivity pneumonia with right heart failure, who received left allograft lobar transplantation and had an uneventful convalescent course for 1 year after transplantation. Histopathologically the excised native lung revealed diffuse infiltration of lung parenchyma by CD3+ and CD8+ cells with an absence of CD4+ cells, whereas T-lymphocyte subsets analysis revealed no abnormalities in the blood. This finding is consistent with the contribution of a local type IV immune reaction to the pathogenesis. In addition, the observation of specific cellular distribution of bronchus-associated lymphoid tissue suggests that chronic antigenic stimulation and /or inflammation in CHP may cause bronchus-associated lymphoid tissue development, which is likely to play an important role in the mucosal immune response of this disease.