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Pigeon‐breeder's disease in a child with selective IgA deficiency

A 5.5-year-old girl presented with an 8-month history of progressive cough, dyspnea, fever, and lack of appetite. She was referred to our hospital from an outside hospital, with the probable diagnosis of cystic fibrosis. Initial physical examination revealed diffuse crackles in the lungs bilaterally and clubbing. The rest of the physical examination was unremarkable. In the laboratory work-up, a chest X-ray showed bilateral peribronchial thickening (Fig. 1). Sweat chloride test, α 1 -antitrypsin level were normal, and arterial oxygen saturation was 95%. Pulmonary function tests were not performed because the patient did not co-operate. Serum levels of IgG, IgG subgroups, IgM and CH 50 , and nitrobluetetrazolium test were normal, while serum IgA level was very low (< 6 mg/dL). A high-resolution computerized tomography (HRCT) scan of the chest showed bilateral diffuse groundglass appearance, disseminated centrilobular densities and air entrapments in the lungs (Fig. 2). Echocardiography revealed findings consistent with pulmonary hypertension. Regarding those clinical findings and radiologic abnormalities, an open lung biopsy was planned. At this time, the patient’s history was carefully checked again, and we learned that the patient’s father had been breeding pigeons for many years. However, the patient was not exposed to pigeons directly, but indirectly by way of her father’s clothes. For this reason, a diagnosis of PBD was considered. Precipitins were strongly positive for pigeon antigens in the patient’s serum. Exposure to pigeon antigens was stopped, and she was treated with prednisolone (2 mg/kg, daily, p.o) for a period of 4 weeks. One month later, excellent clinical response was achieved. Three months later HRCT findings improved significantly (Fig. 3) and echocardiographic findings of pulmonary hypertension improved. The prednisolone dose was reduced to 1 mg/kg per day.