AB 72. Follicular bronchiolitis associated with underlying common variable immunodeficiency

Background: Follicular bronchiolitis is a benign lymphoproliferative disorder of the lungs. We report the case of a patient diagnosed with follicular bronchiolitis that after investigation was attributed to common variable immunodeficiency disorder.

Patients and methods: A 35-year-old female patient was admitted to our Department suffering from fever up to 38.0 ℃ for the last 3 months. At her admission the patient was febrile with a mild non productive cough. Her blood screen showed leucopenia and anemia. The CXR was within normal limits, but the lung HRCT demonstrated regions with ground glass opacities interchangeable with nodular regions at the lower lobes bilaterally, the medium lobe and lingula in addition to bronchiolectasies. Bronchoscopy was not revealing. Bronchoalveolar lavage was of medium cellularity with a high percentage of lymphocytes up to 43%, of neutrophils 10% and of eosinophils 1%. Pulmonary function tests showed a restrictive pattern. On the grounds of an increased suspicion of lymph tissue malignancy, the patient underwent bone marrow biopsy and peripheral blood immunophenotype. The biopsy was negative for malignancy and an important decrease of B lymphocytes was found by the bone marrow aspiration. The immunological check of the patient revealed a significant low value of IgG immunoglobulin and a considerable decrease of the value of IgG and IgA. Two years before her admission to our Department, the patient was investigated for recurrent incidents of fever and radiological imaging of interstitial lung disease.

Results: Lung biopsy by the method of Video Assisted Thoracoscopy diagnosed follicular bronchiolitis. She was treated with corticosteroids for approximately 8 months without any response. The final diagnosis of common variable immunodeficiency was set and intravenous immunoglobulin replacement therapy was initiated to the patient.

Conclusions: Pulmonary manifestations of common variable immunodeficiency disorder, apart from lung infections, include interstitial lung diseases among which follicular bronchiolitis.