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Abstract

Introduction: Follicular bronchiolitis, is a rare pulmonary manifestation of collagen vascular diseases, and is differentiated from lymphocytic interstitial pneumonia (LIP) basing on the severity and the extent of the lung involvement. Lymphocytic interstitial pneumonia is a benign lymphoproliferative disorder characterized by lymphocytic infiltration of the alveolar septa. And there is a reported 5% risk of malignant transformation of LIP and hence exclusion of malignant lymphoma in these patients is important. Here we present a case of follicular bronchiolitis, in a patient with SLE who subsequently developed B cell lymphoma. Case Description: A 50 year old female with history of SLE, diabetes mellitus referred to pulmonary clinic for complaints of shortness of breath, productive cough for 2 months. She was treated multiple times for pneumonia without any significant relief. Physical examination was significant for tachypnea, tachycardia, and oxygen saturation of 91% on 3 liters nasal cannula, diffuse bilateral expiratory wheezing and bibasilar crackles. Pulmonary function tests revealed restrictive defect with decreased DLCO. Work up for sjogren's, HIV, granulomatous disorders and atypical infections was negative. Chest X ray revealed bilateral lower lobe opacities and CT thorax showed scattered ground glass opacities, small nodules, and bronchiectatic changes involving the lower lobes of the lungs with shotty mediastenal, right hilar and axillary lymphadenopathy. Video assisted thoracic surgical biopsy of the affected lung was pursued which revealed follicular bronchiolitis. Patient was treated with methotrexate, plaquenil and prednisone. Initially the symptoms and the radiographic changes improved though persistent, were stable for an year. And thereafter patient again presented with shortness of breath and productive cough. Repeat CT thorax revealed bilateral lower lobe opacities and small nodules, bronchiectatic and fibrotic changes with severe mediastenal, hilar and axillary lymphadenopathy. Workup has been negative for atypical infection. An excision biopsy revealed Non Hodgkin's lymphoma and was treated with R-CHOP regimen and 4 months after the treatment, repeat CT showed improved opacities and decreases in the size of lymphadenopathy. Discussion: Follicular bronchiolitis characterized by hyperplastic lymphoid follicles with reactive germinal centers distributed along the bronchovascular bundles, is the result of altered immune stimulus. It may occur as a part of primary pulmonary lymphoid hyperplasia while some occur as a part of connective tissue disorders and immunodeficiency syndromes. B cell activation in such cases predisposes to lymphoma development. Both follicular bronchiolitis and Non Hodgkin's lymphoma carry a favorable prognosis and respond well to appropriate immunosuppressive therapy.