SESSION TYPE: ILD Case Report Posters
PRESENTED ON: Tuesday, October 23, 2012 at 01:30 PM - 02:30 PM
INTRODUCTION: Interstitial lung diseases associated with collagen vascular diseases are diverse. Among these, there is a low prevalence of bronchiolitis. We present a case with sudden onset of symptoms and significant advanced disease at the time of diagnosis.
CASE PRESENTATION: This is the case of a 49 y/o female with Systemic Lupus Erythematosus (SLE) who presented complaining of exertional chest pain, shortness of breath, dry cough, and orthopnea for two weeks. The physical exam is unremarkable except for oxygen desaturation with exertion and obesity. Cardiopulmonary exam is normal. A Ct angiogram demonstrated no pulmonary embolus but was significant for diffuse thin walled cystic lung disease with interstitial fibrosis and areas of ground glass alveolitis. ANA positive C3 105 C4 13 TSH 2.23 IgG 3137mg/dl, IgA 621mg/dL, IgM 291mg/dL. Pulmonary function test showed restriction (FVC 1.64 64% FEV1 1.41 59% ratio 91) and decreased DLCO 55. Patient underwent VATS with wedge resection of Right Middle Lobe which showed follicular bronchiolitis with vague granulomatous features and multiple meningothelial-like nodules. Patient was started on prednisone, pantoprazole and albuterol. She had improvement of dyspnea and ground glass alveolitis. Fibrosis and innumerable cysts persisted.
DISCUSSION: Follicular bronchiolitis is defined by the presence of hyper plastic lymphoid follicles with reactive germinal centers distributed along bronchovascular bundles. Follicular bronchiolitis results from antigenic stimulation of the bronchial associated lymphoid tissue and polyclonal lymphoid hyperplasia. Follicular bronchiolitis has been associated with numerous diseases including collagen vascular disease (most commonly Sjogren’s and Rheumatoid Arthritis), immunodeficiency states, and hypogammaglobulinemias. The typical presenting symptom is progressive breathlessness and cough although some patients may have fever. Variable pulmonary function abnormalities have been reported, including obstructive, restrictive, and mixed patterns. Patients with follicular bronchiolitis typically have a favorable prognosis. Treatment is directed at the underlying disease or may consist of steroids. Macrolides may reduce inflammation and cytokine production, improving symptoms and lung function. This data has been extrapolated from patients with diffuse panbronchiolitis and requires further investigation.
CONCLUSIONS: Thought follicular bronchiolitis is less commonly associated with SLE it should be considered as a cause of dyspnea. Early detection of follicular bronchiolitis is necessary for initiation of treatment prior to progression to fibrosis. The role of macrolides and immunomodulators need further investigation.
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DISCLOSURE: The following authors have nothing to disclose: Lymaris Garcia, Keren Bakal, Ezra Dweck
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