Follicular Bronchiolitis and Lymphocytic Interstitial Pneumonia in a Patient With SLE and Sjögren Syndrome

  • Jaumally B
  • Sagar A
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INTRODUCTION: Follicular Bronchiolitis (FB) and Lymphocytic Interstitial Pneumonia (LIP) are uncommon benign lymphoproliferative lung disorders that have been identified in patients with autoimmune disorders. Treatment options and prognostic outcomes have not been well defined. CASE PRESENTATION: A 39 year old nurse with a history of Sjogren's syndrome (SS) and SLE diagnosed in 1997 presented to her primary care provider with a severe cough, progressive shortness of breath, sore throat and neck swelling associated with voice changes. A CT of her neck with contrast showed soft tissue swelling and reactive lymphadenopathy. Limited views of the lungs showed apical and basilar parenchymal cystic lesions. A CT chest was performed which showed innumerable bilateral cystic lesions. Pulmonary function testing was normal. HIV testing was negative and serum alpha-1-antitrypsin level was normal. She underwent a screening bronchoscopy and right-sided VATS with mass excision of the right upper lobe and wedge biopsies of the right middle and lower lobes. The specimen was sent for pathology evaluation which demonstrated FB and LIP. The patient was started on Azathioprine. DISCUSSION: FB and LIP may rarely be idiopathic but mostly occur in patients with autoimmune disorders, immunodeficiency syndromes or infections1. Clinical symptoms have been generally non specific with cough and dyspnea being the most common chief complaints. Diffuse ground-glass opacifications and centrilobular nodules are almost always visualized on HRCT. Lymphadenopathy, multiple thin walled cysts, septal thickening and subpleural nodules are also commonly observed2. Small airway involvement is commonly seen in SS of which FB is the most prevalent pattern2. Moreover, SS has been associated with up to 50% of all cases of LIP2. FB is associated with lymphocytic infiltrates along bronchial spaces with reactive germinal centers while LIP is characterized by lymphocytosis within the alveolar septae. Both have demonstrated favorable albeit unpredictable outcomes with corticosteroid use, azathioprine, rituximab, mycophenolate mofetil and macrolides3. A follow up after 6 months is advised to evaluate symptom improvement and need for further imaging or testing1. CONCLUSIONS: Lymphoproliferative airway disorders remain a diagnostic and prognostic challenge. FB and LIP should be included in the differential diagnosis of the dyspneic patient with co-existing autoimmune disorders.




Jaumally, B. A., & Sagar, A. E. (2016). Follicular Bronchiolitis and Lymphocytic Interstitial Pneumonia in a Patient With SLE and Sjögren Syndrome. Chest, 150(4), 807A.

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