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Nodular lymphocyte- predominant lymphoma with lymphocytic interstitial pneumonitis as primary presentation of sjogren syndrome

Jacqueline Chung,Doug Moore,Zachary Depew

CHEST(2019)

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Abstract
SESSION TITLE: Wednesday Fellows Case Report Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: 10/23/2019 09:45 AM - 10:45 AM INTRODUCTION: Primary Sjogren’s syndrome (pSS) is a complex autoimmune disease characterized by chronic lymphocytic infiltration and destruction of glandular tissue. Usual primary sites of involvement are salivary and tear glands but can involve extra-glandular systems. Most common pulmonary presentations are interstitial lung diseases (ILD) and tracheobronchial diseases. Within ILDs associated with pSS, nonspecific interstitial pneumonia is most common, followed by usual interstitial pneumonia and lymphocytic interstitial pneumonitis (LIP). Patients with pSS are also at increased risk of developing lymphoproliferative disorders and malignancies, especially Non-Hodgkin's lymphoma. This case highlights the variable presentation of pSS involving two rare associated diseases. CASE PRESENTATION: A 53 y/o female with no significant past medical history initially presented to the ER on February 2016 for chest pain and was treated with antibiotics for suspected pneumonia. Follow up imaging was concerning for persistent axillary lymphadenopathy with focal ground glass infiltrate (GGI) in the right middle lobe (RML). IR biopsy of left axillary lymph node was significant for Hodgkin’s lymphoma. After completing radiation treatment, a surveillance CT chest in June 2016, showed an enlarged left axillary lymph node and increasing RML infiltrate. PET scan completed 6 months later demonstrated resolution of lymphadenopathy but the non-hypermetabolic GGI remained. One year follow up CT chest with contrast revealed continued growth of the GGI along with scattered cystic lesions. Patient underwent a CT-guided trans-thoracic needle aspiration (TTNA) of RML GGI with resulting pathology significant for lymphoid infiltrates without malignancy. Additional connective tissue and autoimmune serologies were processed and found positive for ANA, SS-A (>8.0) and SS-B (> 8.0) and negative for RF, CCP, and dsDNA. In view of her serologies, changes on CT scan and lymphoid cells on TTNA biopsy, the patient was diagnosed with LIP secondary to pSS. DISCUSSION: ILDs are the most common pulmonary manifestation of pSS accounting for roughly 3-10% of diagnosis, while LIP accounts for only 25% of those cases (1). Pathophysiology of LIP suggests an infiltrative process of alveolar tissue by lymphocyte and other elements alluding to a commonality with pSS. On HRCT, LIP can include perivascular cysts associated with GGI, poorly defined centrilobular and subpleural nodules. Interestingly, lymphoma can have similar radiographic appearance (2). Conversely, LIP has a low frequency of evolving into lymphoma but consolidative nodular opacities should be evaluated (3). CONCLUSIONS: Our case demonstrates the challenges of diagnosing pSS especially with concomitant presentation of non-Hodgkin's lymphoma which occurs in 1% of pSS cases and LIP. It also supports the importance of evaluating of pulmonary involvement seen in pSS independently of lymphoma. Reference #1: Roca, F. Dominique, S. Schmidt, J. Smail, A. Duhaut, P, Levesque, H. Marie, I. (2011). Interstitial Lung disease in Primary Sjogren's Syndrome. Korean Journal of Internal Medicine. 26(1) pp 108-111. Reference #2: Panchabhai, T. Farver, C. Highland, KB. Update on Lymphoid Interstitial Pneumonitis. (2016). Clinical Chest Medicine. 37(3) pp 463-474. Reference #3: Fishback,N. Koss, M. (1996) Update on lymphoid interstitial pneumonitis. Current Opinion in Pulmonary Medicine. 2(5) pp 429-433 DISCLOSURES: No relevant relationships by Jacqueline Chung, source=Web Response no disclosure on file for Zachary Depew; Speaker/Speaker's Bureau relationship with Boehringer Ingelheim Pharmaceuticals, Inc. Please note: $5001 - $20000 Added 03/12/2019 by Doug Moore, source=Web Response, value=Honoraria Speaker/Speaker's Bureau relationship with Genentech Please note: $1001 - $5000 Added 03/12/2019 by Doug Moore, source=Web Response, value=Honoraria
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Pulmonary Involvement
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