Lung Pathology |

Lymphocytic Interstitial Pneumonia (LIP) Transforming to Pulmonary Lymphoma in Primary Sjogren's Syndrome (SS) FREE TO VIEW

Rahul Sangani, MD; Ahmad Alhajhusain, MD
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West Virginia University, Morgantown, WV

Chest. 2015;148(4_MeetingAbstracts):605A. doi:10.1378/chest.2275373
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SESSION TITLE: Lung Pathology Case Report Posters

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 27, 2015 at 01:30 PM - 02:30 PM

INTRODUCTION: LIP is classically associated with SS. The transformation of LIP to pulmonary lymphoma is reported but considered as uncommon phenomenon. We present a case of LIP progressing to pulmonary lymphoma in a patient with SS.

CASE PRESENTATION: 43 year-old nonsmoker female with history of SS presented with dry cough and dyspnea on exertion for 4 months. Physical exam was unremarkable. Labs were significant for a positive serum anti-SSA/Ro and anti-SSB/La antibodies. Chest CT showed ground glass opacities, poorly defined centrilobular nodules, and cystic changes (Fig 1a). Pulmonary function test revealed isolated reduction in diffusion capacity. Transbronchial lung biopsy showed atypical lymphoplasmacytic infiltrates with septal thickening (Fig 2a), confirming the diagnosis of LIP. Recurrent symptoms prevented tapering of steroids and mycophenolate mofetil. She had elevated liver enzymes due to azathioprine. In view of LIP refractory to conventional treatment, a trial of rituximab was started. Few months after rituximab treatment, patient noted marked improvement. At three years interval, she reported recurrence of symptoms. CT chest revealed new poorly defined ground-glass nodules in the bilateral upper lobes with air bronchograms (Fig 1b). Biopsy of the right upper lobe lesion confirmed extranodal marginal zone lymphoma (Fig 2b).

DISCUSSION: LIP is a hyperplastic disorder of the mucosa-associated lymphoid tissues (MALT), lying on a spectrum from follicular bronchiolitis to lymphoma. The lymphoproliferative disorders in SS develop as a multistep process in which polyclonality is followed by monoclonality and t(14;18) chromosomal translocation. The previously reported rate of progression of LIP to malignant lymphoma was 30%. Immuno-histochemical and molecular analyses suggest that the lymphoma was inaccurately diagnosed as LIP. Our patient sustained chronic antigenic stimulation of LIP for 3 years before development of MALT lymphoma. Patient improved significantly with 6 months therapy of bortezomib and dexamethasone.

CONCLUSIONS: Our case highlights the potential of LIP in SS transforming to pulmonary lymphoma. Standard surveillance should include a combined clinical, laboratory and radiologic approach to identify higher risk SS patients.

Reference #1: Sarkar, PK. et al. Pulmonary manifestations of primary Sjogren's syndrome. Indian J Chest Dis Allied Sci. 2009;51(2):93-101

Reference #2: Park, KH. et al. A Case of Pulmonary MALT Lymphoma Arising from Lymphocytic Interstitial Pneumonitis. Tuberc Respir Dis (Seoul), 2012;73(2):115-21

DISCLOSURE: The following authors have nothing to disclose: Rahul Sangani, Ahmad Alhajhusain

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