COEXISTENCE OF LYMPHOCYTIC INTERSTITIAL PNEUMONITIS, AMYLOIDOSIS, AND MALT LYMPHOMA ASSOCIATED WITH SJÖGREN’S SYNDROME

Abstract

SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Various pulmonary abnormalities have been linked with Sjögren’s syndrome. Here we describe a case of diffuse cystic lung disease in a patient with long-standing Sjögren’s syndrome. Pathology revealed three different yet interrelated processes. CASE PRESENTATION: A 60-year-old female never-smoker with long-standing Sjögren’s syndrome underwent computed tomography of the chest to investigate cervical lymphadenopathy noted at physical examination. This identified multiple thin-walled pulmonary cysts in both lungs, together with irregular solid nodules (Figure 1). Her pulmonary function test was normal. Immunologic tests were remarkable for positive ANA, ds-DNA, SS-A, and SS-B antibodies. A thoracoscopic lung biopsy revealed extra-nodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) associated with IgA kappa immunoglobulin deposition, arising in a background of lymphocytic interstitial pneumonia (LIP) (Figure 2-A, 2-B). The patient was referred to Hematology and has remained on annual surveillance with no treatment. Three years later, the patient underwent left apical wedge resection to clarify the nature of an enlarging pulmonary nodule (Figure 1-A). Histopathological examination revealed AL (kappa) amyloid deposition. The patient remained asymptomatic until the last follow up, despite no treatment. DISCUSSION: The case illustrates the coexistence of three different but related pathological processes: LIP, amyloidosis and MALT lymphoma. Autoimmunity is associated with chronic immune system stimulation and Sjögren syndrome is recognized as a systemic autoimmune disorder with an increased risk of lymphoma, usually of B cell origin. Meanwhile, abnormal immunoglobulin can be deposited locally in the tissues and cause amyloid. The prognosis for patients with amyloidosis and LIP varies, the condition resolving in some patients, whereas it progresses to pulmonary fibrosis and respiratory failure in others. A variety of immunosuppressants, including but not limited to corticosteroids, cyclophosphamide, rituximab, methotrexate, and azathioprine have been used. Primary pulmonary MALT lymphoma is a rare entity with no standardized therapy. For localized disease, some reported cases had spontaneous regression. For widespread tumors, systemic chemotherapy has been used . CONCLUSIONS: Cystic lung disease is a rare but notable comorbidity in patients with Sjögren syndrome. It can be associated with different coinciding pathological processes. Reference #1: Bertoni F, Zucca E. Delving deeper into MALT lymphoma biology. J Clin Invest. 2006 Jan; 116(1): 22–6. Reference #2: Gupta N, Vassallo R, Wikenheiser-Brokamp KA, et al. Diffuse cystic lung disease. Part II. Am J Respir Crit Care Med. 2015; 192:17–29 Reference #3: Martínez-Balzano, Carlos D., Sunkaru Touray, and Scott Kopec. “Cystic lung disease among patients with Sjögren syndrome: frequency, natural history, and associated risk factors.” Chest 150.3 (2016): 631-639. DISCLOSURES: No relevant relationships by Matthew Cecchini, source=Web Response No relevant relationships by Heyi Li, source=Web Response Recipient of grant funding relationship with Bristol Myers Squibb Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support Recipient of grant funding relationship with Sun Pharmaceuticals Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support Recipient of grant funding relationship with Pfizer Please note: >$100000 Added 03/23/2020 by Robert Vassallo, source=Web Response, value=Grant/Research Support