MULTIFOCAL PULMONARY NODULAR HYPERPLASIA IN A PATIENT WITH SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)

Abstract

SESSION TITLE: Medical Student/Resident Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Pulmonary nodular lymphoid hyperplasia (PNLH) is a rare lympho-proliferative disorder characterized by benign polyclonal lymphoid proliferation. We describe a case of a 44-year-old woman with biopsy proven multifocal pulmonary nodular lymphoid hyperplasia in the setting of SLE. CASE PRESENTATION: 44-year-old woman presented to the emergency room with complaints of intermittent pleuritic chest pain and exertional dyspnea for 3 months. Review of systems was positive for joint pains and morning stiffness. Physical examination was remarkable for tenderness and swelling at proximal interphalangeal joints. Cardiac work up including electrocardiogram, troponin levels and echocardiogram was unremarkable. CT scan of chest with contrast showed numerous bilateral cavitary ground glass opacities measuring about 1-2 cm in size. Transbronchial biopsy was non-diagnostic. A subsequent video-assisted thoracoscopic surgical biopsy revealed nodular lymphoid hyperplasia. Specimen was negative for malignancy, granulomatous disease or vasculitis. Lymphoma was also ruled out by flow cytometry. AFB, fungal and bacterial stains and cultures were negative. Hepatitis panel and HIV testing was negative. Rheumatologic work up showed negative rheumatoid factor and positive ANA, anti-SSA, anti-dsDNA Ab, anti smith and anti-U1-RNP antibodies. Inflammatory markers, ESR and CRP, were elevated. She was evaluated by rheumatology and further history revealed presence of alopecia and nasal sores in addition to serositis (pleuritis) and arthritis. Her clinical picture and serologies met the diagnostic criteria for SLE. DISCUSSION: Pulmonary lymphoid nodular hyperplasia is a rare benign lymphoproliferative disease. It is relatively more common in women and often an incidental finding on imaging. Most patients are asymptomatic, however symptoms such as cough, chest pain or dyspnea can be present. Diagnosis is made by surgical biopsy. It is essential to rule out malignancy, particularly mucosal associated lymphoid tissue lymphoma and infectious etiologies such as bacterial or fungal pulmonary infections. So far PNLH has been described in patients with Sjogren’s disease, without conclusive association. Most common pulmonary manifestation in SLE is pleurisy. Interstitial lung disease (ILD) is less common in SLE and is seen in patients who have anti–U1-RNP antibodies. Imaging findings are nonspecific; however, ground-glass opacities or consolidations may be present. PNLH has not been described in association to or as a manifestation of SLE in medical literature. There is paucity of data for correlation and larger studies are required to study this potential association. CONCLUSIONS: Pulmonary nodular lymphoid hyperplasia is a rare benign lymphoproliferative disorder. After malignancy and infectious etiology is ruled out autoimmune disease should be considered in patients with PNLH. Further work is warranted to establish this relation. Reference #1: Abbondanzo SL, Rush W, Bijwaard KE, Koss MN. Nodular lymphoid hyperplasia of the lung: a clinicopathologic study of 14 cases. Am J Surg Pathol. 2000;24(4):587–597. Reference #2: Song MK, Seol YM, Park YE, et al. Pulmonary nodular lymphoid hyperplasia associated with Sjögren's syndrome. Korean J Intern Med. 2007;22(3):192–196. doi:10.3904/kjim.2007.22.3.192. Reference #3: Quadrelli SA, Alvarez C, Arce SC, et al: Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies. Lupus 2009; 18: pp. 1053-1060 DISCLOSURES: No relevant relationships by William Han, source=Web Response No relevant relationships by Immad Munawar, source=Web Response No relevant relationships by Theresa Nicol, source=Web Response