A RARE CASE OF INTERSTITIAL LUNG DISEASE IN SYSTEMIC LUPUS ERYTHEMATOSUS

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: Systemic lupus erythematosus (SLE) interstitial lung disease (ILD) is a spectrum of disease that is further characterized by histopathology. Among the literature, usual interstitial pneumonia (UIP) appears to be the rarest form. We present a case of a young female with this rare phenomenon. CASE PRESENTATION: A 38 year old female with a history of SLE was evaluated by pulmonology outpatient for progressively worsening dyspnea on exertion. Rest relieved the dyspnea and she could walk approximately 30 feet before having to stop. CT pulmonary angiography was negative for pulmonary emboli but showed peripheral interstitial markings bilaterally, greatest in the lower lobes. Pulmonary function test showed mild to moderate restrictive lung disease. Bronchoscopy, transbronchial biopsy, and serologic workup was negative except for elevated ANA. Since her diagnosis of SLE, she only had known joint involvement. ANA titer was 1:320. A repeat CT chest several months later showed subpleural interstitial markings within both upper lobe zones and more pronounced at the bases. CT surgery evaluated her and performed a video assisted thoracoscopy with biopsy of lower lung. Biopsy revealed a honeycomb pattern suggestive of UIP and idiopathic pulmonary fibrosis (IPF). A second opinion by a pathologist at a large academic center confirmed the initial diagnosis. She required supplemental oxygen for hypoxia on exertion and started mycophenolate with eventual addition of pirfenidone. Her symptoms improved and she is being evaluated for lung transplantation. DISCUSSION: Although SLE-UIP is rare, clinicians should remain diligent about thoroughly reviewing past medical history and having a broad differential for patients presenting with dyspnea. SLE-UIP has multiple forms including acute, subacute, and chronic although most cases have non-specific findings on high resolution CT or histopathology to subclassify the disease [1]. The prognosis is better than IPF/UIP associated with other connective tissue diseases [1,2,3] with a 5 year mortality rate of approximately 85% [1]. Literature on SLE-UIP is predominantly case reports and the ideal treatment has not been identified but most patients receive glucocorticosteroids with addition of immunsuppressive therapy if needed. In our patient, perhaps she will receive a lung transplant. CONCLUSIONS: Usual interstitial pneumonia in the setting of systemic lupus erythematosus is uncommon with few reported cases. To optimize treatment, further studies are warranted. Reference #1: Enomoto N, Egashira R, Tabata K, et al. Analysis of systemic lupus erythematosus-related interstitial pneumonia: a retrospective multicentre study. Sci Rep. 2019;9(1):7355. Reference #2: Nakamura Y, Chida K, Suda T, et al. Nonspecific interstitial pneumonia in collagen vascular diseases: comparison of the clinical characteristics and prognostic significance with usual interstitial pneumonia. Sarcoidosis Vasc Diffuse Lung Dis. 2003;20(3):235-41. Reference #3: Enomoto N, Suda T, Kato M, et al. Quantitative analysis of fibroblastic foci in usual interstitial pneumonia. Chest. 2006;130(1):22-9. DISCLOSURES: No relevant relationships by Bharat Avirneni, source=Web Response No relevant relationships by Christopher Rosse, source=Web Response No relevant relationships by Christopher Schmitt, source=Web Response