CYSTIC LUNG DISEASE IN A YOUNG MALE WITH SYSTEMIC LUPUS ERYTHEMATOSUS

SESSION TITLE: Challenges in Cystic Fibrosis Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 1:45 pm INTRODUCTION: Pulmonary involvement in Systemic Lupus Erythematosus (SLE) is seen in 30-50% of patients (most commonly Nonspecific Interstitial Pneumonitis) but cystic lung disease is extremely rare (1). Lymphoid interstitial pneumonia (LIP) is an inflammatory lung disease that is characterized by infiltration of lymphocytes and plasma cells (2), and associated with lung cysts. Oftentimes, it is associated with HIV, lymphoma, and primary Sjogren's Syndrome (SS) (2), however there are rare reports of LIP associated with SLE (1). We present a case of a young male with incidental lung cysts who was found to have a new diagnosis of SLE. CASE PRESENTATION: A 24-year-old male with a past medical history of premature birth at 5 months and prior mild COVID-19 infection presented with 3 weeks of abdominal pain, nausea, vomiting, fever, and unintentional 15-pound weight loss. He endorsed dry mouth, frequent cavities, and a new rash involving his chest, face, and lower extremities. Physical exam was significant for malar rash and dry mucous membranes. Labs revealed pancytopenia, sedimentation rate 61 mm/hour and C-reactive protein 5.54 mg/L. Computed tomography (CT) of the chest showed several thin-walled cysts in all bilateral lung lobes (predominant in right upper lobe) and bilateral axillary lymph nodes [Figure 1]. CT abdomen and pelvis was unremarkable. Autoimmune work-up resulted in a positive antinuclear antibody >1:1280, double stranded DNA antibody elevated at 34, elevated SSA and SSB antibodies (>8.0 and 1.4 respectively), and decreased Complement 3 (59.5 mg/dl) and 4 (10.1 mg/dl) levels. Peripheral smear, right axillary lymph node and bone marrow biopsies were negative for malignancy. He was started on prednisone and Plaquenil with symptomatic improvement. There is high suspicion of LIP given the clinical and radiological findings. He will follow up in clinic to obtain PFTs and schedule a lung biopsy. DISCUSSION: Interstitial lung disease in SLE presents in middle-aged patients at a later part of their disease course, with a female preponderance (2,3). An initial presentation of SLE and secondary SS in a young male and associated cystic lung disease is rare. The suspicion for LIP in association with SLE is high in our patient given variable size and distribution of lung cysts and coexisting secondary Sjogren's syndrome, although no ground glass or nodular opacities were found on CT chest as reported in typical LIP (3). Though this patient has no pulmonary symptoms, cysts/LIP in SLE tend to progress and have a high incidence of developing lymphomas, gammaglobulinemia and amyloidosis (2,3). CONCLUSIONS: It is important to establish a histopathological diagnosis and obtain baseline PFTs to monitor pulmonary disease manifestations. In addition to controlling the primary disease with antirheumatic drugs, steroids have been found to be useful in acute pulmonary flares (2). Reference #1: Maeda R, Isowa N, Miura H, Tokuyasu H. Systemic lupus erythematosus with multiple lung cysts. Interact Cardiovasc Thorac Surg. 2009 Jun;8(6):701-2. doi: 10.1510/icvts.2008.200055. Epub 2009 Mar 12. PMID: 19282324. Reference #2: Yood RA, Steigman DM, Gill LR. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus. Lupus. 1995 Apr;4(2):161-3. doi: 10.1177/096120339500400217. PMID: 7795624. Reference #3: Filipek MS, Thompson ME, Wang PL, Gosselin MV, L Primack S. Lymphocytic interstitial pneumonitis in a patient with systemic lupus erythematosus: radiographic and high-resolution CT findings. J Thorac Imaging. 2004 Jul;19(3):200-3. doi: 10.1097/01.rti.0000099464.94973.51. PMID: 15273618. DISCLOSURES: No relevant relationships by Matthew Fain No relevant relationships by Christina Fanous No relevant relationships by Rathnavali Katragadda No relevant relationships by CHRISELYN PALMA

DOES COVID-19 INCREASE THE RISK OF DEVELOPING CYSTIC LUNG DISEASE?

SESSION TITLE: Late Breaking Chest Infections Posters SESSION TYPE: Original Investigation Posters PRESENTED ON: 10/18/2022 01:30 pm - 02:30 pm PURPOSE: (1) Assess the characteristics of COVID-19 patients who developed pulmonary cysts, bullae, blebs, and pneumatoceles. (2) Investigate outcomes of patients who developed cystic lung disease from COVID-19. METHODS: A literature search using Pubmed, Cochrane, and Embase was performed for case reports from 2020 to 2022 describing COVID-19 patients who developed lung cysts, bullae, blebs and pneumatoceles. The following data were extracted: patient demographics, presence of underlying lung disease, history of smoking, maximum oxygen requirements during acute illness, imaging findings, complications, and patient mortality. RESULTS: 65 publications (11 case series and 54 case reports) with a total sample size of 76 patients were analyzed. The mean age of patients was 52.2 ± 15.8 years. A majority of the cases were males (n=67, 88.2%). Twelve (15.8%) cases had an underlying lung disease, such as COPD or asthma, and 16 (21.1%) cases had a history of smoking tobacco. We categorized severity of illness based on the levels of oxygen requirement defined as: (1) mild - 0 to 2 liters of oxygen, (2) moderate - greater than 2 liters of oxygen to face mask/venturi mask and (3) severe - high flow nasal cannula, non-invasive ventilation, or mechanical ventilation. The majority of patients (n=40, 52.6%) had severe illness while 7 (9.2%) and 17 (22.4%) presented with mild and moderate disease, respectively. Of the 25 (32.9%) patients who required invasive mechanical ventilation, duration of ventilator days was provided for 14 patients, with a median of 40 days (interquartile range=54). Twenty-one (27.6%) patients were found to have cysts on imaging, 26 (34.2%) were found to have bullae, 3 (3.9%) were found to have blebs, 15 (19.7%) were found to have pneumatoceles, and 11 (14.5%) were found to have more than one of the aforementioned findings. A total of 53 (69.7%) patients developed pneumothorax and 12 (15.8%) developed pneumomediastinum. Seventeen (22.4%) patients were on the mechanical ventilator while pulmonary complications occurred. Additionally, 41 (53.9%) required chest tube placement, 16 (21.1%) required surgical intervention including open thoracotomy or video assisted thoracoscopy. A total of 47 (61.8%) cases reported either resolution of symptoms and complications, or improved imaging findings following interventions. The rate of inpatient mortality was 11.8%. CONCLUSIONS: Patients with severe COVID-19 may have a higher risk for developing cystic lung disease, hence, increasing the risk for complications such as pneumothorax and pneumomediastinum. CLINICAL IMPLICATIONS: Patients who had severe COVID-19 may benefit from closer follow up and serial imaging for early detection of cystic lung disease. DISCLOSURES: No relevant relationships by Kavita Batra No relevant relationships by Rajany Dy No relevant relationships by Christina Fanous No relevant relationships by Wilbur Ji No relevant relationships by Max Nguyen No relevant relationships by Omar Sanyurah