A CASE OF GRANULOMATOSIS WITH POLYANGIITIS

SESSION TITLE: Critical Systemic Disease Case Report Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Granulomatosis with polyangiitis(GPA) is an autoimmune small vessel vasculitis that is included in the group of anti-neutrophilic cytoplasmic antibody(ANCA)- associated small vessel vasculitides (AAVs). GPA is a systemic disease, however acronym ELK is used to describe the most common involvement of Ear, nose, throat, Lungs, and Kidneys. We report a case of GPA, highlighting its presentation. CASE PRESENTATION: 59-year old female presented with vaginal bleeding, malaise, blurry vision, non productive cough and shortness of breath few days after receiving COVID-19 vaccine. Physical exam was remarkable for bilateral conjunctival injection with right sided ptosis and inguinal lymphadenopathy. Laboratory findings were significant for acute kidney injury and anemia. Computed tomography (CT) of chest revealed bilateral bronchovascular nodules and masses with interlobular septal thickening and enlarged mediastinal lymph nodes. Fine needle aspiration of left inguinal lymph node was negative for malignancy. Bronchoscopy with bronchial brush revealed alveolar hemorrhage with capillaritis;bronchoalveolar lavage(BAL) showed hemosiderin laden macrophages. Tissue biopsy was negative for malignancy. Testing for pulmonary renal syndrome was positive for C-ANCA and proteinase-3 (PR-3) antibodies. Anti-GBM antibody and anti-MPO antibody was negative. Plasmapheresis (PLEX) and pulse dose steroids were initiated however the patient was unable to tolerate the treatment. Her clinical condition continued to decline requiring multiple pressors, broad spectrum antibiotics and continuous renal replacement therapy. She was transitioned to comfort care per family's wishes and passed away. DISCUSSION: GPA is a rare necrotizing granulomatous vasculitis of small to medium sized vessels that can affect any organ but mainly involves the upper and lower respiratory tract. Necrotizing glomerulonephritis is common. Pulmonary involvement can include consolidation, tracheal or subglottic stenosis, diffuse alveolar hemorrhage, pleural effusion and interstitial lung disease. Lymphadenopathy, as seen in our patient is a rare presentation. Tissue biopsy is crucial for the diagnosis. Association with PR-3 ANCA is seen in more than 80% of GPA patients. Cases of AAVs after administration of COVID vaccine have been reported in the literature, although it is difficult to demonstrate causal relationship. Treatment of GPA with immunosuppression, usually corticosteroids, rituximab or cyclophosphamide, is recommended. The role of PLEX continues to evolve with emerging data, but use of this therapy is reasonable for patients with severe kidney injury and DAH secondary to active vasculitis refractory to immunosuppressive therapy. CONCLUSIONS: Early diagnosis of GPA is challenging as it can mimic metastatic lung malignancy. It should be considered in a broad range of differentials when evaluating patients presenting with pulmonary nodules. Reference #1: Greco A, Marinelli C, Fusconi M, Macri GF, Gallo A, De Virgilio A, Zambetti G, de Vincentiis M. Clinic manifestations in granulomatosis with polyangiitis. Int J Immunopathol Pharmacol. 2016 Jun;29(2):151-9. doi: 10.1177/0394632015617063. Epub 2015 Dec 18. PMID: 26684637;PMCID: PMC5806708. Reference #2: Kitching, A. R., Anders, H. J., Basu, N., Brouwer, E., Gordon, J., Jayne, D. R., Kullman, J., Lyons, P. A., Merkel, P. A., Savage, C., Specks, U., & Kain, R. (2020). ANCA-associated vasculitis. Nature reviews. Disease primers, 6(1), 71. https://doi.org/10.1038/s41572-020-0204-y Reference #3: Szymanowska-Narloch, A., Gawryluk, D., Błasińska-Przerwa, K., & Siemińska, A. (2019). Atypical manifestations of granulomatosis with polyangiitis: the diagnostic challenge for pulmonologists. Advances in respiratory medicine, 87(6), 244–253. https://doi.org/10.5603/ARM.2019.0062 DISCLOSURES: No relevant relationships by Sean Davidson No relevant relationships by Eric Flenaugh No relevant relationships by Marilyn Foreman No relevant relationships by KOMAL KAUR No relevant relationships by Gabriela Oprea-Ilies

Sickle Cell Disease (SCD) As a Risk for COVI19 Compared to Those without SCD Among Patients Admitted in a Large Urban Center, As Estimated By PCR Sars-v-2 Positive Vs Negative Testing

A diagnosis of SCD is considered to be at risk for COVD19. To further define the association between SCD and infection with COVID-19, we estimated risk, by comparing presence or absence of COVID19 infections in individuals with and without SCD admitted concurrently to a large urban health care facility (Grady Memorial Hospital, Atlanta, GA;960 beds, 5th largest public hospital in the US). Primary outcome was a positive or negative COVID-19 diagnosis as defined bySARS-CoV-2 PCR testing. A patient was considered to be COVID-19 positive if tested positive withSARS-CoV-2 PCR for the first time, anytime during the study period, irrespective of number of tests. A patient was considered to be COVID-19 negative if patient had no positive tests during the study period, and had one or moreSARS-CoV-2 PCR negative tests. For COVID19 positive patients, the admission of theSARS-CoV-2 PCR positive test was included in the analysis. For COVID19 negative patients, the first admission with aSARS-CoV-2 PCR negative test was considered for analysis. For this interim analysis, SCD was defined by ICD10 and registry data. Clinical diagnosis such as obesity and respiratory failure were defined by ICD10 coding. Data was obtained from quarterly centralized Epic EMR data extractions. Analysis of outcome of COVID19 positive vs negatives was stratified in four separate analysis: all admissions, ICU admissions, those with respiratory failure and those who died. Multivariate dichotomous logistic regression analyses modeled binary outcome effect of SCD, adjusted for age (<40 vs. > 40 years), sex at birth (females vs. males) and obesity (SAS version 9.4 was used for statistical analyses and overall significance level was set at 0.05). To ensure population homogeneity analysis was conducted on patient ages 20 to 60 years that were Black/African American and admitted from the Emergency Department for a short stay and/or the medicine service (variable interactions at a p<0.01). The study was approved by the institutional review board and by the hospital research oversight committee. Overall, between 3/23/2020 and 6/30/2020, 23697 patients were admitted once or more to Grady Memorial Hospital with one or more PCR sars-cov-2 test, of these 405 were patients with SCD (1.7%). Of the total, 2566 patients (10.8%) tested positive for COVID-19, and 48 patients with SCD (11.8%) were positive. Of 7041 (29.7%) were part of the study population, 332 (4.7%) where patients with SCD (hemoglobin [hb] SS/Sbeta0 =252, hbSC n=55, hbS beta thalassemia+ or hbS beta thalassemia undetermined n=21). Among patients without SCD, 36.3% were female, (n=2557) and among patients with SCD, 53.6% (n=178). The mean age of patients without SCD was: 51.1 years (standard deviation [std]) +/- 19.5 years), and for those with SCD: 35.0 years (std +/- 12.0 years). Results of univariate and multivariate analysis are presented in the table. In conclusion, in a Black/African American patients admitted from the Emergency Room for observation and/or the internal medicine service, when adjusted for age, gender and obesity, with SCD are at a significant increased risk for admissions with COVID-19 infection in general as well as ICU admission or admission with respiratory failures. Further studies can help articulate the risk associated with SCD as well as its potential interaction with other factors, with attention to confounders. [Formula presented] Disclosures: No relevant conflicts of interest to declare.

PUL01.01 Ground Glass Opacity: What's in a Name?

Background: Ground glass opacity (GGO), consists of hazy opacity not obscuring the underlying bronchial structures or pulmonary vessels. the underlying pathology varies from benign conditions (pulmonary edema, infections - including COVID-19, cytomegalovirus, Pneumocystis jirovecii, etc), noninfectious interstitial lung diseases and adenocarcinomas. Methods: Following IRB approval our electronic files from January-July 2020 were searched for sampled GGO in comparison with other types of lung lesions found in our patients. Tissue was collected using tip-tracked guided procedures. We compared association of GGO with adenocarcinoma of the lung with lepidic spread (ADCC-L) (Image 1, 2). Results: 122 pathology cases (male=64) were identified: 40 benign and 82 malignant diagnoses. There were 39 cases of ADCC-L (median [interquartile range] age: 63 [60-71]) and 83 cases with various other lesions (age: 62 [57-67]). GGO were reported in 17 ADCC-L cases and 13 of the other cases. The Fisher exact test statistic demonstrated a statistically significant association between ADCC-L and GGO in our overall (n=122, p=0.0014*) and female (n=58, p=0.0151*) cohorts. Conclusion: 1. Tip-tracked guided procedures obtain diagnostic tissue from these challenging to sample lesions. 2. GGO statistically significant association with AADCC-L in our patient population indicates necessary appropriate follow-up for appropriate patient management. [Formula presented] [Formula presented]