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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
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Background: The etiology of pancreatitis is heterogeneous;it may be due to chronic excessive alcohol use, gallstones, medications, infections, autoimmune diseases, metabolic disorders, trauma, congenital malformations etc. Objectives: The aim of this study has been to describe a hypothetic pathway of chronic pancreatitis in post-Covid 19 condition, based on the role of autoimmune and bacterial septic vasculitis in the pathogenesis of chronic pancreatitis (ChrP) in rheumatoid arthritis (RA). Methods: At the National Institute of Rheumatology 9475 patients died between 1969 and 1992;among them 161 with RA and all of them were autopsied. RA was confrmed clinically according to the criteria of the ACR. Tissue samples of pancreas were available for histologic evaluation in 111 patients. Pancreatitis and vasculitis were determined and characterized histo-logically [1,2]. The possible role of autoimmune vasculitis and bacterial septic vasculitis in the pathogenesis of ChrP was analyzed by Pearson's chi-squared (χ2) test. Results: ChrP-characterized by diffuse and/or focal fbrosis and atrophy-was present in 10 (9.01%) of 111 patients. Systemic vasculitis complicated RA in 28 (25.23%) of 111 patients. Twenty-five (89.3 %) of 28 systemic vasculitis proved to be of autoimmune origin. Autoimmune vasculitis involved the pancreatic blood vessels (pAV) in 8 (32.0 %) of these 25 patients. Non-specifc (n=37), fbrinoid necrotic (n=14), and granulomatous type (n=5) of pAV were detected side by side in the same histologic section, involving pancreatic arteries of different sizes. The veins and venules were not involved. p AV was not associated with chronic pancreatitis. The relationship between p AV and ChrP was inverse with a negative colliquation coefficient (c=-1.0, χ2=0. 0801, p <0.7772-NS). Three (10.7 %) of 28 systemic vasculitis cases proved to be of septic (bacterial) origin. Pancreatic blood vessels (pSV) were involved in 2 (66.7 %) of these 3 patients. Granulomatous vasculitis was not seen with pSV, and the veins and venules were also spared. Non-specifc (n=9), fbrinoid necrotic (n=2) vasculitis involving different size of pancreatic arteries were associated with chronic pancreatitis. The relationship between pAV and ChrP was signifcant (c=0.92308, χ2=6.3201, p <0.012). Conclusion: Chronic pancreatitis is characterized clinically by abdominal pain and diarrhea, which are common in post-Covid 19 condition [2]. The strong and signifcant correlation between pSV and ChrP indicates that subclinical or manifest bacterial septic processes may play a role in the pathogenesis of ChrP. Hypothetically a similar pathway is plausible in post-Covid 19 chronic pancreatitis due to viral infection and vasculitis, analogous to bacterial septic vasculitis. Systemic vasculitis of autoimmune origin involving blood vessels of the pancreas may cause a special multifocal relapsing lipo-necrotic pancreatitis [1,2], and according to our results, do not influence the prevalence of ChrP. The autoimmune origin of pancreatic vasculitis may be excluded histologically by the presence of granulomatous vasculitis of the most frequently involved arterioles and small arteries.