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• W4304146145 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case PostersSESSION TYPE: Case Report PostersPRESENTED ON: 10/19/2022 12:45 pm - 01:45 pmINTRODUCTION: Granulomatosis with polyangiitis (GPA) is a predominantly small to medium vessel vasculitis with variable symptoms typically involving upper and lower airways and renal involvement as well. We present an exclusive case of pulmonary limited GPA without positive serum markers, diagnosed by pathology.CASE PRESENTATION: 61-year-old male with a past medical history of CKD III, diabetes, and heart failure presented with acute encephalopathy, acute kidney injury, and failure to thrive. On evaluation, a chest x-ray was obtained to assess for possible ongoing infectious process, which denoted a right upper lobe lesion. A CT chest was obtained which noted multiple large right lung nodular opacities with peripheral ground glass. He was treated with broad-spectrum antibiotics while infectious evaluation including fungal serologies was completed. Infectious workup was unrevealing, and a CT-guided needle biopsy of the peripheral pulmonary nodule was performed with pathology revealing necrotizing granulomatous inflammation with necrosis and vascular inflammation consistent with GPA. He then underwent kidney biopsy due to acute kidney injury and concern for vasculitis involvement. The biopsy was consistent with acute tubular necrosis and global glomerulosclerosis with no evidence of vasculitis. ANCA, MPO, and PR3 remained negative. 60mg prednisone and azathioprine were started.DISCUSSION: GPA is a small and medium vessel vasculitis that typically involves the upper and lower respiratory tracts in addition to renal involvement. Symptoms associated with GPA are nonspecific and vary depending on the organ systems involved. 90% of patients that have GPA are ANCA positive, making it an extremely sensitive test (1). However, a negative ANCA does not exclude the diagnosis of GPA as it can be negative in up to 20% of cases (2). GPA often involves pulmonary and renal systems; however, sole pulmonary involvement is possible. Pulmonary manifestations include lung nodules, alveolar hemorrhage, and ground-glass opacities and often are bilateral in presentation (3). GPA can be associated with interstitial lung disease in up to 23% of cases which adds to the complexity of disease management (3). In this case, we present a patient with pulmonary limited GPA and seronegative testing. This is a complex case with varying differential diagnoses of fungal or bacterial infection, malignancy, or vasculitis process all of which have widely different treatments.CONCLUSIONS: Pulmonary involvement is seen with multiple types of vasculitis diseases but typically have positive serum markers. If the vasculitis process is not correctly identified, it can lead to unnecessary treatments and allow for progression of disease. In patients with vasculitis, further lung parenchyma destruction can lead to interstitial lung disease or airway obstruction, which is why seronegative vasculitis should be considered in cases with pulmonary nodules.Reference #1: Qasim A, Patel JB. ANCA Positive Vasculitis. [Updated 2022 Feb 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK554372/Reference #2: Langford C. Clinical features and diagnosis of small-vessel vasculitis. Cleve Clin J Med. 2012 Nov;79 Suppl 3:S3-7. doi: 10.3949/ccjm.79.s3.01. PMID: 23203642.Reference #3: Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F. Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis. Clin Exp Rheumatol. 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22. PMID: 32324122.DISCLOSURES: No relevant relationships by Jason BallengeeNo relevant relationships by Said ChaabanNo relevant relationships by Sara Qutubuddin SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Granulomatosis with polyangiitis (GPA) is a predominantly small to medium vessel vasculitis with variable symptoms typically involving upper and lower airways and renal involvement as well. We present an exclusive case of pulmonary limited GPA without positive serum markers, diagnosed by pathology. CASE PRESENTATION: 61-year-old male with a past medical history of CKD III, diabetes, and heart failure presented with acute encephalopathy, acute kidney injury, and failure to thrive. On evaluation, a chest x-ray was obtained to assess for possible ongoing infectious process, which denoted a right upper lobe lesion. A CT chest was obtained which noted multiple large right lung nodular opacities with peripheral ground glass. He was treated with broad-spectrum antibiotics while infectious evaluation including fungal serologies was completed. Infectious workup was unrevealing, and a CT-guided needle biopsy of the peripheral pulmonary nodule was performed with pathology revealing necrotizing granulomatous inflammation with necrosis and vascular inflammation consistent with GPA. He then underwent kidney biopsy due to acute kidney injury and concern for vasculitis involvement. The biopsy was consistent with acute tubular necrosis and global glomerulosclerosis with no evidence of vasculitis. ANCA, MPO, and PR3 remained negative. 60mg prednisone and azathioprine were started. DISCUSSION: GPA is a small and medium vessel vasculitis that typically involves the upper and lower respiratory tracts in addition to renal involvement. Symptoms associated with GPA are nonspecific and vary depending on the organ systems involved. 90% of patients that have GPA are ANCA positive, making it an extremely sensitive test (1). However, a negative ANCA does not exclude the diagnosis of GPA as it can be negative in up to 20% of cases (2). GPA often involves pulmonary and renal systems; however, sole pulmonary involvement is possible. Pulmonary manifestations include lung nodules, alveolar hemorrhage, and ground-glass opacities and often are bilateral in presentation (3). GPA can be associated with interstitial lung disease in up to 23% of cases which adds to the complexity of disease management (3). In this case, we present a patient with pulmonary limited GPA and seronegative testing. This is a complex case with varying differential diagnoses of fungal or bacterial infection, malignancy, or vasculitis process all of which have widely different treatments. CONCLUSIONS: Pulmonary involvement is seen with multiple types of vasculitis diseases but typically have positive serum markers. If the vasculitis process is not correctly identified, it can lead to unnecessary treatments and allow for progression of disease. In patients with vasculitis, further lung parenchyma destruction can lead to interstitial lung disease or airway obstruction, which is why seronegative vasculitis should be considered in cases with pulmonary nodules. Reference #1: Qasim A, Patel JB. ANCA Positive Vasculitis. [Updated 2022 Feb 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK554372/ Reference #2: Langford C. Clinical features and diagnosis of small-vessel vasculitis. Cleve Clin J Med. 2012 Nov;79 Suppl 3:S3-7. doi: 10.3949/ccjm.79.s3.01. PMID: 23203642. Reference #3: Sebastiani M, Manfredi A, Vacchi C, Cassone G, Faverio P, Cavazza A, Sverzellati N, Salvarani C, Luppi F. Epidemiology and management of interstitial lung disease in ANCA-associated vasculitis. Clin Exp Rheumatol. 2020 Mar-Apr;38 Suppl 124(2):221-231. Epub 2020 Apr 22. PMID: 32324122. DISCLOSURES: No relevant relationships by Jason Ballengee No relevant relationships by Said Chaaban No relevant relationships by Sara Qutubuddin" @default.
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• W4304146145 title "PULMONARY LIMITED SERONEGATIVE GRANULOMATOSIS WITH POLYANGIITIS VASCULITIS" @default.
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