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• W4387248045 abstract "SESSION TITLE: Pulmonary Manifestations of Systemic Disease Case Report Posters 16 SESSION TYPE: Case Report Posters PRESENTED ON: 10/10/2023 09:40 am - 10:25 am INTRODUCTION: Eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA) are two ANCA-associated vasculitides that can be indistinguishable on biopsy. These two disease processes are closely related and have multiple overlapping similarities. In this case presentation, we present a patient that blurs the lines of GPA vs. EGPA diagnoses. CASE PRESENTATION: A 32-year-old female with a past medical history of asthma, obstructive sleep apnea, obesity, hypothyroidism, and renal disease on hemodialysis who was diagnosed with GPA 12 years ago based on P-ANCA positive, MPO positive, and kidney biopsy showing necrotizing glomerulonephritis came in for hyperkalemia due to missed hemodialysis. She also had a diffuse macular, purple-colored rash, initially considered uremic folliculitis. After hemodialysis corrected potassium, the patient was downgraded to the medicine floor. CODE BLUE was called secondary to hyperkalemia on the seventh day of hospitalization. The patient was intubated, and ROSC was achieved. Underwent hemodialysis again in ICU. On chart review, the patient was noted to have elevated eosinophils on admission at 2800. However, eosinophils at the time of kidney biopsy and diagnosis of end-stage renal disease secondary to related glomerulonephritis 12 years ago were normal. A punch biopsy of skin lesions was performed, showing eosinophilic vasculitis. The patient was found to be P-ANCA and MPO ab positive with markedly elevated IgE levels. Given the history of asthma, peripheral eosinophilia, pansinusitis, and skin biopsy results, a diagnosis of EGPA was established. The patient was started on steroids and cyclophosphamide. She was extubated on Day 15 and weaned to a nasal cannula. The patient was discharged home. DISCUSSION: Although the patient had a diagnosis of GPA 12 years ago, there is sufficient evidence for the diagnosis of EGPA by the American College of Rheumatology and Lanham diagnostic criteria following this hospital stay. However, these criteria would not have been met in the absence of pansinusitis and eosinophilia 12 years prior. Thus, this begs the question of whether this was a misdiagnosis or if these ANCA-associated vasculitides are so closely related that they can transform morphologies or are among a continuum of diagnoses as an overlap syndrome. Although she has atypical features, it would be difficult to discredit either diagnosis. Thus, this leans toward a transformation in pathology vs. overlap syndrome. It may be uncommon, given continued maintenance therapy with rheumatology follow-up. CONCLUSIONS: Diagnosis of EGPA and GPA can have multiple overlapping features. With the two being on a spectrum of ANCA-associated vasculitides, they can behave similarly and morphologically transform from one to another. It may be seen less frequently due to the follow-up and remission of the disease with current therapies. REFERENCE #1: Quan MV, Frankel SK, Maleki-Fischbach M, Tan LD. A rare case report of polyangiitis overlap syndrome: granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis. BMC Pulm Med. 2018 Nov 29;18(1):181. doi: 10.1186/s12890-018-0733-2. PMID: 30497438; PMCID: PMC6267840. DISCLOSURES: No relevant relationships by Joshiah Gordon No relevant relationships by Jeremy Renz No relevant relationships by ALEENA SAMMAR No relevant relationships by Zeeshan Zia" @default.
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• W4387248045 date "2023-10-01" @default.
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• W4387248045 title "EGPA IMITATING GPA: A MISDIAGNOSIS OR EVOLVING PATHOPHYSIOLOGY?" @default.
• W4387248045 doi "https://doi.org/10.1016/j.chest.2023.07.3593" @default.
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