A Rare Case of Granulomatosis with Polyangiitis Presenting with Esophagitis

Introduction: Granulomatosis with polyangiitis (GPA) is a rare autoimmune disorder characterized by necrotizing granulomas, mainly affecting the upper respiratory tract, lungs, and kidneys.The typical presentation of kidney involvement is in the form of RPGN.The exact etiology of GPA remains unknown. Diagnosis should be suspected in any patient with nonspecific constitutional symptoms, clinical evidence of glomerulonephritis with upper or lower respiratory tract involvement, and lab evidence of positive ANCA serology. Here, we present a case of a patient with a baseline serum creatinine of 1.5 who initially presented with esophagitis and was ultimately found to have GPA. Case Description: 76 y/o Caucasian female with PMH of hyperlipidemia, anemia, and GERD who initially presented to outside facility due to weakness and diarrhea. Found to be COVID-19 positive and anemic, with Hb 7.6 and a Hct 23. She underwent EGD and colonoscopy, which revealed esophagitis, esophageal candidiasis, and gastritis. She was discharged after receiving conservative management. She presented again to the same facility 2 weeks later with acute hypoxic respiratory failure. Labs revealed Hb of 5.6 and Cr of 10.40. After initial stabilization, she was transferred to our facility for higher care. Labs revealed Hb 8.2, Na 131, K 5.8, S. Cr 10.58, GFR 3.6. Urinalysis- 2+ protein, 3+ blood, leuk esterase, and many bacteria. ANA, C3, C4, PR3-ANCA, and GBM Ab negative. Protein electrophoresis revealed an M spike. Her C-ANCA titer was found to be greater than 1:640. A kidney biopsy showed necrotizing and crescentic glomerulonephritis with mild interstitial fibrosis and tubular atrophy, consistent with GPA. She was initially started on SLED followed by HD with steroids and Rituximab. Discussion: Due to the wide variety of possible associated clinical manifestations, initial diagnosis of GPA can be difficult. In the case presented in this paper, the patient initially presented to an outside hospital with esophagitis. She did not undergo further workup for her symptoms and presentation until she re-presented with AKI and respiratory distress. At that time, GPA rose on the differential and was confirmed via kidney biopsy. This case showcases a rare presentation of GPA with esophagitis, which we hope to highlight to improve physician awareness.