S1415 Granulomatosis With Polyangiitis Presenting as Acute Cholecystitis

Abstract

INTRODUCTION: Granulomatosis with Polyangiitis (GPA) is a systemic autoimmune disease that mainly affects small vessels and can affect any organ system. It is one of the ANCA associated vasculitides, defined by vascular inflammation associated with autoantibodies known as ANCA. Although rarely seen, GPA can affect the GI system and present with GI complications. CASE DESCRIPTION/METHODS: A 27-year-old female initially presented to the ED with lower extremity weakness, joint pain and swelling, then returned a month later after recent diagnosis of rheumatoid arthritis (RA) with epigastric and right upper quadrant pain. CT abdomen/pelvis and labwork were insignificant. She returned the following day for persistent symptoms. Abdominal ultrasound confirmed cholelithiasis and possibly cholecystitis. She underwent cholecystectomy and was subsequently discharged but soon returned with fevers, nausea, chills, sweats and epigastric pain radiating to the right shoulder. EGD showed findings of gastritis. MRCP and ERCP were unremarkable. Gallbladder pathology showed cholelithiasis and findings consistent with small vessel vasculitis. Further workup was positive for protease 3 antibody, consistent with GPA. She was treated with pulse dose steroids, plasmapheresis and started on Rituxan infusion. Non-opioid pain medications were given while opioids and steroids were tapered. Her abdominal pain improved and she was discharged with outpatient follow up. DISCUSSION: In a retrospective review, GI involvement, most commonly abdominal pain, was found in varying percentages of patients with polyarteritis nodosa, ANCA vasculitides including GPA and RA associated vasculitis. Biopsies from 36 patients via EGD found no signs of vasculitis. Four of five patients with cholecystectomy for cholecystitis were shown to have vasculitis. In an analysis of case records of 34 patients with GPA, nine of 34 had GI complications. EGD samples revealed nonspecific inflammation. The insignificant findings on EGD in the patient cases as well as the unremarkable ERCP and MRCP on our patient show that it is worthwhile to question the necessity of continuous procedures in patients with GPA who present with persistent abdominal pain. It is important to consider systemic vasculitis in patients with unclear causes of GI symptoms especially in those with history of rheumatological disorder. Benefits include avoiding unnecessary imaging and procedures as well as decreased hospital stay thereby utilizing high value care and improving patient experience.Figure 1.: Ultrasound showing cholelithiasis without any wall thickening. There was positive Murphy's sign while obtaining the imaging.Figure 2.: Images show a small artery with fibrinoid necrosis of the vessel wall. There is infiltration in the vessel wall and in the surrounding tissue by neutrophils and lymphocytes. Each image shows varying degrees of obliteration of the vessel lumen with Figures 2.1 and 2.2 showing complete obliteration.