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Eosinophilic Granulomatosis With Polyangiitis (EGPA) Hidden in Acute Appendicitis: A Case Revealed Through
Yoshiyuki Chiba1, Naoki Negami2, Yasunori Isido2
1General Surgery, Juntendo University Nerima Hospital, Tokyo, JPN.
Abstract:
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of necrotising vasculitis characterised by eosinophilic infiltration and systemic involvement, most commonly affecting the lungs, skin, and peripheral nerves. Gastrointestinal (GI) manifestations are common in patients with EGPA, although histopathological confirmation of GI involvement is infrequent. Acute appendicitis is a common surgical emergency, but its presentation as an initial manifestation of EGPA is exceptionally uncommon. We report the case of a 54-year-old man with a history of asthma who presented with fever and epigastric pain that later migrated to the right lower quadrant. Laboratory tests revealed leukocytosis and elevated C-reactive protein levels. Abdominal computed tomography (CT) demonstrated mild appendiceal enlargement, and a diagnosis of acute appendicitis was made. The patient underwent laparoscopic appendectomy. Postoperatively, he developed persistent lower abdominal pain, new-onset bilateral lower extremity pain, and marked eosinophilia (60%). Histopathological examination of the resected appendix revealed dense eosinophilic infiltration and necrotising vasculitis, establishing the diagnosis of ANCA-negative EGPA. This phenotype is known to present with gastrointestinal manifestations and peripheral eosinophilia frequently. He was subsequently transferred to a specialised centre for further treatment. This case highlights the potential for EGPA to mimic acute appendicitis. When persistent eosinophilia and atypical postoperative symptoms are present, systemic diseases such as EGPA should be considered. Histopathological analysis plays a pivotal role in identifying underlying systemic vasculitis, particularly in cases with unusual clinical presentations. Greater awareness of EGPA as a differential diagnosis in atypical appendicitis may facilitate earlier recognition and timely management.
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