• Huijuan Shao, Dong Liu, Xiaofeng Zheng, Jiucong Zhang, Wenbo Li, Peng Chen, Zhen Qian, Jie Yang, and Dongmei Liu.
• Department of Gastroenterology, The 940 Hospital of Joint Logistic Support Force of PLA, Lanzhou, China.
• Medicine (Baltimore). 2024 Jan 26; 103 (4): e36973e36973.

RationaleGranulomatosis with polyangiitis (GPA) is a systematic autoimmune disease. The typical clinical involvement of GPA entails the upper and lower respiratory tracts, and the kidneys. Gastrointestinal (GI) involvement is uncommon and unless detected and treated promptly, may lead to life-threatening complications such as perforation. We aim to review all available publications since the first description in 1982 dealing with GI perforation in patients with Wegener granulomatosis and draw attention to this serious situation.Patient ConcernsWe present a 54-year-old man diagnosed with GPA who presented initially with nasal symptoms and suffered ileal perforation following Corona Virus Disease 2019 infection. We also review previously reported patients with Wegener granulomatosis who had GI perforation to investigate the perforation site and period, pathology, diagnosis, and treatment methods.Diagnoses And InterventionsThe case of a GPA-diagnosed patient who presented initially with nasal symptoms and suffered ileal perforation following Corona Virus Disease 2019 infection. We recommended a renal puncture biopsy, steroids, and immunosuppressants to improve the patient condition. The patient and his family refused these treatment recommendations.OutcomesOur patient exhibited continued progressive vascular inflammatory changes and eventual irreversible systemic damage. These sequelae were attributed to the patient declining prednisolone and immunosuppressant therapy.LessonsGI perforation is rare in GPA but severe complication. Consequently, we recommend that early diagnosis and treatment with steroid hormones and immunosuppressants for GPA patients with GI perforation.Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.

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