• Adriano Basso Dias, Natally Horvat, Maria Dirlei Begnami, Emerson Shigueaki Abe, Publio Cesar Cavalcante Viana, and MachadoMarcel Cerqueira CesarMCCDepartment of Surgery, Hospital Sírio-Libanês, São Paulo, Brazil..
• Department of Radiology.
• Medicine (Baltimore). 2020 Jun 19; 99 (25): e20588.

RationaleImmunoglobulin G4 (IgG4)-related disease is an increasingly recognized immune-mediated entity that can affect virtually every organ system. Depending on the location of the disease, it can present a wide range of clinical manifestations and even mimic malignancies. Appendiceal involvement in patients with IgG4-related disease is particularly rare and very few cases are reported in the literature.Patient ConcernsWe report a case of IgG4-related appendiceal disease in a 42-year-old woman who presents with a subacute onset of right lower quadrant abdominal pain.DiagnosisAbdominal computed tomography showed a markedly enlarged appendix, raising the concern of malignancy. The diagnosis of IgG4 appendiceal disease was confirmed by postoperative histopathologic and immunohistochemical examination.InterventionsThe patient underwent right hemicolectomy.OutcomesAfter the surgery, the patient had an uneventful recovery and reported a resolution of her symptoms. The serum IgG4 was revaluated 5 days after surgery and returned to its normal values. At the 3-year follow up, the patient had no recurrence of symptoms and her imaging exams remain unremarkable.LessonsThis study reports the fifth case of IgG4-related appendiceal disease. Increasing awareness of this condition may influence the management of these patients, once patients with IgG4-related disease should be monitored after treatment, due to the risk of recurrence or involvement of other organs.

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