Case report of IgG4-related appendiceal disease: A challenging disease.

Abstract

RATIONALE:

Immunoglobulin 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 CONCERNS:

We 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.

DIAGNOSIS:

Abdominal 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.

INTERVENTIONS:

The patient underwent right hemicolectomy.

OUTCOMES:

After 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.

LESSONS:

This 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.

• Authors: Adriano Basso Dias, Natally Horvat, Maria Dirlei Begnami, Emerson Shigueaki Abe, Publio Cesar Cavalcante Viana, Marcel Cerqueira Cesar Machado
• Journal: Medicine (Medicine (Baltimore)) Vol. 99 Issue 25 Pg. e20588 (Jun 19 2020) ISSN: 1536-5964 [Electronic] United States
• PMID: 32569185 (Publication Type: Case Reports, Journal Article)

Chemical References

• Immunoglobulin G

Topics

• Adult
• Appendix (diagnostic imaging, pathology, surgery)
• Colectomy (methods)
• Diagnosis, Differential
• Female
• Humans
• Imaging, Three-Dimensional
• Immunoglobulin G (blood)
• Immunoglobulin G4-Related Disease (blood, diagnosis, pathology, surgery)
• Tomography, X-Ray Computed