Surgical management of isolated mesenteric autoimmune disease: Addressing the spectrum of IgG4-related disease and sclerosing mesenteritis

Alissa Greenbaum, Nour Yadak, Steven Perez, Ashwani Rajput

Research output: Contribution to journalArticlepeer-review

Abstract

IgG4-related disease (IgG4-RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG4and tissue IgG4levels, specific histopathological findings, multiorgan involvement and adequate response to glucocorticoid treatment. The low incidence and the heterogeneous nature of the disease has made consensus on diagnostic criteria for IgG4-RD difficult. Whether sclerosing mesenteritis (SM) is considered a manifestation of IgG4-RD is strongly debated. We present the case of a patient with a history of rheumatoid arthritis who presented with a calcified abdominal mass. She was found to have an isolated, pedunculated mesenteric mass positive for IgG4and concurrently elevated serum IgG4levels. Clinical features did not classify her disease as either SM or IgG4-RD as currently described in consensus statements. Concurrent diagnoses of IgG4-RD, SM and other autoimmune disorders, as well as postoperative recommendations for resected isolated IgG4-positive masses, are discussed.

Original languageEnglish (US)
Article number681
JournalBMJ case reports
Volume2017
DOIs
StatePublished - 2017
Externally publishedYes

Keywords

  • Gastroenterology
  • Gastrointestinal surgery
  • Immunology
  • Rheumatology

ASJC Scopus subject areas

  • Medicine(all)

Fingerprint

Dive into the research topics of 'Surgical management of isolated mesenteric autoimmune disease: Addressing the spectrum of IgG<sub>4</sub>-related disease and sclerosing mesenteritis'. Together they form a unique fingerprint.

Cite this