Summary

IgG₄-related disease (IgG₄-RD) is a rare form of autoimmune sclerosing disease, characterised by elevated serum IgG₄ and tissue IgG₄ levels, 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 IgG₄ and concurrently elevated serum IgG₄ levels. 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.