AB1369 Elevated ca-125 in igg4-related mesenteric disease: a red herring? a systematic literature review

Abstract

BackgroundMesenteric panniculitis (MP), a rare fibrotic inflammatory disease of the bowel mesentery, can be a rare manifestation of IgG4-related disease (IgG4-RD). IgG4-RD is a chronic inflammatory disease most commonly affecting the pancreas, characterised by infiltration of IgG4-positive plasma cells and lymphocytes into various organs.1 We recently encountered a male patient with IgG4-related MP who was incidentally found to have a very high level of CA-125, which correlated with CRP levels and normalised after steroid treatment. This prompted a systematic literature review (SLR) to better understand this unexpected phenomenon.ObjectivesTo investigate for associations between CA-125, MP and/or IGG4-RD, understand possible common pathophysiological mechanisms and explore potential clinical implications.MethodsThe SLR was performed using MEDLINE, EMBASE, Web of Science, and Scopus, looking for literature on either MP and CA-125 or IgG4-RD and CA-125 up to January 8 2018, using a comprehensive search strategy with relevant mesh terms and keywords linked to the above broad categories. Literature screening was performed by two independent reviewers.Results24 unique citations were found, of which 13 were unanimously identified as relevant by the two reviewers. The final selected articles included: 8 case reports, 3 conference abstracts of case reports, 1 cohort study of 22 patients, and a retrospective study of 7 patients (table 1). CA-125 was raised in 22/40 patients in the identified reports (shown in red), including males, and was often the only elevated tumour marker (yellow). We also report on the presence of effusions (blue), as this may be linked to the causal mechanism.Table 1 The clinical and laboratory characteristics of cases of IgG4-RD and MPAbstract AB1369 – Figure 1AIAutoimmune, AIH: Autoimmune Hepatitis, AIP: Autoimmune Pancreatitis, SM: Sclerosing Mesenteritis, ↑: Raised, ↔: Normal ConclusionsTo our knowledge, this is the first SLR exploring the association between CA-125 and IgG4-RD and MP. Despite the small sample, our results do indicate that CA-125 was raised in more than 50% of reported cases, the majority of which also had some kind of effusion. Although traditionally a marker of ovarian cancer, this report highlights that a raised CA-125 can be found in other, non-malignant, inflammatory conditions, and potentially correlates with inflammatory burden. CA-125 in this setting may thus represent a useful biomarker and have a role in monitoring treatment response.Reference[1] Minato H, Shimizu J, Arano Y, et al. IgG4-related sclerosing mesenteritis: a rare mesenteric disease of unknown etiology. Pathol Int. 2012;62:281–6.Disclosure of InterestNone declared