Ileocecal IgG4-Related Disease: A Case Report Mimicking Malignancy

Akbari, Ardalan; Carruthers, Mollie; Lee, Lawrence

doi:10.14309/02075970-201805000-00084

Cited by 3 publications

(1 citation statement)

References 13 publications

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“…A. Harada Є повідомлення про випадки IgG4-АЗ з ураженням ілеоцекальної ділянки (і утворенням, що викликає непрохідність) [1] та з ураженням апендикса, що імітує хворобу Крона, апендицит або пухлину апендикса [12,28,32]. Однак лише у кількох цих випадках було визначено s-IgG4, а клінічні, макроскопічні та мікроскопічні ознаки значно перекривалися запальним захворюванням кишечнику (ЗЗК).…”

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Frequency, clinical picture, diagnosis, and differential diagnosis of the pathology of the digestive tract (esophagus, stomach, and bowel) in IgG4-related disease (autoimmune pancreatitis)

Gubergrits

2023

Bul. of the Club of Pancr.

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Despite the publication of European guidelines for the diagnosis and treatment of IgG4-related disease (IgG4-RD), this problem is very complicated from a practical point of view. There are many publications about autoimmune pancreatitis and liver and biliary tract lesions in IgG4-RD, but the data on the digestive tract lesions are not sufficient. Gastrointestinal manifestations of IgG4-RD are considered to be rare or very rare. IgG4-RD presenting as esophageal lesions is rare condition. Only single clinical cases are available. I. Obiorah et al. reported the highest number of cases of IgG4-related esophagitis (8 cases). Interestingly, 7/8 cases reported by I. Obiorah et al. were males (age range: 18 to 79 years), and most patients had dysphagia as the most prominent symptom, with erosions and strictures at endoscopy. Histologically, all patients had a lymphoplasmacytic infiltrate and storiform fibrosis, while obliterative phlebitis was observed in 3/8 patients. Hence, 2 of the 3 major histological “Boston criteria” of IgG4-RD were fulfilled in all patients. Besides, all patients had at least 50 IgG4-positive cells per HPF (range: 50–110), and IgG4/IgG ratios of at least 50% (range: 50–90%). Of I. Obiorah’s patients, s-IgG4 had only been measured in three — all of whom had values in the normal range. Esophageal strictures were identified in three patients, two patients had achalasia. Based on K. Notohara’s series and the other published cases, it can be concluded that gastric IgG4-RD often (but not always) seems to be clinically asymptomatic, that s-IgG4 is elevated in a considerable number of cases, and histologically, that at least two of the Boston criteria often are found. The recommended cut-off of 50 IgG4-positive cells and IgG4/IgG ratios of >40% was often fulfilled in the reported cases of IgG4-RD. There are few reports of IgG4-RD involving the duodenum, duodenal papilla, ileum with small bowel obstruction caused by pseudotumor or sclerosing disease of the small bowel with luminal narrowing. There are case reports of IgG4-RD with lesion of ileocecal area with an obstructing ileocecal mass and appendix, mimicking Crohn’s disease, appendicitis or appendiceal tumor. IgG4-related inflammatory bowel disease may — rarely — also be one of manifestations of IgG4-RD. Treatment should be conducted in accordance with European guidelines.

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Frequency, clinical picture, diagnosis, and differential diagnosis of the pathology of the digestive tract (esophagus, stomach, and bowel) in IgG4-related disease (autoimmune pancreatitis)

Gubergrits

2023

Bul. of the Club of Pancr.

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A reappraisal of sclerosing nodular and/or polypoid lesions of the gastrointestinal tract rich in IgG4‐positive plasma cells

Chetty

2020

Histopathology

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AimsTo describe additional cases of nodular and polypoid sclerosing lesions of the gastrointestinal tract (GIT) that are associated with numerous IgG4‐positive plasma cells, review the pertinent literature to ascertain the relationship with systemic IgG4‐related disease, and provide a reporting framework for such lesions.Methods and resultsFive new cases of sclerosing polyps or nodules were collected over a 10‐year period, occurring in four females and one male ranging in age from 32 years to 56 years (mean, 41.6 years). Patients were asymptomatic or had epigastric pain, and one had rectal bleeding. None had autoimmune or other obvious IgG4‐related disease, and serum IgG4 levels were normal. All were solitary nodules in the stomach (two cases), ileum, caecum, and rectum. Four lesions were submucosal and one was subserosal; all were well circumscribed, composed of hyalinised, keloidal fibrous tissue with lymphoplasmacytic inflammation. Obliterative phlebitis was not seen. Lineage‐specific immunomarkers were negative. In excess of 10 IgG4‐positive plasma cells per high‐power field were seen, and the IgG4/IgG ratios were >0.4.ConclusionsVery few IgG4‐related lesions in the tubular GIT are associated with disease at other sites and/or elevated serum IgG4 levels. The majority may represent a lesion in the spectrum of IgG4‐related disease. The use of the term ‘IgG4‐positive nodule or polyp with probable histological features of IgG4‐related disease’ is advocated for nodular and/or polypoid lesions in the GIT with ≥10 IgG4‐positive plasma cells in a high‐power field and an IgG/IgG4 ratio of >0.4.

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Imaging findings of immunoglobulin G4-related disease: from the head to the pelvis

Ichikawa¹,

Onishi²

2021

smedj

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Ileocecal IgG4-Related Disease: A Case Report Mimicking Malignancy

Cited by 3 publications

References 13 publications

Frequency, clinical picture, diagnosis, and differential diagnosis of the pathology of the digestive tract (esophagus, stomach, and bowel) in IgG4-related disease (autoimmune pancreatitis)

Frequency, clinical picture, diagnosis, and differential diagnosis of the pathology of the digestive tract (esophagus, stomach, and bowel) in IgG4-related disease (autoimmune pancreatitis)

A reappraisal of sclerosing nodular and/or polypoid lesions of the gastrointestinal tract rich in IgG4‐positive plasma cells

Imaging findings of immunoglobulin G4-related disease: from the head to the pelvis

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