An unusual cause of bilateral orbital swelling: Immunoglobulin G4–related orbital disease arising in a patient with ulcerative colitis

Cholangitis in patients with ulcerative colitis (UC) can lead to misdiagnosis of primary sclerosing cholangitis (PSC). Furthermore, it can mimic cholangiocellular carcinoma, which also can lead to inappropriate and potentially harmful treatment of the patient. An 18-year-old male patient with known UC presented with pain in his right upper abdomen and elevation of the cholestatic liver enzymes (alkaline phosphatase: 197 U/L, γ-glutamyltransferase: 229 U/L) and increased inflammatory parameters (leukocytosis and CrP of 13.6 mg/L). Magnetic resonance cholangiopancreatography revealed unclear stenosis in the bifurcation of the main hepatic bile duct as well as in the prepapillary bile duct. Ultrasound (US) examination and endoscopic retrograde cholangiopancreatography showed dilatation of the intra -and extrahepatic bile ducts, which raised the suspicion of PSC. US image with dilated intra- and extrahepatic dilatation of the bile duct was also suggestive for autoimmune cholangitis. However, serum analysis revealed an elevated soluble interleukin-II receptor (1,305 U/mL), while immunoglobulin G4 was within normal ranges. Liver biopsy demonstrated hepatic inflammation and presence of granulomatous cells within the portal fields – convenient to sarcoidosis. After starting treatment with steroids, we observed a rapid clinical response with improvement of the dilated bile ducts and decrease of the initially elevated cholestatic liver enzymes. Sarcoidosis within the bile duct is a rare condition. Steroids are the treatment of choice and – along with the histology – are furthermore helpful to differentiate between several potential differential diagnoses like IgG4 cholangitis, primary biliary cholangitis, or PSC.

Download Full-text

The study of tissue IgG4 in the mucous membrane of the colon in patients with inflammatory bowel disease

GASTROENTEROLOGY ◽

10.22141/2308-2097.55.4.2021.247916 ◽

2021 ◽

Vol 55 (4) ◽

pp. 246-251

Author(s):

Yu.M. Stepanov ◽

T.S. Tarasova ◽

M.V. Stoikevych ◽

Yu.A. Gaydar ◽

N.S. Fedorova

Keyword(s):

Ulcerative Colitis ◽

Inflammatory Bowel Disease ◽

Crohn’S Disease ◽

Crohn's Disease ◽

Positive Result ◽

Mucous Membrane ◽

Bowel Disease ◽

Immunohistochemical Examination ◽

Immunoglobulin G4 ◽

Inflammatory Bowel

Background. Inflammatory bowel disease (IBD) is a global problem today, with a growing prevalence in the world. It significantly increases the economic burden on the health care system. Recently, many studies indicate the important role of immunoglobulin G4 (IgG4) in the formation of chronic inflammation in IBD and the possibility of using it as a biomarker of the inflammatory process. The purpose was to improve the diagnosis of chronic inflammatory bowel diseases by studying the status of IgG4-positive plasma cells in the mucous membrane of the colon in patients with ulcerative colitis (UC) and Crohn’s disease (CD). Materials and methods. We have examined 34 patients with IBD, 25 with UC and 9 with CD, of them 20 women and 14 men, with an average age of (38.8 ± 3.0) and (38.2 ± 3.7) years, respectively. Patients were divided into groups depending on the nosology and severity of the disease. All patients underwent endoscopic examination of the colon to establish or clarify the diagnosis, and biopsy specimens were taken for histological and immunohistochemical examination. Results. In 13 (38.3 %) of 34 examined patients, a positive result for the presence of tissue IgG4 (≥ 10 cells in the field of view) was found. Among patients with UC, 48 % have a positive result of immunohistochemical examination of tissue IgG4, in people with CD, this figure is 11.1 %. This gives us reason to say that in UC, elevation of tissue IgG4 levels occurs 4.4 times more often. Positive tissue IgG4 in patients with moderate UC was found 1.1 times more often than in severe UC. Among patients with mildly active disease, tissue IgG4 was not detected. Conclusions. In UC, IgG4-positive cells in the mucous layer of the colon are more common than in CD, which makes it possible to use this indicator for the differential diagnosis of ulcerative colitis and Crohn’s disease. Positive tissue IgG4 is more common in moderate form than in severe one.

Download Full-text