AI Article Synopsis
- IgG4-related disease is a newly recognized condition that can affect various organs and is associated with specific diseases, such as autoimmune pancreatitis and inflammatory aortic aneurysm.
- Key features of the disease include high serum IgG4 levels, significant IgG4 lymphocyte infiltration, and strong responses to steroids.
- Differentiating IgG4-related disease from primary sclerosing cholangitis and cholangiocarcinoma is crucial due to differing treatments and prognoses, though diagnosis is often difficult, as seen in a case of a 59-year-old man who had normal serum IgG4 levels but presented symptoms similar to unresectable gallbladder cancer.
Article Abstract
Immunoglobulin G4 (IgG4)-related disease is a novel disease entity that can involve diverse organs, causing specific diseases, including autoimmune pancreatitis, sclerosing cholangitis, cholecystitis, inflammatory aortic aneurysm, and inflammatory pseudotumor. IgG4-related disease is characterized by elevated serum IgG4 concentrations, abundant IgG4 lymphoplasmacytic infiltration, and dramatic steroid responses. It is clinically important to differentiate this rare disease from primary sclerosing cholangitis and cholangiocarcinoma, because the treatment and prognosis of these two diseases are completely different. However, the preoperative diagnosis is challenging, and the disease is frequently misdiagnosed. If the serum level of IgG4 is within the normal range, the diagnosis of IgG4-related disease is more difficult. This article reports on a 59-year-old man with IgG4-related disease mimicking unresectable gallbladder cancer with normal serum IgG4 concentrations.
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| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3782679 | PMC |
| http://dx.doi.org/10.5009/gnl.2013.7.5.616 | DOI Listing |
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