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BACKGROUND: Cholangitis is a fibroinflammatory disease, almost always due to obstructive jaundice, which is difficult to differentiate from primary sclerosing cholangitis by imaging; its diagnosis is based on elevated serum IgG4 concentrations and histopathological data. OBJECTIVE: To describe the concept and current classification of SC-IgG4 according to different associations, as well as the pathogenic, epidemiological, clinical and therapeutic aspects. METHODOLOGY: Bibliographic search in the databases GoogleScholar, PubMed, Medline LILACS and Scopus, articles in English and Spanish were included. RESULTS: From the bibliographic review, 60 articles were obtained that met the inclusion criteria, of which 13 were discarded due to several reasons (letters to the editor, case reports and duplicate reviews). To date there are no unified criteria in relation to the diagnosis, classification and management of SC-IgG4. Similarities and differences in relation to the diagnostic criteria of SC-IgG4 between the Endoscopy Society of Japan and the Gastroenterology Societies of Europe and the United States were found. A significant proportion of SC-IgG4 cases present high levels of IgG4 in the blood and usually respond satisfactorily to corticosteroids. CONCLUSIONS: IgG4-related sclerosing cholangitis is a biliary manifestation of the disease related to IgG4, it is very difficult to diagnose and is often confused with cholangiocarcinoma and other types of cholangitis. IgG4-related sclerosing cholangitis has a very good prognosis and responds satisfactorily to corticosteroids. [ABSTRACT FROM AUTHOR]

Sjögren's syndrome is a systemic autoimmune disease characterized by dry eyes and mouth due to the involvement of exocrine glands. However, it can manifest with GI symptoms that cover a broad spectrum from esophageal and intestinal dysmotility, achalasia, hypochlorhydria, and chronic atrophic gastritis to pancreatic enzyme deficiency, biliary dysfunction, and liver cirrhosis, which varies in its clinical manifestations and is often associated with erroneous approaches. This article reviews the GI manifestations of Sjögren's syndrome. It presents the case of a woman in her eighth decade of life with this syndrome. She showed asymptomatic hepatobiliary disease, documented abnormalities in liver profile tests, and a subsequent diagnosis of primary sclerosing cholangitis, for which she received initial treatment with ursodeoxycholic acid. During her condition, the patient has had three episodes of cholangitis, requiring endoscopic retrograde cholangiopancreatography with no findings of stones, with scant biliary sludge and discharge of purulent bile precipitated by her underlying liver disease. The association between Sjögren's syndrome and primary sclerosing cholangitis is rare and calls for special consideration. [ABSTRACT FROM AUTHOR]

Crohn's disease (CD) is considered an immunologically mediated entity that involves the digestive tract. It is characterized by transmural inflammation and can affect any part of the digestive tract, from the oral cavity to the anus. Although it is recognized that its severity varies, extensive and multiple organ failure is unusual. We present the case of a young patient, who initially presented with pulmonary symptoms associated with CD. Years later, digestive and bile duct symptoms appeared. Treatment was based on anti-tumor necrosis factor-alpha antibody therapy, resulting in a satisfactory clinical response. The clinical relevance of this case is its full-blown presentation, which includes extensive gastrointestinal involvement and rare extraintestinal manifestations. [ABSTRACT FROM AUTHOR]