Secondary sclerosing cholangitis: mimics of primary sclerosing cholangitis

Abstract

Sclerosing cholangitis is a chronic cholestatic disease characterized by stricturing, beading, and obliterative fibrosis of the bile ducts. Sclerosing cholangitis is considered primary (PSC) if no underlying etiology is identified or secondary (SSC) if related to another identifiable cause. In this article, we will review the clinical features, pathogenesis, diagnosis, and imaging findings of PSC and SSC, with an emphasis on features that may aid in the distinction of these entities. We will also discuss various etiologies of SSC including recurrent pyogenic cholangitis, other infectious etiologies, ischemic damage, toxic insults, and immunologic, congenital, and miscellaneous causes, highlighting the unique imaging findings and clinical context of each diagnosis. Graphical abstract

Immune-Related Sclerosing Cholangitis and Subsequent Pyogenic Liver Abscesses in Two Patients With Melanoma Treated by Triplet Therapy: A Case Report

“…Moreover, pyogenic cholangitis may further aggravate sclerosing cholangitis. 9 This was observed in our cases, as biliary enzymes and strictures increased after the manifestation of the liver abscesses. Therefore, when biliary enzymes rebound during immunosuppressive therapy for IR-cholangitis, biliary infection needs to be excluded.…”

“…In the present cases, no preceding intervention was conducted, which implies that the bacterial cholangitis and liver abscesses occurred merely on the basis of IR-sclerosing cholangitis and subsequent CS therapy. Moreover, pyogenic cholangitis may further aggravate sclerosing cholangitis 9 . This was observed in our cases, as biliary enzymes and strictures increased after the manifestation of the liver abscesses.…”

Anti-integrin αvβ6 autoantibodies in patients with primary sclerosing cholangitis

“…The patients with PSC were diagnosed based on serum biochemistry, cholangiogram, histological findings, and association with IBD. We also excluded secondary sclerosing cholangitis according to the method described by Ludiwig et al [ 16 ]. The study did not include cases of small-duct PSC, and thus all the patients were categorized as large duct PSC, but included two cases of PSC-autoimmune hepatitis (AIH) overlap syndrome (PSC 41 and 46).…”

Imaging of Benign Biliary Tract Disease

“…Cholangiographic findings include segmental or diffuse thickening of the bile ducts with multifocal stricturing, which may be difficult to differentiate from PSC. 44 Involvement of the GB and cystic duct may be seen. 45 The disease responds well to steroid therapy.…”