IgG4-Related Sclerosing Cholangitis: Rarely Diagnosed, but not a Rare Disease

Abstract

IgG4-related sclerosing cholangitis, a biliary manifestation of an IgG4-related disease, belongs to the spectrum of sclerosing cholangiopathies which result in biliary stenosis. It presents with signs of cholestasis and during differential diagnosis it should be distinguished from cholangiocarcinoma or from other forms of sclerosing cholangitis (primary and secondary sclerosing cholangitis). Despite increasing information and recently established diagnostic criteria, IgG4-related sclerosing cholangitis remains underdiagnosed in routine clinical practice. The diagnosis is based on a combination of the clinical picture, laboratory parameters, histological findings, and a cholangiogram. Increased serum IgG4 levels are nonspecific but are indeed a part of the diagnostic criteria proposed by the Japan Biliary Association and the HISORt criteria for IgG4-SC. High serum IgG4 retains clinical utility depending on the magnitude of elevation. Approximately 90% of patients have concomitant autoimmune pancreatitis, while 10% present with isolated biliary involvement only. About 26% of patients have other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, IgG4-related retroperitoneal fibrosis, or IgG4-related renal lesions. A full-blown histological finding characterized by IgG4-enriched lymphoplasmacytic infiltrates, obliterative phlebitis, and storiform fibrosis is difficult to capture in practice because of its subepithelial localization. However, the histological yield is increased by immunohistochemistry, with evidence of IgG4-positive plasma cells. Based on a cholangiogram, IgG-4 related sclerosing cholangitis is classified into four subtypes according to the localization of stenoses. The first-line treatment is corticosteroids. The aim of the initial treatment is to induce clinical and laboratory remission and cholangiogram normalization. Even though 30% of patients have a recurrent course, in the literature data, there is no consensus on chronic immunosuppressive maintenance therapy. The disease has a good prognosis when diagnosed early.

IgG4‐related sclerosing cholangitis, a mimicker of the cholangiocarcinoma: A case report

“…16 Either (H and Rt) or (S and I) are required for the definitive diagnosis. 17 In our case, serum IgG4 level was increased (S), CT scan showed the stricture in the distal CBD (I) and the patient responded to steroid therapy (Rt), thus confirming the diagnosis of IgG4-SC. The treatment of choice in IgG4-SC is a steroid, which has been proven to induce quicker and consistent remission.…”

Immune-Mediated Inner Ear Disease Associated with Type 1 Autoimmune Hepatitis: A Challenging Coexistence

“…IgG4-RD is a multi-organ immune-mediated disease that imitates various malignant, infectious, and inflammatory disorders. 29 , 30 Hepatic involvement 49 and particularly laboratory features of cholestasis 28 as well as SNHL could be indicative of IgG4-RD. Since pancreato-hepatobiliary disease is the potential clinical phenotype 30 for this patient, then morphological changes of affected organs would be expected to be found.…”

“…In particular, diffuse pancreas enlargement that usually encompasses more than two-thirds of the pancreas or a tumour-like mass are common imaging findings, 27 , 29 , 30 as type 1 autoimmune pancreatitis is associated with IgG4-related sclerosing cholangitis in almost 90% of cases. 28 In addition, it would be anticipated stenosis of the bile duct since the type of biliary involvement that is highly consistent with IgG4-related sclerosing cholangitis affects the proximal biliary tract. 28 The usual clinical presentation of autoimmune pancreatitis is obstructive jaundice induced by concomitant IgG4-related sclerosing cholangitis, secondary diabetes mellitus that occurs in about half of cases, and malabsorption due to exocrine insufficiency.…”

“… 28 In addition, it would be anticipated stenosis of the bile duct since the type of biliary involvement that is highly consistent with IgG4-related sclerosing cholangitis affects the proximal biliary tract. 28 The usual clinical presentation of autoimmune pancreatitis is obstructive jaundice induced by concomitant IgG4-related sclerosing cholangitis, secondary diabetes mellitus that occurs in about half of cases, and malabsorption due to exocrine insufficiency. 29 , 30 The patient did not have such manifestations whereas recurrent fever that was a dominant symptom of his initial presentation is uncommon in IgG4-RD.…”

“… 29 , 49 On the other hand, SNHL is a rare manifestation of IgG4-RD 29 where pachymeningitis, mass forming lesions or erosions of the temporal bone are frequently described 31 ; yet, such imaging findings were not found at the patient. The absence of other organ involvement, such as IgG4-related dacryoadenitis/sialadenitis, retroperitoneal fibrosis, or renal lesions that may coexist in about 26% of patients with IgG4-related sclerosing cholangitis 28 also makes the disease unlikely. Interestingly, in IgG4-RD, ESR can be elevated to a moderate degree whereas CRP is usually normal apart from certain clinical manifestations, namely retroperitoneal and aortic involvement, in which a slight increase can be observed.…”