Immunoglobulin G4-related disease (IgG4-RD) is an increasingly
recognized immune-mediated condition comprised of
a collection of disorders that share particular pathologic, serologic,
and clinical features [1,2]. These disorders were previously
thought to be unrelated [3-5]. The commonly shared features include
tumor-like swelling of involved organs, a lymphoplasmacytic
infiltrate enriched in IgG4-positive plasma cells, and a variable degree
of fibrosis that has a characteristic “storiform” pattern. In
addition, elevated serum concentrations of IgG4 are found in 60 to 70
percent of patients with IgG4-RD.
IgG4-related sclerosing cholangitis (IgG4-SC) is a characteristic
type of sclerosing cholangitis, with an unknown pathogenic
mechanism. Patients with IgG4-SC display increased serum IgG4 levels [6]
and dense infiltration of IgG4-positive plasma cells with
extensive fibrosis in the bile duct wall [7]. Circular and symmetrical
thickening of the bile duct wall is observed in the areas without
stenosis that appear normal on cholangiography, as well as in the
stenotic areas [8]. IgG4-SC has been recently recognized as an IgG4-
related disease. IgG4-SC is frequently associated with autoimmune
pancreatitis (AIP). IgG4-related dacryoadenitis/sialadenitis and
IgG4-related retroperitoneal fibrosis are also occasionally observed in
IgG4-SC [9-12]. However, some IgG4-SC cases do not involve
other organs. IgG4-SC is most common in elderly men. Obstructive
jaundice is frequently observed in IgG4-SC.
A number of diseases, such as, Cystic fibrosis, Chronic obstructive
Choledocholithiasis, Biliary strictures (secondary to
surgical trauma, chronic pancreatitis), Anastomotic strictures in liver
graft, Neoplasms (benign, malignant, metastatic), Infections,
hypertonic saline instillation in the bile ducts, Post-traumatic
sclerosing cholangitis, Systemic vasculitis, Amyloidosis, Radiation
injury, Sarcoidosis, Systemic mastocytosis, Hypereosinophilic syndrome,
Hodgkin’s disease, may easily be confused with IgG4-
related sclerosing cholangitis, or coexist in a patient [13]. In this
case, report 57 years male patient presented with jaundice, fatigue,
weight loss, oral moniliasis and right sided neck swelling. He was
misdiagnosed as Cholangiocarcinoma.
