email   Email Us: phone   Call Us: +1 (914) 407-6109   57 West 57th Street, 3rd floor, New York - NY 10019, USA

Lupine Publishers Group

Lupine Publishers

  Submit Manuscript

ISSN: 2637-6628

Online Journal of Neurology and Brain Disorders

Research Article(ISSN: 2637-6628)

IgG4-Related Disease Misdiagnosed as Cholangiocarcinoma

Volume 1 - Issue 2

El Hadary HF1*, Dadour NM2, Ahmed H3 and Mo’nes D3

  • Author Information Open or Close
    • 1Consultant of Rheumatology & Immunology, Cairo University, Egypt
    • 2Specialist of Rheumatology & Rehabilitation, Egyptian Fellowship, Egypt
    • 3Resident of Rheumatology & Rehabilitation, El Katib Hospital, Egypt

    *Corresponding author: Hala El Hadary, Consultant of Rheumatology & Immunology, Kasr El-Ainy, School of Medicine, Cairo University, Egypt

Received: April 16, 2018;   Published: April 24, 2018

DOI: 10.32474/OJNBD.2018.01.000107

Full Text PDF

To view the Full Article   Peer-reviewed Article PDF


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.

Keywords: IgG4-Related disease; Cholangiocarcinoma; Neck swelling; Jaundice

Abbrevations: IgG4-RD: Immunoglobulin G4-Related Disease; AIP: Auto Immune Pancreatitis; MRCP: Magnetic Resonance Cholangio Pancreatography; CT: Computed Tomography; ERCP: Endoscopic Retrograde Cholangio Pancreatography; PSC: Primary Sclerosing Cholangitis

Abstract| Introduction| Case Report| Discussion| References|