rheumatology-congress-physiology-2019-keynote

Page 11

December 9-10, 2019 | Barcelona, Spain

Journal of Orthopaedics Trauma Surgery

and Related Research

ARTHRITIS AND RHEUMATOLOGY

ANATOMY AND PHYSIOLOGY

International Conference on

Rheumatology Congress 2019 & Anatomy and Physiology 2019

December 09-10, 2019

Volume 14

J Orthop Trauma Surg Rel Res, ISSN: 1897-2276

IgG4-related disease misdiagnosed as cholangiocarcinoma

mmunoglobulin G4-related disease (IgG4-RD) is an immune-mediated fibro-inflammatory disease that consists of a collection

of disorders that share particular pathologic, serologic, and clinical features. These disorders were previously thought to

be unrelated. The most characteristic 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 and dense infiltration of IgG4-positive plasma cells with

extensive fibrosis in the bile duct wall. Circular and symmetrical thickening of the bile duct wall is observed in the areas without

stenosis that appear to be normal on cholangiography, as well as in the stenotic areas. 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 present with IgG4-SC. 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,

Biliarystrictures(secondarytosurgicaltrauma,chronicpancreatitis),Anastomoticstrictures

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. In this case, report a 57 years male patient presented

with jaundice, fatigue, weight loss, oral moniliasis and right sided neck swelling. He was

misdiagnosed as Cholangiocarcinoma.

Biography

Hala El-Hadary is a Rheumatology & Immunology consultant at faculty of medicine, Cairo University. She obtained her M.D. from faculty

of medicine, Cairo University in 2012. She is the head of Rheumatology department in Dar El- Fouad hospital and El-katib hospital. She

creates new pathways for improving healthcare. She has built this model after years of experience in research, evaluation, teaching and

administration both in hospital and education institutions.

Hala El-Hadary

Cairo University, Egypt