Case Report
Copyright ©2013 Baishideng Publishing Group Co., Limited. All rights reserved.
World J Hepatol. Jun 27, 2013; 5(6): 336-339
Published online Jun 27, 2013. doi: 10.4254/wjh.v5.i6.336
Progressive multi-organ expression of immunoglobulin G4-related disease: A case report
Marcello Maida, Fabio Salvatore Macaluso, Giuseppe Cabibbo, Giuseppe Lo Re, Nicola Alessi
Marcello Maida, Fabio Salvatore Macaluso, Giuseppe Cabibbo, Nicola Alessi, Section of Gastroenterology, DIBIMIS, University of Palermo, 90127 Palermo, Italy
Giuseppe Lo Re, Department of Radiology, DIBIMEF, University of Palermo, 90127 Palermo, Italy
Author contributions: Maida M, Macaluso FS and Alessi N contributed to the clinical management and writing of the case report; Cabibbo G and Lo Re G contributed to the diagnosis; all authors had full control over the preparation of the manuscript and approved the final draft of the manuscript.
Correspondence to: Giuseppe Cabibbo, MD, PhD, Section of Gastroenterology, DIBIMIS, P.zza delle Cliniche 2, 90127 Palermo, Italy.
Telephone: +39-91-6552274 Fax: +39-91-6552156
Received: August 1, 2012
Revised: September 30, 2012
Accepted: November 11, 2012
Published online: June 27, 2013
Processing time: 330 Days and 13.2 Hours

A 63-year-old Caucasian man presented with a cholestatic syndrome, renal failure and arthralgias. A laboratory examination revealed high immunoglobulin G (IgG) and IgG4 levels (5.95 g/L; normal range: 0.08-1.4 g/L), pointing to a diagnosis of systemic IgG4-related disease, with definite radiological evidence of biliary and pancreatic expression, and plausible renal, articular, salivary and lacrimal glands involvement. Due to the rarity of the condition, there are currently no random control trials to point to the optimal therapeutic approach. The patient has been on steroid therapy with the subsequent introduction of azathioprine, with a complete resolution of all symptoms, a rapid reduction to normalization of all blood tests, and a complete regression of the radiological picture. Our experience underlines the complexity of IgG4-related disease and its variable and sometimes progressive presentation, while pointing out the need for a careful and complete assessment for possible multi-organ involvement.

Keywords: Immunoglobulin G4 related-disease, Autoimmune cholangitis, Autoimmune pancreatitis, Renal failure