Peribiliary Pseudotumor Like Presentation of IgG4 Related Disease
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SCIENTIFIC LETTER
Peribiliary Pseudotumor Like Presentation of IgG4 Related Disease Jagadeesh Menon 1 & Naresh Shanmugham 1 & Mukul Vij 2 & Sripriya Srinivas 3 & Srinivas Reddy 4 & Mohamed Rela 4,5 Received: 7 January 2020 / Accepted: 28 April 2020 # Dr. K C Chaudhuri Foundation 2020
To the Editor: IgG4 related disease (IgG4-RD) is being increasingly diagnosed in children. It causes protean multisystemic manifestations and unless meticulously looked for, can be missed or misdiagnosed. A 14-y-old boy with pain abdomen of two years duration had a firm splenomegaly on examination. His bilirubin was 0.5 mg/dl, aspartate aminotransferase (AST) 476 U/L (0–40 U/L), alanine aminotransferase 216 U/L (0–40 U/L) and gamma glutamyl transpeptidase 118 U/L (0–49 U/L). A sonography followed by MRI abdomen showed a soft tissue encasing the hilar bilar duct extending into the intrahepatic main hepatic ducts along the two branches of portal vein along with extrahepatic portal vein obstruction (Fig. 1a). Biopsy of the lesion showed intense inflammatory infiltrates with plenty of plasma cells and eosinophils along with obliterative phlebitis and storiform fibrosis (Fig. 1b). The number of IgG4 related plasma cells were 10/hpf. With a diagnosis of IgG4-RD presenting as peribiliary pseudotumor, child was started on oral steroids at 40 mg once daily dosing. The child became asymptomatic after 1 wk with normalisation of liver function tests (LFTs). After 6 mo, the repeat MRI showed significant resolution of the soft tissue lesion.
Azathioprine was added at 25 mg OD and the plan is to give it for long term maintenance as a steroid sparing agent. A diagnosis of IgG4-RD is usually made with typical clinical or radiological features along with classical histopathology, supported by raised IgG4 levels, with the latter being a non-specific marker [1]. Sclerosing cholangitis, pseudotumor and autoimmune hepatitis are the hepatic presentations [2]. Our patient had a pseudotumor like presentation of IgG4-RD which is peribiliary and periportal rather than isolated hepatic. This is previously undescribed in pediatric literature. The
* Naresh Shanmugham [email protected] 1
Department of Pediatric Gastroenterology & Hepatology, Dr Rela Institute & Medical Center, Bharat Institute of Higher Education and Research, Chennai, India
2
Department of Histopathology, Dr Rela Institute & Medical Center, Bharat Institute of Higher Education and Research, Chennai, India
3
Department of Radiology & Imaging Services, Dr Rela Institute & Medical Center, Bharat Institute of Higher Education and Research, Chennai, India
4
Department of Hepatobiliary Surgery & Liver Transplantation, Dr Rela Institute and Medical Center, Bharat Institute of Higher Education and Research, Chennai, India
5
Liver Transplant Unit, Kings College Hospital, London, UK
Fig. 1 a T2 weight MRI abdomen showing hyperintense periportal and peribiliary soft tissue density. b Trucut biopsy displaying fibrosis with focal storiform pattern along with plenty of plasma cells
Indian J Pe
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