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  Table of Contents    
CASE REPORT  
Year : 2020  |  Volume : 63  |  Issue : 2  |  Page : 282-285
Does IgG4 level evaluation in pancreatic mass play role in avoiding major surgery in uncertain presentation: A case report


1 Department of Pathology, All India Institute of Medical Sciences, Raipur, Chhattisgarh, India
2 Department of Pathology, G B Pant Institute of Postgraduate Medical Education and Research, New Delhi, India
3 Department of Gastro-intestinal Surgery, G B Pant Institute of Postgraduate Medical Education and Research, New Delhi, India

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Date of Web Publication18-Apr-2020
 

   Abstract 


A 66-year-old male presented with chief complaints of anorexia associated with mild dull, intermittent epigastric pain for 6 months. The patient was a known diabetic on oral hypoglycemic and on routine checkup was found to have deranged liver function profile. On radiology, an ill-defined hypoechoic enhancing lesion involving head, neck, and uncinate process of pancreas was noted. Whipple's pancreaticodudenectomy was done and reported as IgG4-related autoimmune pancreatitis. Later, IgG (slightly) and IgG4 were found to be markedly raised. We report this case to highlight the importance of IgG4 evaluation prior to major surgery in uncertain pancreatic mass.

Keywords: Autoimmune, IgG4, pancreatic mass, pancreatitis

How to cite this article:
Gupta RK, Sakhuja P, Govind H, Agarwal AK. Does IgG4 level evaluation in pancreatic mass play role in avoiding major surgery in uncertain presentation: A case report. Indian J Pathol Microbiol 2020;63:282-5

How to cite this URL:
Gupta RK, Sakhuja P, Govind H, Agarwal AK. Does IgG4 level evaluation in pancreatic mass play role in avoiding major surgery in uncertain presentation: A case report. Indian J Pathol Microbiol [serial online] 2020 [cited 2020 Aug 10];63:282-5. Available from: http://www.ijpmonline.org/text.asp?2020/63/2/282/282690





   Introduction Top


Sarles et al. initially suggested that some pancreatitis are autoimmune in nature, due to the associated hypergammaglobulinemia.[1] However, autoimmune pancreatitis (AIP) as a distinct entity was first described by Yoshida et al. in 1995.[2] AIP is categorized into two types based on demographic and histopathological features. Type 1 is classified under IgG4-related diseases which typically show pancreatic parenchymal destruction by dense lymphoplasmacytic infiltration, marked fibrosis, and obliterative phlebitis. Since steroid shows a dramatic response in type 1 AIP, it should be considered as the first line of treatment instead of surgical intervention. We present a case of AIP type 1 with atypical radiological presentation but classical histological features.


   Case Report Top


A 66-year-old male presented with chief complaints of decreased appetite for 6 months associated with mild dull, non-radiating intermittent epigastric pain. The patient was a known diabetic and was taking oral hypoglycemic since 2 years. Except derangement of liver profile (total bilirubin 3.9 g/dL, serum glutamic oxaloacetic transaminase (SGOT) 100 U/L, serum glutamic pyruvic transaminase (SGPT) 160 U/L, alkaline phosphatase (ALP) 218 U/L), the remaining biochemical parameters including hemogram, kidney function test, and serum electrolytes were within normal limits. Ultrasound abdomen showed central intrahepatic biliary radical dilatation (IHBRD), dilation of common bile duct (CBD) (9.5 mm), and gall bladder (GB) sludge.

Contrast-enhanced computed tomography (CT) showed an ill-defined hypoechoic enhancing lesion measuring 29 × 35 × 47 mm involving head, neck, and uncinate process of pancreas. However, body and tail portion were unremarkable. The lesion was encasing the distal CBD with proximal CBD dilatation (11 mm), mild bilateral IHBRD, and distended GB. The lesion was also abutting superior mesenteric vein for a circumference of 90° [Figure 1]a and [Figure 1]b.
Figure 1: (a and b) Contrast-enhanced computed tomography images showing an ill-defined hypoechoic enhancing lesion involving head, neck, and uncinate process of pancreas encasing distal CBD with proximal CBD dilatation and mild bilateral IHBRD. (c) Magnetic resonance cholangiopancreatography showing proximal and mid CBD dilatation with bilateral IHBRD, mildly prominent pancreatic duct in body and tail and bulky pancreatic head and uncinate process

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Magnetic resonance cholangiopancreatography (MRCP) showed proximal and mid CBD dilatation (10 mm) with bilateral IHBRD, mildly prominent pancreatic duct in body and tail (2 mm), and bulky pancreatic head and uncinate process [Figure 1]c.

Endoscopic ultrasound showed dilated CBD till lower end blocked by mass arising from head and uncinate process of the pancreas measuring 1.5 × 1 cm with coarse lobulations in pancreas. The pancreatic duct measured 3.5 mm in the body.

A staging laparoscopy followed by open Whipple's pancreaticoduodenectomy (WPD) was done. Introperatively, a firm 1 × 1 cm mass was noted in the head of pancreas with PD measuring 3 mm and multiple lymph nodes. Postoperatively, serum bilirubin progressively increased in the first week which later subsided.


   Histopathology Top


Gross: A WPD specimen was received in our department, cut-section of which showed diffusely enlarged pancreas with peripheral capsule-like rim of fibrosis. No definite mass was seen.

Multiple sections examined from the pancreas showed diffuse infiltration by lymphoplasmacytic cells with dense storiform fibrosis, atrophic acini, focal antral metaplasia of the ducts, and obliterative phlebitis. The inflammation was seen to involve pancreatic ducts, nerve bundles, and also extending into the peripancreatic tissue. Focal intraductal secretions and medial calcification of blood vessel were also noted. The lymphocytes showed mainly CD3-positive T cells comprising admixture of mainly CD4 and focal CD8 T cells, while plasma cells delineated diffuse CD138 and IgG4 positivity (20–25 cells/hpf) [Figure 2]. Based on the characteristic histomorphology, final diagnosis of autoimmune pancreatitis (AIP) type 1 was made.
Figure 2: Histological and immunohistochemistry images showing (a) dense lymphoplasmacytic infiltrates involving nerve bundle and storiform fibrotic bands (HE ×100); (b) peripancreatic extension of inflammatory process in the soft tissue (HE × 100); (c) periductal inflammation with antral metaplasia (HE ×200), (d) obliterative phlebitis in vessel wall (HE ×100); (e and f) CD138 and IgG4 stains highlighting plasma cells, respectively (IHC ×400); (g and h) CD4 and CD8 stains showing admixture of T lymphocytes (IHC ×400); and (i) CD20 stain showing occasional B lymphocytes (IHC ×400)

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Postoperatively, after 10 days the serum IgG and IgG4 levels were measured and found to be raised (17.7 g/L, normal: 6.3–13.5 g/L) and (11.3 g/L, normal: 0.03–2 g/L), respectively. The repeat ultrasound did not reveal IHBRD. Currently, the patient is symptom-free and doing well at 7 months of follow-up.


   Discussion Top


Yoshida et al. first described AIP as distinct entity.[2] The International Consensus Diagnostic Criteria for AIP (ICDC) were proposed in 2011.[3] AIP is an autoimmune disease that results in destruction of the pancreatic parenchyma mediated by both humoral and cellular components. ICDC proposed two types of AIP: (1) lymphoplasmacytic sclerosing pancreatitis or type 1 and (2) idiopathic duct-centric pancreatitis or type 2.[3] The AIP type 1 occurs in Asian region in elderly men with elevated IgG4 and may show association with other autoimmune diseases. However, type 2 is more common in western young population with equal gender incidence showing granulocytic epithelial lesion, unaccompanied by IgG4 elevation and may be associated with inflammatory bowel disease. Jaundice, weight loss, and abdominal pain are the most common presentations, and diabetes is found in 60% of the patients. Radiologically, AIP type 1 usually shows a diffusely enlarged, sausage-shaped pancreas with homogeneous attenuation and moderate enhancement but may also present as a focal mass-forming lesion as in this case which should be differentiated from pancreatic malignancy.[4] In most of the cases, pancreatic duct is not dilated while 60% show thickening of the CBD wall.[5] In this case, pancreatic duct was unremarkable and CBD was mildly dilated. On CT scan, a capsule-like rim indicating fibrosis may be seen which is highly suspicious for the diagnosis of AIP type 1.[6] In our case, capsule-like rim was appreciated both in CT images and grossly. MRCP is not recommended for the evaluation of main pancreatic duct status in AIP. Different diagnostic criteria have been defined including various parameters. Clinical diagnostic criteria proposed by ICDC and HISORt criteria are the two commonly used methods for diagnosis and classification.[3],[7] Histologically, type 1 AIP is characterized by diffuse lymphoplasmacytic infiltrate, storiform fibrosis, and obliterative phlebitis with extension of inflammatory reaction into the periportal tissue. Raised serum IgG4 (>135mg/dL) is the most reliable diagnostic method with a sensitivity of 80% and specificity of 97%; however, this is not disease-specific.[8] In patients with early or limited IgG4-related diseases (IgG4-RD), a serum IgG4/IgG ratio >% serves as a better parameter.[9] It also serves as good prognostic marker which can be used to correlate with the treatment outcomes.[10] In tissue, IgG4/IgG plasma cell ratios of >% and IgG4-positive cells > per HPF are both sensitive and highly specific feature used for the diagnosis of IgG4-RD. However, Deshpande et al., in consensus statement of the pathology of IgG4-RD, suggested that serum IgG4+ plasma cell count should always be used in combination with the characteristic histological features for the diagnosis.[11] They have also proposed a terminology scheme for the diagnosis of IgG4-related disease based primarily on histological characteristics. Sometimes, the disease may resolve spontaneously; however, steroid therapy acts as the mainstay of treatment and is associated with an increased remission when compared with no treatment (98% vs. 75%, P < 0.001).[12] Treatment usually comprises 40 mg of prednisone/day for 4 weeks followed by tapering dose to 5 mg/week. Clinical relapse can occur in up to 30% of patients. Rituximab can also be used when patients are unable to take steroids for the induction and maintenance of remission.[13]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Sarles H, Sarles JC, Muratore R, Guien C. Chronic inflammatory sclerosis of the pancreas – An autonomous pancreatic disease? Am J Dig Dis 1961;6:688-98.  Back to cited text no. 1
    
2.
Yoshida K, Toki F, Takeuchi T, Watanabe S, Shiratori K, Hayashi N. Chronic pancreatitis caused by an autoimmune abnormality. Proposal of the concept of autoimmune pancreatitis. Dig Dis Sci 1995;40:1561-8.  Back to cited text no. 2
    
3.
Shimosegawa T, Chari ST, Frulloni L, Kamisawa T, Kawa S, Mino-Kenudson M, et al. International Consensus Diagnostic Criteria for Autoimmune Pancreatitis: Guidelines of the International Association of Pancreatology. Pancreas 2011;40:352-8.  Back to cited text no. 3
    
4.
Shimosegawa T. The amendment of the Clinical Diagnostic Criteria in Japan (JPS2011) in response to the proposal of the International Consensus of Diagnostic Criteria (ICDC) for autoimmune pancreatitis. Pancreas 2012;41:1341-2.  Back to cited text no. 4
    
5.
Kamisawa T, Egawa N, Nakajima H, Tsuruta K, Okamoto A, Kamata N, et al. Comparison of radiological and histological findings in autoimmune pancreatitis. Hepatogastroenterol 2006;53:953-6.  Back to cited text no. 5
    
6.
Sahani DV, Kalva SP, Farrell J, Maher MM, Saini S, Mueller PR, et al. Autoimmune pancreatitis: Imaging features. Radiology 2004;233:345-52.  Back to cited text no. 6
    
7.
Chari ST, Smyrk TC, Levy MJ, Topazian MD, Takahashi N, Zhang L, et al. Diagnosis of autoimmune pancreatitis: The Mayo Clinic experience. Clin Gastroenterol Hepatol 2006;4:1010-6.  Back to cited text no. 7
    
8.
Okazaki K, Kawa S, Kamisawa T, Ito T, Inui K, Irie H, et al. Working Committee of the Japan Pancreas Society and the Research Committee for Intractable Pancreatic Disease supported by the Ministry of Health, Labour and Welfare of Japan. Amendment of the Japanese Consensus Guidelines for Autoimmune Pancreatitis, 2013 I. Concept and diagnosis of autoimmune pancreatitis. J Gastroenterol 2014;49:567-88.  Back to cited text no. 8
    
9.
Masaki Y, Kurose N, Yamamoto M, Takahashi H, Saeki T, Azumi A, et al. Cutoff values of serum IgG4 and histopathological IgG4+plasma cells for diagnosis of patients with IgG4-related disease. Int J Rheumatol 2012;2012:580814.  Back to cited text no. 9
    
10.
Lian MJ, Liu S, Wu GY, Liu SY. Serum IgG4 and IgG for the diagnosis of autoimmune pancreatitis: A systematic review with meta-analysis. Clin Res Hepatol Gastroenterol 2016;40:99-109.  Back to cited text no. 10
    
11.
Deshpande V, Zen Y, Chan JK, Yi EE, Sato Y, Yoshino T, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25:1181-92.  Back to cited text no. 11
    
12.
Kamisawa T, Shimosegawa T, Okazaki K, Nishino T, Watanabe H, Kanno A, et al. Standard steroid treatment for autoimmune pancreatitis. Gut 2009;58:1504-7.  Back to cited text no. 12
    
13.
Khosroshahi A, Bloch DB, Deshpande V, Stone JH. Rituximab therapy leads to rapid decline of serum IgG4 levels and prompt clinical improvement in IgG4-related systemic disease. Arthritis Rheum 2010;62:1755-62.  Back to cited text no. 13
    

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Correspondence Address:
Rakesh Kumar Gupta
Department of Pathology, Third Floor, Administrative Block, All India Institute of Medical Sciences, Raipur - 492 099, Chhattisgarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_289_19

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