An Unusual Cause of Upper Gastrointestinal Bleeding

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• Answer to the Clinical Challenges and Images in GI Question: Image 2 (page 211): Autoimmune Pancreatitis With a Focal Mass Lesion Causing Splenic Vein Thrombosis and Gastric Fundal Varices
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Question: A 45-year-old man presented to the hospital with coffee ground emesis, melena, and syncope. Initial evaluation revealed signs of hemodynamic instability with a heart rate of 120 beats/min and a blood pressure of 88/62 mmHg. Initial laboratory investigations were normal except for a decreased hemoglobin level of 7.8 g/dL (normal, 13.0–17.0). After resuscitation, an evaluation for suspected upper gastrointestinal bleeding using an esophagogastroduodenoscope was performed; a focal area of mucosal erythema was noticed in the fundus with enlarged mucosal folds, no blood or active bleeding was seen. Endoscopic ultrasonography (EUS) was performed and revealed multiple, well-circumscribed, anechoic, tubular structures, that demonstrated vascular flow on color Doppler (Figure A), the appearance was consistent with that of isolated gastric fundal varices. A hypoechoic, heterogeneous, poorly defined mass lesion arising from the tail of the pancreas, measuring 25 × 19 mm, was identified by EUS. Fine needle aspiration was performed using a 22-gauge needle (Figure B), but did not yield a diagnosis. Computed tomography was performed and demonstrated splenomegaly of 13 cm, chronic splenic vein thrombosis with increased gastrosplenic collaterals, and focal enlargement of the mid pancreatic body. The patient underwent splenectomy for long-term control of variceal bleeding and the pancreatic mass was resected (Figure C).

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Answer to the Clinical Challenges and Images in GI Question: Image 2 (page 211): Autoimmune Pancreatitis With a Focal Mass Lesion Causing Splenic Vein Thrombosis and Gastric Fundal Varices 

Histologic evaluation of the surgical specimen showed infiltration of pancreas and peripancreatic adipose tissue by lymphocytes and plasma cells, acinar fibrosis, lymphocytic infiltration of pancreatic ducts, and obliterating phlebitis (Figure C), compatible with autoimmune pancreatitis (AIP) type 1.¹ Serum immunoglobulin G4 level was found to be elevated to 156.5 mg/dL (reference range, 3.9–86.4).

AIP is a distinct form of chronic inflammatory and sclerosing disease of the pancreas with a presumed autoimmune etiology. A variety of pancreatic manifestations have been described in patients with AIP, including a pancreatic mass lesion. In a case series of 26 patients from a tertiary care center,² the median age at presentation was 62.5 years (range, 23–86). They were predominantly males (65%), and on imaging 85% were found to have a pancreatic mass, enlargement, or prominence. Accurate distinction is crucial between this form of AIP and pancreatic adenocarcinoma. Patients with AIP can develop a number of extra-pancreatic manifestations including biliary lesions, sialadenitis, retroperitoneal fibrosis, enlarged celiac and hilar lymph nodes, chronic thyroiditis, as well as interstitial nephritis. Gastric varices as a complication of AIP has been described in the literature³. An extensive review and guidelines were recently published by the working members of Research Committee for Intractable Pancreatic Disease and Japan Pancreas Society.

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References 

1. Chari ST , Kloeppel G , Zhang L , et al.  Histopathologic and clinical subtypes of autoimmune pancreatitis: the Honolulu consensus document . Pancreas . 2010;39:549–554
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2. Raina A , Yadav D , Krasinskas AM , et al.  Evaluation and management of autoimmune pancreatitis: experience at a large US center . Am J Gastroenterol . 2009;104:2295–2306
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3. Fuke H , Shimizu A , Shiraki K . Gastric varix associated with autoimmune pancreatitis . Clin Gastroenterol Hepatol . 2006;4: xxxii
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