Abstract | December 19, 2022

Acute Necrotizing Pancreatitis with Splanchnic Thrombosis and Walled-Off Necrosis: A Curious Presentation of Uncertain Etiology

Presenting Author: Khyati Somayaji, BS, Medical Student, 4th Year, Lewis Katz School of Medicine - Temple University, Philadelphia, PA

Coauthors: Katya Ahr, MD, Internal Medicine, PGY2, Temple University Hospital, Philadelphia, Pennsylvania Si Li, MD, Assistant Professor Clinical Medicine, Internal Medicine, Temple University Hospital, Philadelphia, Pennsylvania, Sara Peterson, MD, Associate Professor Clinical Medicine, Temple University Hospital, Philadelphia, Pennsylvania

Learning Objectives

  1. Discuss relatively uncommon etiologies of acute necrotizing pancreatitis in a young female patient
  2. Compare and contrast diagnostic methods for ruling out common etiologies of acute necrotizing pancreatitis and their relative sensitivities in the context of a more severe and remitting case of pancreatitis
  3. Clarify treatment guidelines for acute necrotizing pancreatitis and the complexities of genetic testing for young individuals when a significant genetic finding is present

Introduction: Necrotizing pancreatitis consists of 5-10% of total cases of acute pancreatitis. Complications include splanchnic thrombosis and walled-off necrosis (WON), however this case highlights the persistently unidentifiable etiology of acute necrotizing pancreatitis in a young female. Type 2 autoimmune pancreatitis (T2AIP) is a possible diagnosis, among others.

 

Case: A 21-year-old female with medical history of two episodes of idiopathic pancreatitis and gastritis presented with severe abdominal pain radiating to back, vomiting, and subjective fevers/chills beginning evening before arrival. Initial evaluation revealed abdominal tenderness, lipase 14,653, leukocytosis 32.5, and lactate 6.4. Abdominal computerized tomography showed decreased attenuation of pancreas with peripancreatic fat stranding and fluid concerning for necrotizing pancreatitis without WON. She received 5 days of meropenem for sepsis and hypoxic respiratory failure until infectious etiology was ruled out with negative procalcitonin. Repeat imaging revealed non-occlusive thrombus in superior mesenteric, portal, and splenic veins. Gallstones, pancreatic divisum, and biliary sludge were ruled out with MRI/MRCP. Patient drank alcohol rarely with last drink 3 weeks prior. No family history of cystic fibrosis or pancreatitis with triglycerides normal on admission. PAIgG, IgG1, IgG2, and IgG3 were low, IgG4 level was normal, and ANA was positive at 1:1280. Seven days later, she was readmitted with a 18x22x27cm multiloculated fluid collection treated with Axios stent and necrosectomy. Finally, a percutaneous drain was placed to remove septic retroperitoneal abscess.

 

Working Diagnosis: Two most common etiologies in young female patients, gallstone pancreatitis and heavy alcohol use, were ruled out. Differential diagnosis remained genetic, idiopathic, autoimmune, or microlithiasis-induced pancreatitis. Of these, type 2 autoimmune pancreatitis (T2AIP) fit the constellation of clinical and immunologic markers most succinctly. This patient’s acute onset of symptoms, age, and normal IgG4 level, and two previous episodes of pancreatitis kept autoimmune pancreatitis high on differential diagnoses list. Considering normal IgG4 level, young age, and lack of biliary/extra-pancreatic findings, T2AIP remains more likely than type 1 autoimmune pancreatitis (T1AIP).

 

Management: She continues outpatient follow-up with Gastroenterology. Genetic studies were negative for PRSS1 and SPINK, heterozygous for CFTR mutation more consistent with chronic rather than acute pancreatitis. Recent ultrasound indicated gallbladder sludge but T2AIP still cannot be excluded.

 

References:

  1. https://pubmed.ncbi.nlm.nih.gov/23100216/
  2. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3982646/
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