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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis
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Recognition of Epidermal Transglutaminase by IgA and Tissue Transglutaminase 2 Antibodies in a Rare Case of Rhesus Dermatitis

Published on: December 15, 2011

[A case requiring differentiation of IgG4-related sclerosing disease from periampullary cancer]

Naoki Aomatsu1, Yoshihiro Okita, Yasuyuki Kato

  • 1Dept. of Surgery, Osaka Medical Prison, Japan.

Gan to Kagaku Ryoho. Cancer & Chemotherapy
|December 27, 2012
PubMed

Insights

Immunoglobulin G4-related sclerosing disease (IgG4-RSD) can mimic pancreatic cancer. Prompt diagnosis and steroid treatment (prednisolone) led to significant symptom improvement and imaging changes in a patient presenting with abdominal pain and elevated biomarkers.

Area of Science:

  • Gastroenterology
  • Immunology
  • Radiology

Background:

  • Immunoglobulin G4-related sclerosing disease (IgG4-RSD) is a fibroinflammatory condition that can affect multiple organs, often mimicking malignancy.
  • Distinguishing IgG4-RSD from periampullary cancer is crucial for appropriate patient management and avoiding unnecessary surgical interventions.

Observation:

  • A 56-year-old male presented with right upper quadrant pain, liver dysfunction, and elevated serum HbA1C and CA19-9.
  • Imaging revealed a diffusely enlarged pancreas, pancreatic and bile duct stenosis, and retroperitoneal fibrosis, with elevated serum IgG and IgG4 levels.
  • Initial investigations, including brush cytology, were negative for malignancy.

Findings:

  • The patient's presentation and laboratory findings were consistent with IgG4-related sclerosing disease.
  • Treatment with oral prednisolone (30 mg/day) resulted in rapid symptom resolution, normalization of liver function tests, and significant reduction in pancreatic and bile duct dimensions.
  • Imaging demonstrated shrinkage of the enlarged pancreas and decreased bile duct wall thickness.

Implications:

  • IgG4-RSD should be strongly considered in the differential diagnosis of patients presenting with pancreatic and biliary abnormalities, especially when malignancy is suspected.
  • Early recognition and initiation of immunosuppressive therapy, such as corticosteroids, can lead to dramatic clinical and radiological improvement.
  • Accurate differentiation from periampullary cancer is essential to prevent potentially morbid surgical procedures and ensure timely, effective treatment.

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