Home > Gastroenterology > Autoimmune Pancreatitis

Diagnosis

Serum Immunoglobulin G4 (IgG4) - levels greater than 140 mg/DL has a sensitivity of 76% and specificity of 93%.

Endoscopic ultrasound with biopsy. Early imaging usually shows diffusely enlarged pancreas; this can appear like a tumor.

Treatment Regimens

Prednisone 30 to 40 mg PO qday for 4 to 8 weeks. Taper 5 mg per week.

Consider azathioprine 2-2.5 mg/kg/day or mycophenolate mofetil 750 mg twice daily if there is no response to prednisone (2).

Normalization of IgG4 could take several months. 30-40% have relapse, requiring repeat course and maintenance dose of 5 to 10 mg PO qday.

Consider H. pylori testing since the antibodies react to H. pylori.

IGG4 Systemic Disease

Pathology: lymphoplasmacytic infiltrates, IgG4-positive plasma cells and fibrosis (4).

Progression: Cirrhosis, portal hypertension, aortic aneurysm and dissection, salivary and lacrimal gland dysfunction, fibrosis of thyroid gland (2).

Please consider an official gastroenterology consult and refer to the sources below.

Sources:
1. Sleisenger and Fordtran's Gastrointestinal and Liver Disease
2. IGG4 Systemic Disease Treatment
3. A clinical overview of IgG4-related systemic disease

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