Autoimmune Pancreatitis (IgG4-Related)

Autoimmune involvement of the pancreas within the IgG4-related disease spectrum.

Written by: Saygı Hospital Health Guide Editorial Board

Published: February 1, 2026

This content is for general information; please consult your physician for diagnosis and treatment.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Dahiliye (İç Hastalıkları) department. Book Appointment →

What is Autoimmune Pancreatitis (IgG4-Related)?

Autoimmune pancreatitis (AIP) is classified into two types. Type 1 (lymphoplasmacytic sclerosing pancreatitis) is the pancreatic involvement of IgG4-related disease and is the most common form. Type 2 (idiopathic duct-centric pancreatitis) is a different entity associated with inflammatory bowel disease.

In Type 1 AIP, serum IgG4 level is elevated, the pancreas shows diffuse or focal enlargement (sausage shape), and other organs may also be involved (bile ducts, kidneys, retroperitoneum, salivary glands).

Differentiation from pancreatic cancer is very important. Dramatic response to steroid therapy supports the diagnosis and prevents unnecessary surgery.

Symptoms

Painless obstructive jaundice (most common presenting symptom)

Mild abdominal pain or discomfort

New-onset diabetes

Weight loss

Steatorrhea (fatty stools)

Risk Factors

Male sex (2:1 male predominance)

Older age (greater than 60 years — for Type 1)

History of involvement in other IgG4-related organs

Inflammatory bowel disease (for Type 2)

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

If painless jaundice develops (pancreatic cancer must be excluded)
If new-onset diabetes and jaundice occur together
If unexplained weight loss and steatorrhea are present
If multi-organ IgG4-related involvement is suspected

Treatment Methods

Prednisone 0.6-1 mg/kg/day (initial — 2-4 weeks, then gradual taper)

Maintenance: Low-dose prednisone (2.5-5 mg/day, up to 3 years — relapse prevention)

Rituximab (in steroid-refractory or relapse cases)

Biliary stent (temporary relief of obstructive jaundice)

Monitoring response to steroid therapy (IgG4 levels + imaging)

Which Department to Visit?

You can visit our Dahiliye (İç Hastalıkları) department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.