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Table 1 The key epidemiological, clinical and pathological differences between type 1 and type 2 autoimmune pancreatitis

From: Abdominal manifestations of IgG4-related disease: a pictorial review

 

Type 1 AIP

Type 2 AIP

Gender

Male > female

Male = Female

Age

Older (6th decade)

Younger (4th decade)

Clinical Presentation

Painless obstructive jaundice

Painless obstructive jaundice, acute pancreatitis and abdominal pain

Histology

IgG4-rich periductal lymphoplasmocytic infiltrates

Granulocyte epithelial lesions (GEL)

Serum IgG4

Usually elevated

Normal

Extrapancreatic involvement

Salivary glands, biliary tree, kidneys, retroperitoneum

None

Treatment outcome

Excellent response to steroid, but recurrence is common

Excellent response to steroid, and recurrence is rare