Table 1: Patient’s demographic and clinical characteristics at the outbreak of the disease.

Age (mean ± SD) years 4 7 . 5 ± 1 4 . 4 (16–69) years.
Gender (male)8 (53%)
Family history of autoimmune diseases3 (20%)
Initial manifestations
 Weight loss13 (87%)
 Abdominal pain9 (60%)
 Bile duct stenosis/obstructive jaundice8 (53%)
 Palpebral or periorbital swelling2 (13%)
 Recurrent acute pancreatitis2 (13%)
 Salivary gland swelling/Xerostomy1 (7%)
Pancreatic focal swelling1 (7%)
Other autoimmune diseases2 (13%)*
Increased serum IgG4 (>135 mg/dL)2/10
Rheumatoid factor4/8
Positive antinuclear antibodies6/9
Diabetes mellitus3/15
Increased IgG5/10

*Sjögren’s syndrome and Graves’ disease.