Table 1 Clinical profile and comparison of IgG4-positive and IgG4-negative type 1 AIP.
From: Clinicopathological features of type 1 autoimmune pancreatitis without elevated serum IgG4 level
Characteristics | All patients | IgG4-positive | IgG4-negative | P-value |
|---|---|---|---|---|
n = 301 | n = 281 | n = 20 | ||
Age at diagnosis (years) | 69 (61–75) | 69 (62–74) | 67 (59–80) | 0.996 |
Sex, male/female | 249/52 | 232/49 | 17/3 | 1 |
Jaundice | 84 (27.9) | 83 (29.5) | 1 (5.0) | 0.018 |
Abdominal pain | 80 (26.6) | 73 (26.0) | 7 (35.0) | 0.432 |
Serological findings at diagnosis | ||||
Serum IgG level (mg/dL) | 1804 (1455–2281) | 1840 (1486–2294) | 1363 (1039–1623) | < 0.001 |
Serum IgG4 level (mg/dL) | 385 (210–675) | 407 (254–704) | 77 (36–110) | < 0.001 |
Antinuclear antibodies positive | 54/196 (27.6) | 47/181 (26.0) | 7/15 (46.7) | 0.128 |
Rheumatoid factor positive | 18/57 (31.6) | 14/43 (32.6) | 4/14 (28.6) | 1 |
Enlargement of the pancreas | 0.062 | |||
Diffuse | 140 (46.5) | 135 (48.0) | 5 (25.0) | |
Segmental/focal | 161 (53.5) | 146 (52.0) | 15 (75.0) | |
Irregular narrowing of the MPD | 0.011 | |||
Diffuse/multiple | 118 (39.2) | 116 (41.3) | 2 (10.0) | |
Segmental/focal | 183 (60.8) | 165 (58.7) | 18 (90.0) | |
Extra-pancreatic lesion | 105 (34.9) | 94 (33.5) | 11 (55.0) | 0.086 |
Extra-pancreatic sclerosing cholangitis | 29 (9.6) | 25 (8.9) | 4 (20.0) | 0.113 |
Sclerosing dacryoadenitis/sialadenitis | 42 (14.0) | 42 (14.9) | 0 | 0.086 |
Retroperitoneal fibrosis | 42 (14.0) | 35 (12.5) | 7 (35.0) | 0.012 |
Renal lesion | 10 (3.3) | 10 (3.6) | 0 | 1 |
Glucocorticoid therapy | 260 (86.4) | 247 (87.9) | 13 (65.0) | 0.010 |
Follow-up period from diagnosis (months) | 58 (25–102) | 57 (24–100) | 81 (28–126) | 0.156 |
