Stratification
Departmentsin Japan
Selectionrate (%)
Departmentsnominated
Departmentsreplying
Replyrate (%)
Departmentsreplying
New cases(male/female)
Continuous cases(male/female)
Total(male/female)
Specialty hospital
80
100
80
58
72.5
58
121 (89/32)
255 (201/54)
376 (290/86)
University hospital
294
100
294
184
62.6
184
107 (84/23)
229 (186/43)
336 (270/66)
≥500 beds
679
100
679
241
35.5
241
94 (72/22)
97 (68/29)
191 (140/51)
400–499 beds
633
80
506
166
32.8
166
35 (29/6)
65 (36/29)
100 (65/35)
300–399 beds
1,156
40
462
142
30.7
142
21 (15/6)
22 (16/6)
43 (31/12)
200–299 beds
1,689
20
338
100
29.6
100
6 (4/2)
8 (6/2)
14 (10/4)
100–199 beds
3,903
10
390
128
32.8
128
5 (5/0)
0 (0/0)
5 (5/0)
≤99 beds
5,324
5
266
95
35.7
95
2 (2/0)
2 (1/1)
4 (3/1)
Total
13,758
–
3,015
1,114
36.9
1,114
391 (300/91)
678 (514/164)
1,069 (814/255)
In response to the questionnaire in the second-stage survey, we collected detailed clinicoepidemiological information on 546 patients (51.0 %; 418 males, 114 females, and 14 patients of unknown sex) who had been identified by the first questionnaire. One hundred seventy-two patients were newly diagnosed in 2007, and 374 patients had been already diagnosed before 2007. All patients were Japanese. The male to female ratio of the AIP patients was 3.7. The mean age was 63.0 ± 11.4 years (male/female = 63.6 ± 10.8/60.3 ± 12.4),and patients aged 60–69 years formed a peak in the age distribution curve (Fig. 2.1).
Diagnosis
Imaging findings of the reported patients are summarized in Table 2.2. Approximately 90 % of the cases had enlargement of the pancreas. Among them, nearly 30 % of the cases showed localized enlargement, which was not included in the 2002 diagnostic criteria [5]. MPD narrowing was seen in more than 90 % of the cases, but narrowing was within one-third of the entire length in approximately one-third of the cases. These cases, again, did not fulfill the JPS 2002 diagnostic criteria.
Table 2.2
AIP-related imaging findings
Imaging | Number of cases (%) |
|---|---|
Enlargement of the pancreas | Enlarged, 478 (87.5 %);not enlarged, 52 (9.5 %) |
Area of pancreatic enlargement | Diffuse, 322 (67.4 %);localized, 141 (29.5 %) |
Narrowing of the pancreatic duct | Narrowing, 468 (85.7 %);no narrowing, 40 (7.3 %) |
Length of the narrowing pancreatic duct | >1/3 of 307 (65.6 %); <1/3 of 151 (32.3 %) |
The serum IgG4 level was increased (≥135 mg/dl) in 87.6 % of the patients (Table 2.3). The IgG level was increased in 56.9 %, but the positive rates for γ-globulin, antinuclear antibody, rheumatoid factor, and eosinophilia were less than half.
Table 2.3
Serological findings in AIP patients
Serological items
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