Table 2.
Summary of serology, imaging, and histopathologic features in children with AIP
| Literature (n=30 total) | INSPPIRE/CUSL (n=18 total) | |||
|---|---|---|---|---|
| Serology and biochemistry | No. of positive/tested | (%) | No. of positive/tested | (%) |
| Increased lipase (>1× ULN) | 5/14 | (36%) | 9/12 | (75%) |
| Increased amylase (>1× ULN) | 6/15 | (40%) | 4/8 | (50%) |
| Increased serum IgG4 (>1× ULN) | 6/24 | (25%) | 3/16 | (19%) |
| ANA | 2/14 | (14%) | 5/10a | (50%) |
| ANCA | 4/7 | (57%) | 1/7 | (14%) |
| Anti-LKM, RF, SSA, SSB | 0/6 | (0%) | 0/4 | (0%) |
| Anti-DNA, ASCA | 0/5 | (0%) | 1/4 | (25%) |
| Imaging findings (diagnosis) | No. of patients/reported | (%) | No. of patients/reported | (%) |
| Gland enlargement mass/focal | 16/29 | (55%) | 9/18 | (50%) |
| Gland enlargement global | 9/29 | (31%) | 5/18 | (28%) |
| Capsule-like rim (MRCP) | 3/14 | (21%) | 2/18 | (11%) |
| Pancreas atrophy, pseudocysts, calcification | 0/29 | (0%) | 0/18 | (0%) |
| Main pancreatic duct irregularity | 22/29 | (76%) | 8/18 | (44%) |
| CBD stricture/tapering | 17/29 | (59%) | 9/18 | (50%) |
| CBD dilatation | 16/29 | (55%) | 9/18 | (50%) |
| Retroperitoneal fibrosis | 1/29 | (3%) | ND | — |
| Pancreas histopathology | No. of patients/reported | (%) | No. of patients/reported | (%) |
| Performed | 22 | 9 | ||
| Noninterpretable | 3 | (14%) | 2 | (22%) |
| Pancreas fibrosis | 12/19 | (63%) | 7/7 | (100%) |
| Venulitis/phlebitis | 2/19 | (11%) | 1/7 | (14%) |
| Lymphoplasmacytic infiltration | 19/19 | (100%) | 5/7 | (71%) |
| IgG4 (+) plasmacytes (>10/HPF) | 1/19 | (5%) | 0/7 | (0%) |
| Granulocytic epithelial lesion | 14/19 | (74%) | 4/7 | (57%) |
| Acinar atrophy | 3/19 | (16%) | 4/7 | (57%) |
AIP, autoimmune pancreatitis; ANA, antinuclear antibody; ANCA, antineutrophil cytoplasmic antibody; ASCA, anti-Saccharomyces cerevisiae antibody; CBD, common bile duct; CUSL, Cliniques Universitaires St-Luc; DNA, anti-DNA antibody; HPF, high-power field; LKM, liver kidney microsome antibody; MRCP, magnetic resonance cholangiopancreatography; ND, no data; RF, rheumatoid factor; SSA, SS-A/Ro antibody; SSB, SS-B/La antibody; ULN, upper limit of normal.
a
Measured ANA titers ranged between 1/80 and 1/640.