Table 1.
Demographics, clinical presentation, workup and treatment of patients with autoimmune pancreatitis fulfilling the HISORt criteria seen at the University of Pittsburgh Medical Center from 1998 to 2007
| Patient number | Age at presentation (years)/gender | Presentation | Laboratory data | Serum lgG4 (mg/dl) | Imaging (CT, ERCP, EUS, MRI) | Histology | Associated findings/extra pancreatic manifestations | Response to treatment (initial treatment) | Follow-up (months) | Recurrence | HISORt group | Comments | ||||||
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| Lipase | ALP | Direct bilirubin | Site | Finding | Rx | Clinical | Lab. | Imaging | ||||||||||
| 1 | 23/male | Abdominal pain | 1,980(↑) | 88 | <0.1 | 8 | Pancreatic tail mass, pancreatic duct stricture | Pancreas | LPSP | — | Sx | Yes | Yes | Yes | 8 | No | A | — |
| 2 | 29/female | Abdominal pain, jaundice, weight loss, steatorrhea | 465(↑) | 60 | 0.2 | 64 | Prominent hypoechoic pancreatic tail pancreatic duct stricture | Pancreas | LPSP | Retroperitoneal fibrosis | Sx | Yes | Yes | Yes | 10 | NO | A | ANA=l:320(↑) |
| 3 | 41/male | Jaundice, weight loss | 520s(↑) | 320(↑) | 1.6 | ND | Pancreatic head mass, distal CBD stricture | Pancreas | LPSP | — | Sx | Yes | Yes | Yes | 1 | Lost to follow up | A | — |
| 4 | 68/female | Jaundice, weight loss | 550(↑) | 250(↑) | ND | ND | Pancreatic head prominence, distal CBD stricture | Pancreas | LPSP | New-onset diabetes mellitus | Sx | Yes | Yes | Yes | 36 | No | A | ANA= 1:160(↑), CA 19–9=3,422(↑), CEA=1.4 |
| 5 | 72/male | Jaundice, weight loss | NA | 128 | NA | ND | Pancreatic head mass, distal CBD stricture | Pancreas | LPSP | — | Sx | Yes | — | Yes | 6 | No | A | CA 19–9=34, CEA=0.8 |
| 6 | 75/male | Abdominal pain, back pain | 48 | 77 | <0.1 | ND | Pancreatic body mass | Pancreas | LPSP | — | Sx | Yes | Yes | Yes | 3 | No follow data | A | — |
| 7 | 86/male | Abdominal pain, jaundice, weight loss, pruritus | 14 | 332(↑) | 2.4(↑) | 79 | Pancreatic head mass, pancreatic duct stricture | Pancreas, submandibular gland | LPSP, CSS (specimens not available to review) | IHBS, CSS | P | Yes | Yes | Yes | 5 | Yes | A | ANA<1: 40, serum lgG4=368(↑) (on recurrence) |
| 8 | 50/female | Abdominal pain, anorexia, bloating | 809(↑) | NA | NA | 178(↑) | Pancreatic tail mass, pancreatic duct stricture | — | — | — | P | Yes | Yes | Yes | 13 | Yes | B | ANA= 1:160(↑) |
| 9 | 43/male | Jaundice, steatorrhea, pruritus | 1,432(↑) | 240(↑) | 1.5(↑) | 199(↑) | Diffuse pancreatic enlargement, distal CBD stricture | — | — | New-onset diabetes mellitus, submandibular gland enlargement | P | Yes | Yes | Yes | 9 | Yes | B | ANA <1:40, CA 19–9=53 |
| 10 | 61/female | Abdominal pain, jaundice, weight loss, polyuria | 651(↑) | 71 | <0.1 | 148(↑) | Pancreatic head mass, pancreatic duct stricture | — | — | New-onset diabetes mellitus, submandibular gland enlargement | P | Yes | Yes | Yes | 36 | Yes | B | ANA= 1:640(↑) |
| 11 | 64/male | Weight loss, steatorrhea | 13 | 13 | <0.1 | 785(↑) | Pancreatic head mass, pancreatic duct stricture | Ampullary biopsy | Inconclusive | — | P | Yes | Yes | Yes | 7 | Yes | B | ANA= 1:80(↑), CA 19–9=10.6, CEA<0.5 |
| 12 | 68/female | Jaundice | 550(↑) | 250(↑) | NA | 825(↑) | Distal CBD stricture, pancreatic duct stricture, hypoechoic pancreas | — | — | — | P | Yes | Yes | Yes | 2 | Lost to follow up | B | ANA <1:40 |
| 13 | 69/male | Abdominal pain | 158 | 92 | <0.1 | 735(↑) | Multifocal pancreatic head and body mass, pancreatic duct stricture | — | — | — | P | Yes | Yes | Yes | 2 | On steroids taper at the end of study period | B | ANA< 1:80, CA 19–9=25.4 |
| 14 | 72/male | Abdominal pain, jaundice, weight loss, steatorrhea, pruritus | NA | 425(↑) | 1.3(↑) | 452(↑) | Pancreas head mass, pancreatic duct stricture | Bile duct | Inadequate sample | Hilar stricture, liver mass | P | Yes | Yes | Yes | 5 | No | B | ANA= 1:160(↑), CEA=1.5, |
| 15 | 78/male | Abdominal pain | 396(↑) | 71 | <0.1 | 286(↑) | Pancreatic tail prominence, pancreatic duct stricturing | — | — | — | P | Yes | Yes | Incomplete response | 3 | Steroids tapered and on azathiprine at end of study period | B | — |
| 16 | 70/male | Abdominal pain, jaundice, weight loss, pruritus | 44 | 664(↑) | 1.5(↑) | 57 | Pancreatic head prominence, irregular pancreatic duct | Sub-mandibular gland | CSS | New-onset diabetes mellitus, hilar stricture, CSS | P | Yes | Yes | Yes | 5 | Yes | C | ANA< 1:40, CA 19–9=27 |
CBD, common bile duct; CSS, chronic sclerosing sialadenitis (IgG>10/hpf); CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; EUS, endoscopic ultrasound; IAC, immunoglobulin-4
(IgG4)-associated cholangitis; IHBS, intrahepatic biliary stricture; Lab., laboratory; LPSP, lymphoplasmacytic sclerosing pancreatitis (IgG>10 hpf); MRI, magnetic resonance imaging; NA, not available; ND, not done;
P, prednisone; PBDS, proximal common bile duct stricture; Rx, treatment; Sx, surgical resection.
All but 3 patients (1, 3, 10) were Caucasians. Abdominal pain was mild in all patients, not requiring narcotics. Normal laboratory values: lipase ≤ 200 IU/l, alkaline phosphatase (ALP) = 40–125 IU/l, direct bilirubin = 0.1–0.4 mg/dl, serum IgG4 ≤ 140 mg/dl, Carcinoembryonic antigen (CEA)≤5 ng/ml, Cancer associated antigen 19–9 (CA 19–9) ≤ 38 U/ml, Anit-nuclear antibody (ANA) ≤ 1:80.