isolated proximal IgG4-SC
Conditions
Interventions
None listed
Sponsors
Eligibility
Inclusion criteria
Inclusion criteria: 1.Chalacteristic diffuse or segmental bile duct stricture with mucosal thickness 2.IgG4SC without autoimmune pancreatitis, nor atrophic pancreas 3.Presence of elavated serum IgG4 level over 135mg/dL 4.The presence with IgG4-related dacryoadenitis and/or IgG4-related retroperitoneal fibrosis 5.The histopathological findings; 1) marked Lymphoplasma cells infiltraion and fibrosis in the bile duct 2) IgG4-bearing plasmacells infiltration over 10cell/HPF 3) storiform fibrosis 4) obliterative phlebitis 6.steroid effectiveness Definite diagnosis; 1+2+3+4 1+2+3+6 or 5
Exclusion criteria
Exclusion criteria: IgG4-SC with autoimmune pancreatitis
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| diagnostic rate for isolated proximat IgG4-SC | — |
Secondary
| Measure | Time frame |
|---|---|
| 1.cholangiography (ERC or MRCP) 2.endosonographic and/or intraductal urtrasonographic features of the bile duct 3. findings of extra-bile duct lesions | — |
Countries
Japan
Contacts
Yokohama City University Hospital Endoscopic devision