Primary objective: to investigate the incidence of IAC in patients resected for presumed PHC.Secundary objectives:- Evaluation of the diagnostic accuracy of histological criteria for IAC compared to IgG4 qPCR test- Evaluation of disease activity by…
ID
Source
Brief title
Condition
- Hepatic and hepatobiliary disorders
Synonym
Research involving
Sponsors and support
Intervention
Outcome measures
Primary outcome
1) Histological assessment of resection specimens by an experienced pathologist
according to diagnostic consensus criteria for IAC. The combination of >10
IgG4+ B cells/high-power field plus two out of the three following histological
criteria is considered highly suggestive of IAC:
- Lymphoplasmacytic infiltration
- Storiform (*cartwheel* or *mat-like*) fibrosis
- Obliterative phlebitis
2) Confirmation of diagnosis of IAC by laboratory assessment of IgG4 RNA and
protein levels.
Secondary outcome
1) Evaluation of diagnostic accuracy of the histological criteria for IAC
2) Evaluation of disease activity by laboratory assessment, including:
- Chemistry: ASAT, ALAT, alkaline phosphatase, gamma-GT, total bilirubin,
CA19-9, total IgG.
- Immunology: IgG subclasses.
3) Screening of serum for auto-antibodies
Background summary
Distinguishing perihilar cholangiocarcinoma (PHC; Klatskin tumor) from benign
forms of sclerosing cholangitis affecting the hilar bile ducts is challenging,
since histological confirmation is difficult to obtain by brush or biopsy and
accurate non-invasive diagnostic tests are lacking. Immunoglobulin
G4-associated cholangitis (IAC), an imitator of PHC, is a newly recognized
inflammatory disease that can present as sclerosing cholangitis with/without
(peri-)hilar tumor formation and is responsive to corticosteroid treatment.
Occasionally, histological signs of IAC are found in resection specimens of
patients that underwent surgery for presumed PHC.
Study objective
Primary objective: to investigate the incidence of IAC in patients resected for
presumed PHC.
Secundary objectives:
- Evaluation of the diagnostic accuracy of histological criteria for IAC
compared to IgG4 qPCR test
- Evaluation of disease activity by measurement of laboratory parameters
- Screening of serum for auto-antibodies
Study design
Observational cohort study.
Study burden and risks
Risks associated with participation are limited, since patients will be invited
to visit the outpatient clinic for a one-time blood draw only (approximately 30
mL). For patients who have experienced other IgG4-RD manifestations or
symptoms, re-evaluation of diagnosis may provide better insight in their
disease. Patients with active disease may benefit from prednisone treatment.
Meibergdreef 9
Amsterdam 1105AZ
NL
Meibergdreef 9
Amsterdam 1105AZ
NL
Listed location countries
Age
Inclusion criteria
- Patient underwent resection for presumed PHC at the AMC between 1984 and May 2016
- Histological assessment revealed a benign (sclerosing) tumor
- Patient is alive according to the municipal records database
Exclusion criteria
not applicable
Design
Recruitment
Followed up by the following (possibly more current) registration
No registrations found.
Other (possibly less up-to-date) registrations in this register
In other registers
| Register | ID |
|---|---|
| CCMO | NL58029.018.16 |
| OMON | NL-OMON20357 |