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2014 ; 59
(5
): 1954-63
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Serum immunoglobulin G4 and immunoglobulin G1 for distinguishing immunoglobulin
G4-associated cholangitis from primary sclerosing cholangitis
#MMPMID24375491
Boonstra K
; Culver EL
; de Buy Wenniger LM
; van Heerde MJ
; van Erpecum KJ
; Poen AC
; van Nieuwkerk KM
; Spanier BW
; Witteman BJ
; Tuynman HA
; van Geloven N
; van Buuren H
; Chapman RW
; Barnes E
; Beuers U
; Ponsioen CY
Hepatology
2014[May]; 59
(5
): 1954-63
PMID24375491
show ga
The recent addition of immunoglobulin (Ig)G4-associated cholangitis (IAC), also
called IgG4-related sclerosing cholangitis (IRSC), to the spectrum of chronic
cholangiopathies has created the clinical need for reliable methods to
discriminate between IAC and the more common cholestatic entities, primary (PSC)
and secondary sclerosing cholangitis. The current American Association for the
Study of Liver Diseases practice guidelines for PSC advise on the measurement of
specific Ig (sIg)G4 in PSC patients, but interpretation of elevated sIgG4 levels
remains unclear. We aimed to provide an algorithm to distinguish IAC from PSC
using sIgG analyses. We measured total IgG and IgG subclasses in serum samples of
IAC (n = 73) and PSC (n = 310) patients, as well as in serum samples of disease
controls (primary biliary cirrhosis; n = 22). sIgG4 levels were elevated above
the upper limit of normal (ULN = >1.4 g/L) in 45 PSC patients (15%; 95%
confidence interval [CI]: 11-19). The highest specificity and positive predictive
value (PPV; 100%) for IAC were reached when applying the 4 × ULN (sIgG4?> 5.6
g/L) cutoff with a sensitivity of 42% (95% CI: 31-55). However, in patients with
a sIgG4 between 1 × and 2 × ULN (n = 38/45), the PPV of sIgG4 for IAC was only
28%. In this subgroup, the sIgG4/sIgG1 ratio cutoff of 0.24 yielded a sensitivity
of 80% (95% CI: 51-95), a specificity of 74% (95% CI: 57-86), a PPV of 55% (95%
CI: 33-75), and a negative predictive value of 90% (95% CI: 73-97). CONCLUSION:
Elevated sIgG4 (>1.4 g/L) occurred in 15% of patients with PSC. In patients with
a sIgG4 >1.4 and <2.8 g/L, incorporating the IgG4/IgG1 ratio with a cutoff at
0.24 in the diagnostic algorithm significantly improved PPV and specificity. We
propose a new diagnostic algorithm based on IgG4/IgG1 ratio that may be used in
clinical practice to distinguish PSC from IAC.