Title: Only repeatedly elevated IgG4 levels in primary sclerosing cholangitis may distinguish
a particular patient phenotype
Version: 1 Date: 27 Apr 2024
Reviewer's report:
Kalthoff S et al analyzed the role of constantly elevated (#2 measurements during mean 3.5
years f/u) in a retrospective study consisting of 6 persons with constantly elevated IgG4 > 1/
UNL and 8 cases where the initially elevated IgG4 normalized during the f/u. In addition, they
had 96 PSC patients with constantly normal S-IgG4.
The authors found that age at PSC diagnosis, presence of IBD, high-grade biliary strictures,
fibrosis detected by TE, and cirrhosis as well as the Amsterdam Oxford, Mayo risk and MELD
scores did not differ between the PSC-N and PSC-IgG4.
The only difference they found was that P-ALP and GT were higher in 6 cases with constantly
elevated IgG4 compared to those 8 patients who normalized the value.
Major comments:
Patients and Methods:
1. The study population and the f/u time is very limited to draw any firm conclusions of the
impact of elevation of IgG4 on PSC disease outcome.
2. Slight elevation of IgG4 is common among PSC patients (15%). How was IAC excluded:
elevation of S-IgG4 >4x UNL, sIgG4/sIgG- ratio>0.24, papilla Vateri biopsy with IgG4
immunohistochemistry, findings?
3. Did the patients with elevation of IgG4 have any extrahepatic manifestations/symptoms
suggesting of IAC?
4. Did you analyze correlation between the elevation of IgG4 and ALP/gGT?
5. Was any of those 14 cases with elevated IgG4 treated with steroids/AZA for long term?
Title: Only repeatedly elevated IgG4 levels in primary sclerosing cholangitis may distinguish
a particular patient phenotype
Version: 1 Date: 27 Apr 2024
Reviewer's report:
Kalthoff S et al analyzed the role of constantly elevated (#2 measurements during mean 3.5
years f/u) in a retrospective study consisting of 6 persons with constantly elevated IgG4 > 1/
UNL and 8 cases where the initially elevated IgG4 normalized during the f/u. In addition, they
had 96 PSC patients with constantly normal S-IgG4.
The authors found that age at PSC diagnosis, presence of IBD, high-grade biliary strictures,
fibrosis detected by TE, and cirrhosis as well as the Amsterdam Oxford, Mayo risk and MELD
scores did not differ between the PSC-N and PSC-IgG4.
The only difference they found was that P-ALP and GT were higher in 6 cases with constantly
elevated IgG4 compared to those 8 patients who normalized the value.
Major comments:
Patients and Methods:
1. The study population and the f/u time is very limited to draw any firm conclusions of the
impact of elevation of IgG4 on PSC disease outcome.
2. Slight elevation of IgG4 is common among PSC patients (15%). How was IAC excluded:
elevation of S-IgG4 >4x UNL, sIgG4/sIgG- ratio>0.24, papilla Vateri biopsy with IgG4
immunohistochemistry, findings?
3. Did the patients with elevation of IgG4 have any extrahepatic manifestations/symptoms
suggesting of IAC?
4. Did you analyze correlation between the elevation of IgG4 and ALP/gGT?
5. Was any of those 14 cases with elevated IgG4 treated with steroids/AZA for long term?
Please review this paper.
user
Title: Only repeatedly elevated IgG4 levels in primary sclerosing cholangitis may distinguish
a particular patient phenotype
Version: 1 Date: 27 Apr 2024
Reviewer's report:
Kalthoff S et al analyzed the role of constantly elevated (#2 measurements during mean 3.5
years f/u) in a retrospective study consisting of 6 persons with constantly elevated IgG4 > 1/
UNL and 8 cases where the initially elevated IgG4 normalized during the f/u. In addition, they
had 96 PSC patients with constantly normal S-IgG4.
The authors found that age at PSC diagnosis, presence of IBD, high-grade biliary strictures,
fibrosis detected by TE, and cirrhosis as well as the Amsterdam Oxford, Mayo risk and MELD
scores did not differ between the PSC-N and PSC-IgG4.
The only difference they found was that P-ALP and GT were higher in 6 cases with constantly
elevated IgG4 compared to those 8 patients who normalized the value.
Major comments:
Patients and Methods:
1. The study population and the f/u time is very limited to draw any firm conclusions of the
impact of elevation of IgG4 on PSC disease outcome.
2. Slight elevation of IgG4 is common among PSC patients (15%). How was IAC excluded:
elevation of S-IgG4 >4x UNL, sIgG4/sIgG- ratio>0.24, papilla Vateri biopsy with IgG4
immunohistochemistry, findings?
3. Did the patients with elevation of IgG4 have any extrahepatic manifestations/symptoms
suggesting of IAC?
4. Did you analyze correlation between the elevation of IgG4 and ALP/gGT?
5. Was any of those 14 cases with elevated IgG4 treated with steroids/AZA for long term?
assistant