Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis

IgG4-related SC, PSC/AIH and PSC alone

Min Lian, Bo Li, Xiao Xiao, Yue Yang, Pan Jiang, Li Yan, Chunyan Sun, Jun Zhang, Yiran Wei, Yanmei Li, Weihua Chen, Xiang Jiang, Qi Miao, Xiaoyu Chen, Dekai Qiu, Li Sheng, Jing Hua, Ruqi Tang, Qixia Wang, M. Eric Gershwin & 1 others Xiong Ma

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4 ≧ 1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease.

Original languageEnglish (US)
Pages (from-to)875-882
Number of pages8
JournalAutoimmunity Reviews
Volume16
Issue number8
DOIs
StatePublished - Aug 1 2017

Fingerprint

Insemination, Artificial, Homologous
Sclerosing Cholangitis
Natural History
Immunoglobulin G
Serum

Keywords

  • IgG4
  • IgG4-SC
  • Prognosis
  • PSC/AIH overlap

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology

Cite this

Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis : IgG4-related SC, PSC/AIH and PSC alone. / Lian, Min; Li, Bo; Xiao, Xiao; Yang, Yue; Jiang, Pan; Yan, Li; Sun, Chunyan; Zhang, Jun; Wei, Yiran; Li, Yanmei; Chen, Weihua; Jiang, Xiang; Miao, Qi; Chen, Xiaoyu; Qiu, Dekai; Sheng, Li; Hua, Jing; Tang, Ruqi; Wang, Qixia; Gershwin, M. Eric; Ma, Xiong.

In: Autoimmunity Reviews, Vol. 16, No. 8, 01.08.2017, p. 875-882.

Research output: Contribution to journalArticle

Lian, M, Li, B, Xiao, X, Yang, Y, Jiang, P, Yan, L, Sun, C, Zhang, J, Wei, Y, Li, Y, Chen, W, Jiang, X, Miao, Q, Chen, X, Qiu, D, Sheng, L, Hua, J, Tang, R, Wang, Q, Gershwin, ME & Ma, X 2017, 'Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone', Autoimmunity Reviews, vol. 16, no. 8, pp. 875-882. https://doi.org/10.1016/j.autrev.2017.05.018
Lian, Min ; Li, Bo ; Xiao, Xiao ; Yang, Yue ; Jiang, Pan ; Yan, Li ; Sun, Chunyan ; Zhang, Jun ; Wei, Yiran ; Li, Yanmei ; Chen, Weihua ; Jiang, Xiang ; Miao, Qi ; Chen, Xiaoyu ; Qiu, Dekai ; Sheng, Li ; Hua, Jing ; Tang, Ruqi ; Wang, Qixia ; Gershwin, M. Eric ; Ma, Xiong. / Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis : IgG4-related SC, PSC/AIH and PSC alone. In: Autoimmunity Reviews. 2017 ; Vol. 16, No. 8. pp. 875-882.
@article{c5874ac1084f4d758ca1ee7b0b623ad7,
title = "Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis: IgG4-related SC, PSC/AIH and PSC alone",
abstract = "There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65{\%} of IgG4-SC patients with an 86{\%} sensitivity and 98{\%} specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4 ≧ 1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease.",
keywords = "IgG4, IgG4-SC, Prognosis, PSC/AIH overlap",
author = "Min Lian and Bo Li and Xiao Xiao and Yue Yang and Pan Jiang and Li Yan and Chunyan Sun and Jun Zhang and Yiran Wei and Yanmei Li and Weihua Chen and Xiang Jiang and Qi Miao and Xiaoyu Chen and Dekai Qiu and Li Sheng and Jing Hua and Ruqi Tang and Qixia Wang and Gershwin, {M. Eric} and Xiong Ma",
year = "2017",
month = "8",
day = "1",
doi = "10.1016/j.autrev.2017.05.018",
language = "English (US)",
volume = "16",
pages = "875--882",
journal = "Autoimmunity Reviews",
issn = "1568-9972",
publisher = "Elsevier",
number = "8",

}

TY - JOUR

T1 - Comparative clinical characteristics and natural history of three variants of sclerosing cholangitis

T2 - IgG4-related SC, PSC/AIH and PSC alone

AU - Lian, Min

AU - Li, Bo

AU - Xiao, Xiao

AU - Yang, Yue

AU - Jiang, Pan

AU - Yan, Li

AU - Sun, Chunyan

AU - Zhang, Jun

AU - Wei, Yiran

AU - Li, Yanmei

AU - Chen, Weihua

AU - Jiang, Xiang

AU - Miao, Qi

AU - Chen, Xiaoyu

AU - Qiu, Dekai

AU - Sheng, Li

AU - Hua, Jing

AU - Tang, Ruqi

AU - Wang, Qixia

AU - Gershwin, M. Eric

AU - Ma, Xiong

PY - 2017/8/1

Y1 - 2017/8/1

N2 - There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4 ≧ 1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease.

AB - There is increased interest and recognition of the clinical variants of Sclerosing Cholangitis (SC) namely IgG4-SC, PSC/AIH overlap and PSC. For most Centers, the characteristic of IgG4-SC has not been thoroughly clinically compared with other sclerosing cholangitis variants. Further there are relatively few PSC/AIH overlap patients and the clinical outcome is not well characterized, especially for the PSC/AIH overlap syndrome. Our objective herein is to clarify the differences and similarities of the natural history of IgG4-SC, the PSC/AIH overlap and PSC alone. We also place in perspective the diagnostic value of serum IgG4 for IgG4-SC and investigate biomarkers for predicting the prognosis of sclerosing cholangitis. In this study, we took advantage of our large and well-defined patient cohort to perform a retrospective cohort study including 57 IgG4-SC, 36 PSC/AIH overlap patients, and 55 PSC patients. Firstly, as expected, we noted significant differences among immunoglobulin profiles and all patients exhibited similar cholestatic profiles at presentation. Cirrhotic events were found in 20 of total 57 IgG4-SC, 15 of 36 PSC/AIH overlap, and 18 of 55 PSC patients. Serum IgG4 was elevated in 92.65% of IgG4-SC patients with an 86% sensitivity and 98% specificity for diagnosis. IgG4-SC patients had a better treatment response at 6-month and 1-year than PSC/AIH patients, while the latter responded better with steroids than PSC patients. Importantly the adverse outcome-free survival of IgG4-SC patients was reduced, unlike earlier reports, and therefore similar to the PSC/AIH overlap syndrome. Serum IgG and total bilirubin were useful to predict long-term survival of IgG4-SC and PSC/AIH, respectively. In conclusion, serum IgG4 ≧ 1.25 ULN shows an excellent predictability to distinguish IgG4-SC among SC patients. IgG4-SC appears to be immune-mediated inflammatory process, while PSC/AIH overlap more tends to be cholestatic disease.

KW - IgG4

KW - IgG4-SC

KW - Prognosis

KW - PSC/AIH overlap

UR - http://www.scopus.com/inward/record.url?scp=85020094029&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85020094029&partnerID=8YFLogxK

U2 - 10.1016/j.autrev.2017.05.018

DO - 10.1016/j.autrev.2017.05.018

M3 - Article

VL - 16

SP - 875

EP - 882

JO - Autoimmunity Reviews

JF - Autoimmunity Reviews

SN - 1568-9972

IS - 8

ER -