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A case report of immunoglobulin G4-related sclerosing cholangitis with multiple relapse.

Abstract Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is classified as a biliary tract manifestation of immunoglobulin G4-related disease (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the optimal starting dose, adequate maintaining dose and withdrawal time remain disputable.
PMID
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Authors

Mayor MeshTerms
Keywords
Journal Title medicine
Publication Year Start




PMID- 29742724
OWN - NLM
STAT- MEDLINE
DCOM- 20180516
LR  - 20180516
IS  - 1536-5964 (Electronic)
IS  - 0025-7974 (Linking)
VI  - 97
IP  - 19
DP  - 2018 May
TI  - A case report of immunoglobulin G4-related sclerosing cholangitis with multiple
      relapse.
PG  - e0700
LID - 10.1097/MD.0000000000010700 [doi]
AB  - RATIONALE: Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is
      classified as a biliary tract manifestation of immunoglobulin G4-related disease 
      (IgG4-RD). Glucocorticoid is the first-line therapy for most patients, but the
      optimal starting dose, adequate maintaining dose and withdrawal time remain
      disputable. PATIENT CONCERNS: An elderly male patient presented to our hospital
      with neoplasms of the bile duct and pancreas at first visit in December 2011.
      Further examination revealed bile duct stenosis and obstruction, and elevated
      serum IgG4 level. DIAGNOSES: A diagnosis of IgG4-SC was established by
      examination results and effectiveness of steroid therapy, although IgG4-positive 
      plasma cells were seldom seen in the liver sample. INTERVENTIONS: Prednisolone
      was started from 40 mg daily, tapered gradually, and totally withdrawn after 22
      months of treatment. OUTCOMES: A new-onset cholangitis was detected 2 months
      later. Prednisolone 10 mg daily was administered again. Prednisolone was reduced 
      to 5 mg every other day without consultation with his doctor 1 year ago in May
      2017, then he presented to our hospital again with recurrent abdominal pain and
      jaundice. LESSONS: IgG4-SC is a protean condition and can be distinguished from
      primary sclerosing cholangitis, malignancy, and other inflammatory disorders
      based on 4 clinical criteria. Serum IgG4/IgG1 ratio is a practicable diagnostic
      algorithm to distinguish PSC from IgG4-SC. The dose and duration of
      glucocorticoid for treatment should be adjusted according to clinical situations,
      and proper maintaining dose is essential for a better prognosis.
FAU - Dong, Xiaoqin
AU  - Dong X
AD  - Department of Infectious Diseases, Center for Liver Disease.
FAU - Huo, Na
AU  - Huo N
AD  - Department of Infectious Diseases, Center for Liver Disease.
FAU - Wu, Zhao
AU  - Wu Z
AD  - Department of Infectious Diseases, Center for Liver Disease.
FAU - Wang, Guiqiang
AU  - Wang G
AD  - Department of Infectious Diseases, Center for Liver Disease.
FAU - Wang, He
AU  - Wang H
AD  - Department of Radiology, Peking University First Hospital, Beijing, China.
FAU - Zhao, Hong
AU  - Zhao H
AD  - Department of Infectious Diseases, Center for Liver Disease.
LA  - eng
PT  - Case Reports
PT  - Journal Article
PL  - United States
TA  - Medicine (Baltimore)
JT  - Medicine
JID - 2985248R
RN  - 0 (Anti-Inflammatory Agents)
RN  - 0 (Immunoglobulin G)
RN  - 9PHQ9Y1OLM (Prednisolone)
SB  - AIM
SB  - IM
MH  - Aged
MH  - Anti-Inflammatory Agents/*therapeutic use
MH  - Cholangitis, Sclerosing/*diagnosis/*drug therapy/immunology
MH  - Drug Administration Schedule
MH  - Humans
MH  - Immunoglobulin G/*blood
MH  - Male
MH  - Prednisolone/*therapeutic use
MH  - Recurrence
EDAT- 2018/05/10 06:00
MHDA- 2018/05/17 06:00
CRDT- 2018/05/10 06:00
PHST- 2018/05/10 06:00 [entrez]
PHST- 2018/05/10 06:00 [pubmed]
PHST- 2018/05/17 06:00 [medline]
AID - 10.1097/MD.0000000000010700 [doi]
AID - 00005792-201805110-00043 [pii]
PST - ppublish
SO  - Medicine (Baltimore). 2018 May;97(19):e0700. doi: 10.1097/MD.0000000000010700.