Variant syndromes of primary biliary cholangitis

Lisa Schulz; Marcial Sebode; Sören A Weidemann; Ansgar W Lohse

doi:10.1016/j.bpg.2018.06.003

Variant syndromes of primary biliary cholangitis

Standard

Variant syndromes of primary biliary cholangitis. / Schulz, Lisa; Sebode, Marcial ; Weidemann, Sören A ; Lohse, Ansgar W.

In: BEST PRACT RES CL GA, Vol. 34-35, 23.10.2018, p. 55-61.

Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review

Harvard

Schulz, L, Sebode, M , Weidemann, SA & Lohse, AW 2018, 'Variant syndromes of primary biliary cholangitis', BEST PRACT RES CL GA, vol. 34-35, pp. 55-61. https://doi.org/10.1016/j.bpg.2018.06.003

APA

Schulz, L., Sebode, M., Weidemann, S. A., & Lohse, A. W. (2018). Variant syndromes of primary biliary cholangitis. BEST PRACT RES CL GA, 34-35, 55-61. https://doi.org/10.1016/j.bpg.2018.06.003

Vancouver

Schulz L, Sebode M , Weidemann SA , Lohse AW. Variant syndromes of primary biliary cholangitis. BEST PRACT RES CL GA. 2018 Oct 23;34-35:55-61. https://doi.org/10.1016/j.bpg.2018.06.003

Bibtex

@article{0a0100357e22405bba70067d375cc2f2,

title = "Variant syndromes of primary biliary cholangitis",

abstract = "Patients with primary biliary cholangitis (PBC) can show biochemical, serological and/or histological features of autoimmune hepatitis (AIH). The term 'AIH-PBC overlap syndrome' has been used frequently for these cases and implies the coexistence of two separate diseases. However, the boundaries between 'classical' PBC, PBC with features of AIH and 'classical' AIH are difficult to define, and therefore the term 'variant syndrome' should be preferred. A variant syndrome must primarily be assumed in PBC patients showing pronounced hepatitic activity, either expressed by elevated transaminases and raised levels of serum IgG/gammaglobulins or more specifically by liver biopsy showing a modified hepatitis activity index (mHAI) score of >4/18. The presence of AIH-specific autoantibodies also supports the diagnosis of a variant syndrome. The diagnosis must not be missed because individually adapted immunosuppressive treatment, analogous to AIH therapy, appears to have an important beneficial impact on the prognosis and should therefore be offered to these patients.",

keywords = "Adult, Autoantibodies, Biopsy, Female, Hepatitis, Autoimmune, Humans, Immunosuppressive Agents, Liver Cirrhosis, Biliary, Male, Prognosis, Undifferentiated Connective Tissue Diseases, Journal Article, Review",

author = "Lisa Schulz and Marcial Sebode and Weidemann, {S{\"o}ren A} and Lohse, {Ansgar W}",

year = "2018",

month = oct,

day = "23",

doi = "10.1016/j.bpg.2018.06.003",

language = "English",

volume = "34-35",

pages = "55--61",

journal = "BEST PRACT RES CL GA",

issn = "1521-6918",

publisher = "Bailliere Tindall Ltd",

}

RIS

TY - JOUR

T1 - Variant syndromes of primary biliary cholangitis

AU - Schulz, Lisa

AU - Sebode, Marcial

AU - Weidemann, Sören A

AU - Lohse, Ansgar W

PY - 2018/10/23

Y1 - 2018/10/23

N2 - Patients with primary biliary cholangitis (PBC) can show biochemical, serological and/or histological features of autoimmune hepatitis (AIH). The term 'AIH-PBC overlap syndrome' has been used frequently for these cases and implies the coexistence of two separate diseases. However, the boundaries between 'classical' PBC, PBC with features of AIH and 'classical' AIH are difficult to define, and therefore the term 'variant syndrome' should be preferred. A variant syndrome must primarily be assumed in PBC patients showing pronounced hepatitic activity, either expressed by elevated transaminases and raised levels of serum IgG/gammaglobulins or more specifically by liver biopsy showing a modified hepatitis activity index (mHAI) score of >4/18. The presence of AIH-specific autoantibodies also supports the diagnosis of a variant syndrome. The diagnosis must not be missed because individually adapted immunosuppressive treatment, analogous to AIH therapy, appears to have an important beneficial impact on the prognosis and should therefore be offered to these patients.

AB - Patients with primary biliary cholangitis (PBC) can show biochemical, serological and/or histological features of autoimmune hepatitis (AIH). The term 'AIH-PBC overlap syndrome' has been used frequently for these cases and implies the coexistence of two separate diseases. However, the boundaries between 'classical' PBC, PBC with features of AIH and 'classical' AIH are difficult to define, and therefore the term 'variant syndrome' should be preferred. A variant syndrome must primarily be assumed in PBC patients showing pronounced hepatitic activity, either expressed by elevated transaminases and raised levels of serum IgG/gammaglobulins or more specifically by liver biopsy showing a modified hepatitis activity index (mHAI) score of >4/18. The presence of AIH-specific autoantibodies also supports the diagnosis of a variant syndrome. The diagnosis must not be missed because individually adapted immunosuppressive treatment, analogous to AIH therapy, appears to have an important beneficial impact on the prognosis and should therefore be offered to these patients.

KW - Adult

KW - Autoantibodies

KW - Biopsy

KW - Female

KW - Hepatitis, Autoimmune

KW - Humans

KW - Immunosuppressive Agents

KW - Liver Cirrhosis, Biliary

KW - Male

KW - Prognosis

KW - Undifferentiated Connective Tissue Diseases

KW - Journal Article

KW - Review

U2 - 10.1016/j.bpg.2018.06.003

DO - 10.1016/j.bpg.2018.06.003

M3 - SCORING: Journal article

C2 - 30343711

VL - 34-35

SP - 55

EP - 61

JO - BEST PRACT RES CL GA

JF - BEST PRACT RES CL GA

SN - 1521-6918

ER -