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==Genetics==
==Genetics==
*[Disease name] is transmitted in [mode of genetic transmission] pattern.
*Primary biliary cirrhosis may be familial and is related to factors inherited maternally.<ref name="pmid7737573">{{cite journal| author=Brind AM, Bray GP, Portmann BC, Williams R| title=Prevalence and pattern of familial disease in primary biliary cirrhosis. | journal=Gut | year= 1995 | volume= 36 | issue= 4 | pages= 615-7 | pmid=7737573 | doi= | pmc=1382507 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7737573  }} </ref>
*Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
*Primary biliary cirrhosis tends to present at an earlier age in the second generation.
*The development of [disease name] is the result of multiple genetic mutations.
 
==Associated Conditions==
==Associated Conditions==
Conditions associated with primary biliary cirrhosis include:<ref name="pmid18215315">{{cite journal| author=Kumagi T, Heathcote EJ| title=Primary biliary cirrhosis. | journal=Orphanet J Rare Dis | year= 2008 | volume= 3 | issue=  | pages= 1 | pmid=18215315 | doi=10.1186/1750-1172-3-1 | pmc=2266722 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18215315  }} </ref><ref name="pmid15208427">{{cite journal| author=Watt FE, James OF, Jones DE| title=Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. | journal=QJM | year= 2004 | volume= 97 | issue= 7 | pages= 397-406 | pmid=15208427 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15208427  }} </ref><ref name="pmid2347546">{{cite journal| author=Tsianos EV, Hoofnagle JH, Fox PC, Alspaugh M, Jones EA, Schafer DF et al.| title=Sjögren's syndrome in patients with primary biliary cirrhosis. | journal=Hepatology | year= 1990 | volume= 11 | issue= 5 | pages= 730-4 | pmid=2347546 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2347546  }} </ref>
Conditions associated with primary biliary cirrhosis include:<ref name="pmid18215315">{{cite journal| author=Kumagi T, Heathcote EJ| title=Primary biliary cirrhosis. | journal=Orphanet J Rare Dis | year= 2008 | volume= 3 | issue=  | pages= 1 | pmid=18215315 | doi=10.1186/1750-1172-3-1 | pmc=2266722 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18215315  }} </ref><ref name="pmid15208427">{{cite journal| author=Watt FE, James OF, Jones DE| title=Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. | journal=QJM | year= 2004 | volume= 97 | issue= 7 | pages= 397-406 | pmid=15208427 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=15208427  }} </ref><ref name="pmid2347546">{{cite journal| author=Tsianos EV, Hoofnagle JH, Fox PC, Alspaugh M, Jones EA, Schafer DF et al.| title=Sjögren's syndrome in patients with primary biliary cirrhosis. | journal=Hepatology | year= 1990 | volume= 11 | issue= 5 | pages= 730-4 | pmid=2347546 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=2347546  }} </ref>

Revision as of 17:46, 6 February 2018


Primary Biliary Cirrhosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Overview

The exact pathogenesis of [disease name] is not fully understood.

OR

It is thought that [disease name] is the result of / is mediated by / is produced by / is caused by either [hypothesis 1], [hypothesis 2], or [hypothesis 3].

OR

[Pathogen name] is usually transmitted via the [transmission route] route to the human host.

OR

Following transmission/ingestion, the [pathogen] uses the [entry site] to invade the [cell name] cell.

OR


[Disease or malignancy name] arises from [cell name]s, which are [cell type] cells that are normally involved in [function of cells].

OR

The progression to [disease name] usually involves the [molecular pathway].

OR

The pathophysiology of [disease/malignancy] depends on the histological subtype.

Pathophysiology

Pathogenesis

  • The exact pathogenesis of primary biliary cirrhosis is not fully understood.
  • It is thought that primary biliary cirrhosis is the result of antimitochondrial antibodies (AMAs), directed to the E2 component of the pyruvate dehydrogenase complex (PDC-E2).[1]


Algorithm showing postulated hypothesis of pathogenesis of primary biliary cirrhosis[2][3][4][5]


 
 
 
 
 
 
 
 
 
 
 
Biliary epithelial cells (BEC)
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Overexpression of Bcl-2 in small apoptotic BEC
 
 
 
 
 
Cell lineage specific lack of glutathione
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Inhibition of PDC-E2 glutathiolation
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Prevents loss of immunogenicity after apoptpsis of BEC
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
PDC-E2 component remains intact and antigenic in apoptotic blebs of BEC
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Antigenic PDC-E2 are engulfed by intrahepaticc dendritic cells
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Dendritic cells transfer antigenic PDC-E2 to regional lymph nodes
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Antigenic PDC-E2 is recognized by MHC class I restricted CD8+ T cells
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Initiates autoimmunity
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Primary biliary cirrhosis
 
 
 
 
 
 
 
 
 
 

Genetics

  • Primary biliary cirrhosis may be familial and is related to factors inherited maternally.[6]
  • Primary biliary cirrhosis tends to present at an earlier age in the second generation.

Associated Conditions

Conditions associated with primary biliary cirrhosis include:[7][8][9]

Gross Pathology

  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of [disease name].

Microscopic Pathology

  • On microscopic histopathological analysis, asymmetric destruction of the intralobular bile ducts within portal triads is characteristic findings of primary biliary cirrhosis.

Histopathology

References

  1. ↑ Matsumura S, Van De Water J, Leung P, Odin JA, Yamamoto K, Gores GJ; et al. (2004). "Caspase induction by IgA antimitochondrial antibody: IgA-mediated biliary injury in primary biliary cirrhosis". Hepatology. 39 (5): 1415–22. doi:10.1002/hep.20175. PMID 15122771.
  2. ↑ Lleo A, Selmi C, Invernizzi P, Podda M, Coppel RL, Mackay IR; et al. (2009). "Apotopes and the biliary specificity of primary biliary cirrhosis". Hepatology. 49 (3): 871–9. doi:10.1002/hep.22736. PMC 2665925. PMID 19185000.
  3. ↑ Odin JA, Huebert RC, Casciola-Rosen L, LaRusso NF, Rosen A (2001). "Bcl-2-dependent oxidation of pyruvate dehydrogenase-E2, a primary biliary cirrhosis autoantigen, during apoptosis". J Clin Invest. 108 (2): 223–32. doi:10.1172/JCI10716. PMC 203018. PMID 11457875.
  4. ↑ Charlotte F, L'Herminé A, Martin N, Geleyn Y, Nollet M, Gaulard P; et al. (1994). "Immunohistochemical detection of bcl-2 protein in normal and pathological human liver". Am J Pathol. 144 (3): 460–5. PMC 1887102. PMID 8129031.
  5. ↑ Shimoda S, Harada K, Niiro H, Yoshizumi T, Soejima Y, Taketomi A; et al. (2008). "Biliary epithelial cells and primary biliary cirrhosis: the role of liver-infiltrating mononuclear cells". Hepatology. 47 (3): 958–65. doi:10.1002/hep.22102. PMID 18181218.
  6. ↑ Brind AM, Bray GP, Portmann BC, Williams R (1995). "Prevalence and pattern of familial disease in primary biliary cirrhosis". Gut. 36 (4): 615–7. PMC 1382507. PMID 7737573.
  7. ↑ Kumagi T, Heathcote EJ (2008). "Primary biliary cirrhosis". Orphanet J Rare Dis. 3: 1. doi:10.1186/1750-1172-3-1. PMC 2266722. PMID 18215315.
  8. ↑ Watt FE, James OF, Jones DE (2004). "Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study". QJM. 97 (7): 397–406. PMID 15208427.
  9. ↑ Tsianos EV, Hoofnagle JH, Fox PC, Alspaugh M, Jones EA, Schafer DF; et al. (1990). "Sjögren's syndrome in patients with primary biliary cirrhosis". Hepatology. 11 (5): 730–4. PMID 2347546.

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