Primary biliary cirrhosis pathophysiology

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).

Algorithm showing postulated hypothesis of pathogenesis of primary biliary cirrhosis

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 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

• [Disease name] is transmitted in [mode of genetic transmission] pattern.
• Genes involved in the pathogenesis of [disease name] include [gene1], [gene2], and [gene3].
• The development of [disease name] is the result of multiple genetic mutations.

Associated Conditions

Conditions associated with primary biliary cirrhosis include:

• Addison's disease
• Autoimmune thrombocytopenic purpura
• Celiac disease
• CREST syndrome
• Crohn's disease
• Dermatomyositis
• Diabetes mellitus type 1
• Gallstones
• Glomerulonephritis
• Grave's disease
• Hashimoto's thyroiditis
• Lichen planus
• Mixed connective tissue disorder
• Myasthenia gravis
• Pemphigoid
• Pernicious anemia
• Polymyositis
• Psoriasis
• Pulmonary fibrosis
• Raynaud's disease
• Rheumatoid arthritis
• Sarcoidosis
• Scleroderma
• Sjogren's syndrome
• Systemic lupus erythematosus
• Ulcerative colitis

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