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Difference between revisions of "Primary biliary cholangitis"
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Primary biliary cholangitis (view source)
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| Image = Primary_biliary_cirrhosis_intermed_mag.jpg | | Image = Primary_biliary_cirrhosis_intermed_mag.jpg | ||
| Width = | | Width = | ||
| Caption = Primary biliary | | Caption = Primary biliary cholangitis. [[H&E stain]]. | ||
| Synonyms = primary biliary cirrhosis (obsolete term) | |||
| Micro = "florid duct lesion": intraepithelial lymphocytes - in bile duct, bile duct epithelial cells with eosinophilic cytoplasm; plasma cells; +/-[[granulomas]] (close to bile duct); +/-"garland" cirrhosis -- has irregular border | | Micro = "florid duct lesion": intraepithelial lymphocytes - in bile duct, bile duct epithelial cells with eosinophilic cytoplasm; plasma cells; +/-[[granulomas]] (close to bile duct); +/-"garland" cirrhosis -- has irregular border | ||
| Subtypes = | | Subtypes = | ||
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| ClinDDx = | | ClinDDx = | ||
}} | }} | ||
'''Primary biliary | '''Primary biliary cholangitis''', abbreviated '''PBC''', is a rare [[medical liver disease]]. | ||
It was previously known as ''primary ciliary cirrhosis''.<ref name=pmid26416194>{{cite journal |authors=Beuers U, Gershwin ME, Gish RG, Invernizzi P, Jones DE, Lindor K, Ma X, Mackay IR, Parés A, Tanaka A, Vierling JM, Poupon R |title=Changing Nomenclature for PBC: From 'Cirrhosis' to 'Cholangitis' |journal=Am J Gastroenterol |volume=110 |issue=11 |pages=1536–8 |date=November 2015 |pmid=26416194 |pmc=4679751 |doi=10.1038/ajg.2015.312 |url=}}</ref> | |||
==General== | ==General== | ||
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www: | www: | ||
*[http://www.gidesigns.net/images/MC-copper-flower-garland-L.jpg Garland - wreath of flowers (gidesigns.net)]. | *[http://www.gidesigns.net/images/MC-copper-flower-garland-L.jpg Garland - wreath of flowers (gidesigns.net)]. | ||
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[[File:1 PBC 4 680x512px.tif|Inflamed triads [arrows] amid undisturbed hepatocytes (Row 1 Left 40X).]] | |||
[[File:2 PBC 4 680x512px.tif|Granuloma in portal triad (Row 1 Right 400X).]] | |||
<br> | |||
[[File:3 PBC 4 680x512px.tif|Florid duct lesion (Row 2 Left 400X).]] | |||
[[File:4 PBC 4 680x512px.tif|Triad missing interlobular bile duct (Row 2 Right 400X).]] | |||
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Primary biliary cirrhosis. Inflamed triads [arrows] amid undisturbed hepatocytes (Row 1 Left 40X). Granuloma in portal triad (Row 1 Right 400X). Florid duct lesion (Row 2 Left 400X). Triad missing interlobular bile duct (Row 2 Right 400X). | |||
{| | |||
[[File:1 PBC 3 680x512px.tif| Ill defined triads, inflamed lobule (40X).]] | |||
[[File:2 PBC 3 680x512px.tif| Triad without a bile duct. Macrophages & occasional lymphocytes without epithelioid cells needed for granuloma and space where bile duct likely once was [cyan arrowhead] (400X).]] | |||
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[[File:3 PBC 3 680x512px.tif| PAS without diastase shows triad lacking bile duct and piecemeal necrosis (200X).]] | |||
[[File:4 PBC 3 680x512px.tif| PAS with diastase shows bile duct/proliferating bile ductules with epithelial injury and hepatocytes with ballooning degeneration (400X).]] | |||
<br> | |||
[[File:5 PBC 3 680x512px.tif| Trichrome shows space of Disse collagenization and periportal fibrosis without definite bridging (100X)..]] | |||
[[File:6 PBC 3 680x512px.tif| Iron stain shows isolated focus of hepatocytes with cytoplasmic blue granules (200X)]] | |||
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Primary biliary cirrhosis. AMA positive. Viral serology/ANA negative. No definite granulomas. Ill defined triads, inflamed lobule (Row 1 Left 40X). Triad without a bile duct. Macrophages & occasional lymphocytes without epithelioid cells needed for granuloma and space where bile duct likely once was [cyan arrowhead] (Row 1 Right 400X). PAS without diastase shows triad lacking bile duct and piecemeal necrosis (Row 2 Left 200X). PAS with diastase shows bile duct/proliferating bile ductules with epithelial injury and hepatocytes with ballooning degeneration (Row 2 Right 400X). Trichrome shows space of Disse collagenization and periportal fibrosis without definite bridging (Row 3 Left 100X). Iron stain shows isolated focus of hepatocytes with cytoplasmic blue granules (Row 3 Right 200X). | |||
{| | |||
[[File:1 PBC 2 680x512px.tif|Two inflamed triads accompany acini with mild steatosis (20X).]] | |||
[[File:2 PBC 2 680x512px.tif|A triad bears a poorly formed granuloma [yellow arrowhead]. A bile duct is also seen [red arrowhead] (200X).]] | |||
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[[File:3 PBC 2 680x512px.tif|A triad lacks a bile duct (200X).]] | |||
[[File:4 PBC 2 680x512px.tif|Trichrome of the same triad shows portal fibrosis (200X).]] | |||
|} | |||
Primary biliary cirrhosis. Two inflamed triads accompany acini with mild steatosis (Row 1 Left 20X). A triad bears a poorly formed granuloma [yellow arrowhead]. A bile duct is also seen [red arrowhead] (Row 1 Right 200X). A triad lacks a bile duct (Row 2 Left 200X). Trichrome of the same triad shows portal fibrosis (Row 2 Right 200X). | |||
{| | |||
[[File:1 PBC - 1 - 40X 680x512px.tif|Acini & lobules distorted by inflammation]] | |||
[[File:2 PBC - 1 - 200X 680x512px.tif|Loose granuloma in triad without duct]] | |||
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[[File:3 PBC - 1 - 200X 680x512px.tif|Well-formed granuloma]] | |||
[[File:4 PBC - 1 -200X 680x512px.tif|Piecemeal necrosis, PAS without diastase]] | |||
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[[File:5 PBC - 1 -400X 680x512px.tif|Damaged bile duct, PAS with diastase]] | |||
[[File:6 PBC - 1 -200X 680x512px.tif|Bridging fibrosis, trichrome]] | |||
|} | |||
Primary biliary cirrhosis with bridging fibrosis. Acini & lobules distorted by inflammation (Row 1 Left 40X) Loose granuloma in triad without duct (Row 1 Right 200X) Well-formed granuloma (Row 2 Left 200X) Piecemeal necrosis, PAS without diastase (Row 2 Right 200X) Damaged bile duct, PAS with diastase (Row 3 Left 400X) Bridging fibrosis, trichrome (Row 3 Right,200X) | |||
{| | |||
[[File:1 PBC 5 680x512px.tif|Stage 4 PBC.]] | |||
[[File:2 PBC 5 680x512px.tif| Stage 4 PBC.]] | |||
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[[File:3 PBC 5 680x512px.tif| Stage 4 PBC.]] | |||
[[File:4 PBC 5 680x512px.tif| Stage 4 PBC.]] | |||
<br> | |||
[[File:5 PBC 5 680x512px.tif| Stage 4 PBC.]] | |||
[[File:6 PBC 5 680x512px.tif| Stage 4 PBC.]] | |||
|} | |||
Primary biliary cirrhosis with Metavir stage 4 fibrosis (extensive fibrosis/cirrhosis). The patient was AMA positive and ANA negative. A. Inflamed tracts and bridges are intensely inflamed, with odd appearing edges of hepatocytes (arrows), suggestive of a jig saw pattern [40x]. B. Trichrome shows thin fibrous bands bounding regenerative isles (arrows) [Trichrome, 100X]. C. Reticulin shows two cell thick cords and cords lacking orientation, documenting regeneration; black lines about single cells document piecemeal necrosis [100X]. D. Lymphocytes, plasma cells, and macrophages, show piecemeal necrosis, surrounding hepatocytes (cyan arrows), as well as spreading into the lobule (green arrows), with aggregates reflecting spotty necrosis (black arrow). [400X]. E. PAS with diastase highlights a proliferated bile ductule (arrows) with epithelial damage [400X]. F. PAS with diastase shows an arteriole (green arrow) at great distance from an isolated bile ductule at the periphery of the triad (cyan arrow), consistent with loss of bile duct; neutrophils (black arrows) do not prove ascending cholangitis [400X]. | |||
===Staging PBC=== | ===Staging PBC=== | ||