PBC and PSC Flashcards

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1
Q

what is thee pathophysiology of PBC ?

A

autoimmune disease where the small intralobular bile ducts are affected , causes inflammation of these small bile ducts , followed by outflow obstruction causing cholestasis

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2
Q

what is the clinical piicture of PBC ?

A

middle aged female
jaundiced
complains of pale greasy stools
signs of liver cirrhosis
xanthoma or xanthelasma
fever and rigors do not occur

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3
Q

what are the other diseases associated with PBC ?

A

autoimmune hashimoto thyroiditis
celiac disease
sjogren syndrome
systeemic sclerosis
IBD

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4
Q

what are the autoantibodies associated with PBC ?

A

AMA positive - more specific to PBC
ANA positive and anti smooth muscle in some patients may be positive

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5
Q

what is thee serum testing result that indicates biliary obstruction ?

A

high ALP
high GGT
conjugated hyperbilirubinemia in the more advanced stages

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6
Q

what is the serum testing resultt for PBC ?

A

High ALP
High AMA

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7
Q

what are the different sources of raised ALP ?

A

rapid bone growth in children
pregnancy
pagets disease
vitamin d deficiency

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8
Q

how do we know if elevated ALP is due to liver affection ?

A

if the elevated ALP is accompanied by elevated GGT or high direct bilirubin

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9
Q

what is the first line investigation for the diagnosis of PBC ?

A

abdominal ultrasound in order to exclude causes of mechanical obstruction

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10
Q

wheen is liver biopsy indicated in PBC ?

A

if there is uncertainty in the diagnosis

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11
Q

what is the management for PBC ?

A

UDCA as the first line therapy ( for life)
Cholestyramine

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12
Q

what are some other issues associated with PBC ?

A

steatorrhea
osteoporosis
HCC

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13
Q

how should a patient with osteoporosis due to PBC be managed ?

A

regular DEXA scan at prresntation
vitamin DD supplementation
bisphophonates

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14
Q

what must be taken into consideration for patients taking bisphophonates and have PBC ?

A

must be carefully used for patients with varicies

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15
Q

when should liver transplant be taken into consideration in cases of PBC ?

A

with liver cirrhosis

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16
Q

what is the pathogenesis with PSC ?

A

progressive fibrosis and destruction of the intra and extrahepatic ducts of any and all sizes

17
Q

what are the diagnostic criteria for PSC ?

A

generalized beading and stenosis of the biliary system
abscence of cholledocholithiasis
exclusion of bile duct cancer

18
Q

what is the C/P of PSC ?

A

middle aged male
fatigue
intermittent jaundice
RUQ pain
Pruritus

19
Q

what other conditions is PSC associated with ?

A

Ulcerative colitis

20
Q

what is the most common cause of death is PSC ?

A

cholangiocarcinoma

21
Q

what is a cholestatic pattern in lab tests ?

A

disproportionate rise in ALP in comparison to ALT and AST

22
Q

what is the key investigation for PSC ?

A

MRCP

23
Q

what is the diagnostic finding on liver biopsy showing early features of PSC ?

A

periductal onion skin
later on becomes vanishing duct syndrome

24
Q

what is the trreatment for PSC ?

A

no definitive treatment
manage complications
cholestyramine is the first line treatment UDCA should not be used
ERCP biliary dilatation

25
Q

what are the antibodies found in PSC ?

A

p-ANCA
ANA
anticardiolipin antibodies