PBC\ PSC\ Ascites Flashcards
What is the most common auto-immune disease associated with PBC?
Sjogren
PBC causes intra or extra hepatic obstruction?
Intra-hepatic
Which antibody positive in PBC?
AMA
Survival rate in PBC?
Asx patient: 16 years.
Symptomatic patient: 7 years.
Early symptoms of PBC?
Majority of patients are asymptomatic. Itching can appear as early symptom
Signs and symptoms of PBC?
1) High cholesterol level.
2) Keratoconjuctivitis.
3) Jaudice.
4) Arthopathy.
5) Kerostomia (Hyperpigmentation)
Findings in Biopsy of PBC?
Flouride duct lesion.
Management of PBC?
Liver transplant
Medical options to treat PBC?
UDCA
- Decrease histological complications and increase survival.
- This will only happen in 25 % of patients.
How to monitor patients on medical management of PBC?
Biochemical response:
- Decrease ALT and AST
- Decrease in bilirubin.
- Decrease ALP < 1.67 ULN
How to diagnose PBC?
- Increase ALP 1.5 ULN
- AMA +
If AMA + No need for biopsy.
Note:
- Always do US or MRCP to rule out extrahepatic obstruction
What if AMA negative. And you still suscpect PBC. Next step?
- Do other PBC Specific Ab
1) Sp100 2) Gp210
What if other PBC-Specific antibodies are negative?
Liver biopsy.
PBC
Any other medical treatment? Other than UDCA
Fibrate can be used as off-label.
Cirrhosis
What are the two main aspects of ascites pathophysiology?
1) Portal HTN
2) Na And H2O retention
Cirrhosis
How portal hypertension causes ascites? (3)
1) Stellate cell necrosis. Lead to fibrosis.»_space; Vasoconstriction.
2) Production of NO lead of splanchnic vasodilation.»_space; Water retention.
Cirrhosis
How Na and H2O retention causes ascites? (2)
1) Water retention due to increase NO.
2) Decrease renal blood flow.
3) Activation of RAAS System.
Cirrhosis
How ascites develop in patient with cancer or infection?
Increase albumin pass into the periteneoum and leak of proteneousus material to the peritoneum
Cirrhosis
How ascites develop in patient with pancreatitis?
Pancreatic enzyme leak to peritoneum
Cirrhosis
What are the causes of Low SAAG < 1.1?
High albumin in ascetic fluid
Low Albumin level in the serum
- Nephrotic syndrome
- Protein losing enteropathy.
High Albumin in the ascitec fluid:
- Malignancy: Meig syndrome, perotoneal mets
- Shistocoma.
- Other infections.
- Inflammation: pancreatitis, serositis, bowel obstruction.
Cirrhosis
What are the causes of high SAAG > 1.1?
Divided to
1) Pre-sinosuidal:
- Portal.V thrombosis or splenic V thrombosis
- Shistosoma.
- Sarcoidosis.
2) Sinosuidal: Cirrhosis.
3) Post-sinosoidal:
- SOS, Budd-chiary syndrome and R. CHF
Cirrhosis
How to differentiate between high SAAG ascites causes
Protein level. If more than 2.5 = cardiac if less than 2.5 = liver
Cirrhosis
Management of ascites
1) Na restriction < 2 g\Day
2) Diuretics
Cirrhosis
Options of diuretics in ascites
Spironolactone and Furesamide in ratio of 5 to 2.
Cirrhosis
Goal of diuresis per day
1 Liter per day
Urine Na\K Ratio > 1
Cirrhosis
Which medications you can stop to ensure good diuresis
NSAID’s and ACE
Cirrhosis
What is the maximum dose of diuretics?
Spironolactone 400 mg per day
Cirrhosis
Alternative for spironolactone?
Amiloride 5 to 40 mg\D
Cirrhosis
What is the definition of refractory ascites?
No adequate diuresis with max dose of diuretics or intolerable SE of diuretics.
Cirrhosis
What option to go for in refractory ascites?
1) Paracetesis. If failed.
2) LVP. If failed.
3) TIPS. If failed
4) End-willing catheter (palliative option)
In LVP start 25 % Albumin 6 to 8 g\L for each liter above 5 liter.
Cirrhosis
Two conditions to avoid LVP?
- SBP
- AKI
Cirrhosis
What if LVP failed? next step?
TIPS
Cirrhosis
Mention 4 CI for TIPS?
1) HE Grade II
2) Heart failure
3) P. HTN
4) Biliary disease.
5) Infections.
Cirrhosis
4 COMPLICATIONS OF TIPS
- Bleeding.
- Thrombosis.
- Infection.
- Increase risk of HE by 20 to 30 %
Cirrhosis
Palliative option for refractory ascites?
Ind-welling catheter
High risk of complications. Used as palliative option in refractory ascites
Cirrhosis
What is the Dx:
- Ascites + Benign Ovarian tumor + R. Sided pleural effusion
Meig syndrome