AIH guidelines Flashcards

1
Q

Definition of AIH

A

Characteristic histologic abnormalities (lymphoplasmacytic interface hepatitis),
-elevated AST, ALT
-elevated total IgG and the presence of one or more characteristic autoantibodies
- exclusion of viral, hereditary, cholestatic liver injury and DILI

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2
Q

Acute severe AIH definition

A

Jaundice, INR > 1.5 < 2, no encephalopathy; no previously recognized liver disease

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3
Q

ALF definition

A

INR ≥ 2; hepatic encephalopathy within 26 weeks of onset of illness; no previously recognized liver disease

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4
Q

AIH biochemical vs histological remission

A

biochem: Normalization of serum AST, ALT, and IgG* levels

histo: Absence of inflammation in liver tissue after treatment

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5
Q

AIH treatment failure vs incomplete response vs relapse

A

treatment failure: Worsening laboratory or histological findings despite compliance with standard therapy

incomplete response: Improvement of laboratory and histological findings that are insufficient to satisfy criteria for remission

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6
Q

AIH relapse

A

Exacerbation of disease activity after induction of remission and drug withdrawal (or nonadherence)

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7
Q

AIH treatment intolerance

A

Inability to continue maintenance therapy due to drug-related side effects

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8
Q

AIH epidemiology

A
  • female predominant
  • bimodal (age 10-30 & 40-60)
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9
Q

What is interface hepatitis?

A

Inflammation of periportal hepatocytes (as compared to lobular hepatitis)

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10
Q

Type 1 AIH vs Type 2 AIH

A

type 1:
+ ANA or ASMA/(anti-actin is subset of ASMA)

type 2:
+ anti-LKM 1
negative ANA/ASMA

20% of AIH are negative for all these auto-antibodies (sero-negative AIH). Can have +anti-SLA (very specific)

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11
Q

Atypical antibodies

A

anti-SLA - associated w/ severe disease and relapse. High specificity

p-ANCA - NOT specific. Also seen w/ PSC, AIH-PSC overlap, UC, minocycline DILI

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12
Q

AIH diagnostic algorithm

A

ANA, SMA + liver biopsy

Labs:
If negative, check SLA, pANCA, ttG, AMA
SLA + = AIH
pANCA+ = AIH or PSC
+ttG = celiac disease
+ AMA = PBC, overlap, AIH+AMA
all negative = antibody-negative PBC

Liver biopsy:
If interface hepatitis +- plasma cells = AIH
Bile duct injury or loss = overlap, PBC, or PSC
Fat = MASH or AIH/MASH

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13
Q

AIH histologic findings

A

interface hepatitis +/- lobular hepatitis, centrilobular necrosis

emperipolesis (penetration of one intact cell into another intact cell)

hepatocyte rosettes

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14
Q

Histologic features of AIH w/ ALF

A
  1. Central perivenulitis
  2. Plasma cell enriched inflammatory infiltrate
  3. Massive hepatic necrosis
  4. Lymphoid follicles
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15
Q

Most common presenting symptom of AIH?

A

Fatigue

Pruritus is NOT seen. Physical signs usually absent.

~1/4 - 1/3 of patients are asymptomatic

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16
Q

Imaging finding of acute severe hepatitis?

A

65% of patients have heterogenous hypoattenuated liver on CT non-con

17
Q

Concurrent immune diseases

A
  • more common in women
  • over 60: thryoid/RA
  • <30: IBD, AIHA

**all should be screened for thyroid and celiac. Consider workup for IBD, AIHA, DM, others.

18
Q

What are the Paris criteria?

A
  • identify patients w/ AIH-PBC overlap

Need to meet at least 2 of the following PBC criteria:
1) ALP >= 2x ULN or GGT >=5x ULN
2) presence of +AMA
3) florid bile duct lesions on histo

19
Q

Criteria for AIH in the setting of PBC:

A
  • interface hepatitis
  • ALT >= 5x ULN
  • IgG > 2x ULN or ASMA
20
Q

Criteria for AIH-PSC overlap

A
  • typical AIH features
  • lack of AMA
  • evidence of large duct PSC (ERCP/MRCP) or small duct PSC (histo w/ onion skinning)
21
Q

All patients with AIH and UC need to be evaluated for what?

A

large duct PSC

22
Q

Drugs associated with AIH-like injury?

A
  • minocycline
    -nitrofurantoin
  • infliximab

Other common ones: alpha-methyldopa, adallimumab, halothane,

23
Q

Characteristics of ALH DILI?

A
  • latency varies
  • mainly women
  • may have fever, rash, eosinophilia
  • concurrent AI diseases unusual
  • no HLA association
  • rarely causes cirrhosis
  • rarely relapses

biopsy features similar to AIH except cirrhosis is rare

24
Q

Treatment of ALH?

A
  • remove offending agent
    -consider steroids if:
    – meet’s Hy’s law (aminotransferase >3x ULN and TB >2x ULN)
    – fails to improve w/ drug withdrawl
    – worsening of symptoms/labs
25
Q

Use of non-invasive testing (ie fibroscan) in AIH?

A
  • not good for first 3 months since it is more of a marker of inflammation than fibrosis
  • improvements after 6 months tx are associated w/ biochemical remission, fibrosis regression, prognosis
26
Q

Pretreatment evaluation for AIH?

A
  • TPMT activity (if low, increased risk for severe bone marrow suppression)
  • check HBV serologies; consider monitoring vs ppx treatment
  • vaccines, esp hep A & B
  • vitamin D at dx and annually
  • DEXA and q2 years if risk factors for osteoporosis (steroid use, postmenopausal, hx fragility fx, age >65 if F, >70 if M)
  • evaluation for metabolic syndrome (obesity, impaired glucose tolerance, elevated TG, low HDL, HTN)
  • mental health assessment
27
Q

Bone ppx for all patients on steroids

A
  • calcium 1000-1200 mg/day
  • vit D (400-800 IU/day)
28
Q

Pregnancy & AIH

A
  • if poorly controlled, assoc w/ amenorrhea and decreased fertility
  • stillbirth/loss similar to those w/ chronic disease
  • 20% have premies (due to AIH flare). Flares are 3x more common post-partum
  • APLA are associated w/ AIH

Goal: biochemical remission for 1 year prior to conception; avoid flares

29
Q

Liver medications & pregnancy AE

A

terlipressin - uterine ischemia
octreotide, lactulose - none known
rifaximin - limited data
beta blockers - fetal brady, IUGR
steroids- ?cleft abnormalities
AZA - premies
MMF - birth defects, spontaneous abortion
tacrolimus - premie, transient neonatal AKI

AVOID: MMF
Continue: AZA and steroid maintenace

30
Q

Induction Treatment for AIH

A

pred + AZA

(prednisone 40-60 mg
OR prednisone 20-40 mg daily + AZA (50-150 mg)

+ antacid

31
Q

Response-guided therapy

A
  • once biochemical remission, taper to 20 mg with labs q 2 weeks
  • then gradual taper (2.5-5 mg q 2-4 weeks) to lower maintenance dose (5-10 mg daily)
32
Q

Alternatives to AZA?

A

MMF

(if low TPMT or concomitant malignancy, AZA is contraindicated; also avoid in decompensated cirrhosis)

33
Q

Contraindications to using budesonide instead of prednisone?

A

Cirrhosis - can lead to PVT

34
Q
A