FN: Autoimmune Hepatitis

Question 1

Pathophysiology

Answer 1

􏰀 Inflammatory disease of unknown cause characterised by Abs directed vs. hepatocyte surface antigens
􏰀 Predominantly young and middle-aged women 􏰀 Classified according to Abs
􏰁 T1: Adult, SMA+ (80%), ANA+ (10%), ↑IgG 􏰁 T2: Young, LKM+
􏰁 T3: Adult, SLA+

Question 2

Presentation in teens and early 20s

Answer 2

􏰁25%
 Constitutional: fatigue, fever, malaise 􏰁 Cushingoid: hirsute, acne, striae
􏰁 Hepatitis
􏰁 HSM
􏰁 Fever
􏰁 Amenorrhoea
􏰁 Polyarthritis
􏰁 Pulmonary infiltration 􏰁 Pleurisy

Question 3

Presentation Post/peri-menpausal

Answer 3

􏰁 Present insidiously ̄c chronic liver disease

Question 4

Associated Diseases

Answer 4

􏰀 Autoimmune thyroiditis
􏰀 DM
􏰀 Pernicious anaemia
􏰀 PSC
􏰀 UC
􏰀 GN
􏰀 AIHA (Coombs +ve)

Question 5

Ix

Answer 5

􏰀 ↑LFTs
􏰀 ↑IgG
􏰀 Auto Abs: SMA, LKM, SLA, ANA
􏰀 ↓WCC and ↓plats = hypersplenism
􏰀 Liver biopsy

Question 6

Mx

Answer 6

􏰀 Immunosuppression
􏰁 Prednisolone
􏰁 Azathioprine as steroid-sparer
􏰀 Liver transplant (disease may recur)

Question 7

Prognosis

Answer 7

􏰀 􏰀

Remission in 80% of patients 10yr survival 80%