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Hepatita autoimunStudeni UVVG Medicin an III

Mihai Adrian Panainte, grupa 7

Claudia di Benedetto, grupa 8

Raluca Marinela Rusu, grupa 2

Coordonator:

.L. Dr. Camelia Ciacli

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Descriere

Hepatit periportal cu infiltraii celule plasmatice,hipergammaglobulinemie, prezena auto-anticorpilor n

ser.

Boal cronic de ficat, criptogen (idiopatic)

Incidena 10-20/100000/an

Predilecie la femei tinere (70%)

3 tipuri

Statistic Dg. trziu (ciroz)

Fr tratament deces n ~10 ani, 5 ani forme severe

Bine tratat cu terapie imunosupresoare

Posibil overlap (+ ciroza biliara primitiv, colangita sclerozantprimitiv, hepatita cronica C, steatohepatita non-alcoolic)

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Fiziopatologie

Predispoziie genetic Factori declanatori:

Hepatitele virale (A, B, C)

Epstein-Barr Rubeol

Medicamente: (methyldopa, nitrofurantoin, minocycline)

DDx: boala Wilson, hepatite virale,medicamente hepatotoxice, consum exagerat

de etanol.

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Autoreactivarea LT

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Mecanismul HAI

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Clinic

Debut insidios, asimptomatic sau cu simptome nespecifice

(oboseal, anorexie, scdere ponderal, schimbri

comportamentale, amenoree). 25% din pacieni: epistaxis,

sngerri gingivale, echimoze la traume minore.

Prezentare:

Hepatit acut: febr, icter, ficat dur remisie, cr., insuficien

hepatic, ascit, encefalopatie.

Hepatit cronic: oboseal, discomfort abd. sup., prurit, anorexie,

mialgie, diaree, artralgie, edem, hirsutism, erupii cutanate, amenoree,

slabire, icter, hipotonie muscular, com.

Ciroz: varice hemoragice, ascit, edeme, prurit, angioame pianjen,

encefalopatie

Asociaie de boli autoimune: anemie hemolitic autoimun,

anemie pericinoas, tiroidit autoimun, artrit reumatoid,

sindromul Sjgren, scleroz sistemic, celiachie, glomerulonefrit,..

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Clinic

Semne comune: Hepatomegalie (83%)

Icter (69%)

Splenomegalie (32%)

Angioame-pianjen (58%)

Ascit (20%)

Encefalopatie (14%)

Complicaii:

Ciroz i complicaiile cirozei (ascit, coagulopatie, com hepatic) Hipertensiune portal

Varice esofagiene

Malnutriie (cu cretere deficitar la copii)

Chiar i fr ciroz

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Icter

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Icter

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Ascit

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Ascit

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Angioame pianjen

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Angioame pianjen

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Paraclinic

Analize hepatice uzualetransaminaze x 1.5 - 50

imunoglobulinele, IgG

Seropozitivitate: ANA, ASMA, LKM-1, SLA

Profil genetic HLA

OBS: Puncie hepatic obligatoriu aspect de hepatit

periportal

Test online:

http://napervillegi.com/contrivances/aihcalc.html

http://napervillegi.com/contrivances/aihcalc.htmlhttp://napervillegi.com/contrivances/aihcalc.html

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Ficat normal vs HAI

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HVB

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HVD

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Anticorpi

HAI-1 - ASMA (Antismooth muscle antibody) iANA (antinulear antiody)

HAI-2 - Anti

LKM-1 (Antiliver-kidneymicrosomal antibody)

HAI-3 - AntiSLA (Antibodies to soluble liver

antigen)

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ClasificareClinic Tipul 1 Tipul 2 Tipul 3

Anticorpi ASMA

ANA

Antiactin

Anti-LKM

P-450 IID6

Synthetic core

motif peptides

254-271

Soluble liver-kidneyantigen

Cytokeratins 8 and 18

Vrst 10 ani -

vrstnici

copiii (2-14 ani)

rar la aduli

Aduli (30-50 ani)

(%) 78 89 90

Alte boli autoimune (%) 41 34 58

Gamma globuline +++ + ++

IgA Nu Ocazional Nu

HLA asociat B8, DR3, DR4 B14, Dr3, C4AQO Uncertain

Rspuns la steroizi +++ ++ +++

Progresie la ciroz(%) 45 82 75

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Bibliografie

Medscape Reference

gastromed.ro

Wikipedia

Stritcht School of Medicine