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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
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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