A195 TRIPLE OVERLAP SYNDROME? A RARE CASE OF AIH, PBC AND PSC OVERLAP
Notice bibliographique
Résumé
Autoimmune liver diseases share the principle of immune-mediated damage directed towards either hepatocytes or bile ducts. They are classified into Autoimmune Hepatitis and Primary Biliary Cholangitis (AIH-PBC), AIH and Primary Sclerosing Cholangitis (AIH-PSC) and AIH and undefined cholestatic syndrome. AIH-PBS and AIH-PSC are recognized in the literature. Few case reports presented PBC-PSC overlap. The occurrence of all three entities together is extremely rare. To describe a rare presentation of immune-mediated liver disease with features of AIH, PBC and PSC. Case report and literature review. A 61-year-old Caucasian lady was referred to our hepatology clinic with a diagnosis of PBC for 10 years and subtle features of AIH on liver biopsy. She was on ursodeoxycholic acid (UDCA) dose of 1.5 g/day which was reduced due to weight loss and anorexia. She had no features of cirrhosis. A year after, she experienced severe itching with an increase in her ALT to 150 U/L, AST to 134 U/L and AP to 367 U/L. Her total bilirubin (TBIL) was 12.3 umol/L and direct bilirubin (DBIL) was 6.9 umol/L. Her ANA and SMA levels were negative. Her AMA titre was > 1:320 with IgG level of 17.3, normal IgA and IgM. Her IgG subtypes, p-ANCA and c-ANCA levels were normal along with CA19-9. She, subsequently, underwent a liver biopsy which showed loose granulomas, Batts/Ludwig fibrosis grade of 2 /4 with activity of 1 /4, positive AMA, periportal lymphoplasmacytic infiltrates and patchy interface hepatitis. There was no definite ductopenia. Based on these findings, we increased her UDCA dose back and started her on budesonide 9 mg/day with azathioprine. Her itching along with ALT, AST and AP levels improved with this regimen at 3 months follow up. We then started weaning off her budesonide. Her pancreatic enzymes, TBIL and DBIL increased which led to stopping azathioprine and increasing her budesonide dose. Abdominal imaging showed normal pancreas, splenomegaly with heterogenous liver parenchyma and dilated intrahepatic bile ducts. Gastroscopy showed small esophageal varices and portal hypertensive gastropathy. She remained asymptomatic with no itching. Her TBIL and DBIL levels improved. Her ALT, AST and AP levels were 76, 96, 266 U/L, respectively. MRCP showed moderate intra and extra-hepatic biliary dilatations with multifocal strictures, and extrahepatic duct wall thickening and stenosis with stones and sludge can’t be ruled out. ERCP confirmed MRCP findings and intrahepatic PSC. She is currently maintained on budesonide and UDCA. Her MELD-Na score was 6 at her last clinic visit. To the best of our knowledge, this is only the third reported case presenting with overlapping features of AIH-PBC-PSC together. Treatment of similar rare presentations is usually challenging and individualized with variable responses given the lack of randomized controlled trials (RCTs). None
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Prédiction distillée sur la base complète
Imitation des enseignantsNi prévalence calibrée, ni vérité terrain. Validation humaine à venir. Apprise à partir de 10 348 étiquettes directes de Codex et de 10 348 étiquettes directes de Gemma. Le mode candidate est l'union des têtes enseignantes seuillées; le consensus est leur intersection. Ces sorties portent le statut machine_predicted_unvalidated et ne sont ni des étiquettes humaines ni des étiquettes directes de modèles de pointe.
Scores Codex et Gemma par catégorie
| Catégorie | Codex | Gemma |
|---|---|---|
| Métarecherche | 0,001 | 0,001 |
| Méta-épidémiologie (sens strict) | 0,000 | 0,000 |
| Méta-épidémiologie (sens large) | 0,001 | 0,000 |
| Bibliométrie | 0,000 | 0,000 |
| Études des sciences et des technologies | 0,000 | 0,000 |
| Communication savante | 0,000 | 0,000 |
| Science ouverte | 0,000 | 0,000 |
| Intégrité de la recherche | 0,000 | 0,000 |
| Charge utile insuffisante (le modèle a refusé de juger) | 0,000 | 0,000 |
Scores machine (provisoires)
Les deux têtes enseignantes du modèle étudiant, lues sur ce travail. Un score ordonne la base pour la relecture; il n'affirme jamais une catégorie, et le statut de validation accompagne chaque rangée tel quel.
Scores de référence d'un modèle non mature (critères de maturité non atteints, 7 itérations). Un score ordonne; il n'affirme jamais une catégorie.
score_only:v0-immature-baseline · tel quel depuis la passe de notation : score_only signifie que le nombre peut ordonner les travaux, et qu'aucune étiquette de catégorie n'en découleClassification
machine, non validéePrédiction automatique; un appel candidat d’une seule tête enseignante, pas un consensus.
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