A294 CHOLESTASIS SECONDARY TO XANTHOGRANULOMATOUS CHOLANGITIS: A CASE REPORT
Notice bibliographique
Résumé
Abstract Background An 86-year-old female presented with four weeks of intermittent abdominal pain, postprandial nausea with an associated 10 lbs weight loss. Initial labs demonstrated a predominantly cholestatic liver enzyme elevation. Imaging revealed a thickened gallbladder with dilated CBD up to 15mm with no calculi, obstruction or pancreatic mass. EUS demonstrated a prominent ampulla with an area of hyperechogenicity, and ERCP was completed with sphincterotomy and biopsy of the ampulla. A cholangiogram showed a prominent common bile duct and a smooth distal taper without filling defects. Tumor markers, ampullary biopsy, and cytology of CBD brushings were negative for malignancy. Repeat ERCP for non-resolving symptoms with an extension of the sphincterotomy, brushings and insertion of a metal stent was completed without improvement in liver enzymes and a standard hepatitis workup returned negative. Her clinical course was further complicated by recurrent gram-negative bacteremia with CT findings of innumerable abscesses or metastasis in the liver. A US-guided targeted liver biopsy revealed large duct periductal tissue expansion by foamy macrophages with admixed neutrophils, eosinophils, plasma cells, and lymphocytes. These features were consistent with xanthogranulomatous cholangitis. We were planning to start ursodiol given the small duct disease however the patient succumbed to recurrent bacteremia and passed away. Aims Review of methods to attain diagnosis of xanthogranulomatous cholangitis. Methods Case Report. Results Diagnosis of xanthogranulomatous cholangitis can be attained from targetted US-guided biopsy, surgical excision, or EUS-guided CBD biopsy. Conclusions Xanthogranulomatous cholangitis is an extremely rare entity described in limited case reports, frequently mimicking neoplastic disease. Pathology is difficult to attain and historically case reports required surgical resections. One recent case reported diagnosis confirmed via EUS-guided FNA of the CBD. In our case, we attained the diagnosis through a targeted liver biopsy. Pathology of xanthogranulomatous cholangitis typically demonstrates foamy histiocytes, lipid-laden macrophages, eosinophils, lymphocytes, plasma cells, and fibrosis indicating xathogranulomas. The pathogenesis is thought to be an extension of xanthogranulomatous cholecystitis in which bile is extravasated into the gallbladder wall through Rokitansky-Aschoff sinuses or ulceration of the mucosa resulting in an inflammation caused by fibroblasts and macrophages as they phagocytose the lipids in bile, resulting in the formation of xanthoma cells. Given the rarity of the disease, there is no guideline on management and a majority of patients reported underwent surgical excision. This case demonstrates the utility of liver biopsy to establish this rare diagnosis, allowing for better detection and subsequently better-studied management. Funding Agencies 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) | 0,000 | 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 | 0,001 |
| 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.
Le détail, modèle par modèle et score par score, se trouve en fin de page sous « Comment cette classification a été obtenue ».