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Overlap syndrome
This document discusses overlap syndromes, specifically hepatic overlap syndromes where a patient presents with features of both autoimmune hepatitis (AIH) and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). It provides details on the proposed explanations for overlap syndromes, features that distinguish AIH, PBC and PSC, criteria for diagnosing AIH-PBC and AIH-PSC overlap syndromes, treatment recommendations, and practical points regarding implications for prognosis and management depending on the underlying conditions.
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Overlap syndrome
- 1. Overlap syndrome DrYasser M Fouad Professor of Gastroenterology and Hepatology Head of Tropical Medicine Department Minia University
- 2. Types of overlapsyndromes - Systemic sclerosis with other CT diseases - Myelodysplasia/myeloproliferative disorder - Atrial arrhythmia and Brughada syndrome - Chronic Asthma and chronic obstructive sleep apnea syndrome - Functional dyspepsia-IBS syndrome - Hepatic overlap syndrome
- 3.
- 5. Variants of AIHor separate entities ? PBC AIH PSC
- 6. Genetic susceptibility andenvironmental factors Cellular immunity Humoral immunity Immune mediated injury Hepatocytes Bile ducts AIH PBC or PSC Overlap syndrome
- 7. Proposed explanations foroverlap syndrome - Coexistence or sequential presentation of two independent diseases in a susceptible patient - “Overlap syndromes” represent distinct entities - “Overlap syndromes” are in the middle of a wide continuum of manifestations, ranging from pure hepatitic to pure cholestatic -“Overlap syndromes” are the result of heterogeneous manifestations of a primary disorder
- 8. Features of AIH,PBC, PSC variable AIH PBC PSC age All ages 30-65 30-50 Predominantly males Predominantly females Predominantly females sex Normal or mild elevation Normal or mild elevation AST&ALT Marked elevation Bilirubin Normal or elevated Normal or elevated Normal or elevated Moderate to marked elevation Moderate to marked elevation Normal or mild elevated ALP
- 9. Features of AIH,PBC, PSC variable AIH PBC PSC immunoglobulins Ig G Ig M Ig M & Ig G ANA 70-80% 30% 8-70% SMA 50% May be present Variable (0-80%) LKM 3-4% Negative Negative SLA 10-30% Yes or no Yes or no
- 10. Features of AIH,PBC, PSC variable AIH PBC PSC Occasionaly positive in 90-95% May be present low titre AMA pANACA 50-96% - 26-94% Inflammatory biliary Periductal fibrosis injury Liver histology Interface hepatitis Cholangiogram normal Normal Multifocal stricturing 80% No effective TTT Very rare UDKA 3-10% Immunosupressives IBD Treatment
- 11. Primary biliary cirrhosisAIH overlap syndrome • ALP˃ 2 times or GGT ˃5 times ULN • Positive AMA • Histological evidence of bile duct injury PBC • ALT ˃ 5 times ULN • Ig G ˃2 times ULN or positive SMA • Liver biopsy: Moderate to severe Periportal or periseptal inflammation AIH Diagnosis require at least 2 out of 3 features in each component of the overlap
- 12. Autoimmune hepatitis –PSC overlap syndrome AIH-PSC Cholestatic biochemical alteration Concurrence of IBD Cholangiographic evidence of intra and or extrahepatic biliary dilation Histological evidence of ductpoenia and bile stasis ANA or SMA seropositivity + Hypergamaglobulinemia + Interface hepatitis
- 13. Treatment AIH –PBC ALK LESS THAN 2 FOLDS ULN • Prednisone or prednisolone: • 30 mg 1 week • 20 mg 1 week • 15mg 2 weeks • 10 mg daily • Combined with Azathioprine 50 mg from start AIH - PBC • Same + • Ursodeoxycholic acid 13-15 mg /kg/day AIH-PSC Prednisone or • prednisolone combined with azathioprine 50 -75 mg/day • Ursodeoxycholic acid 13-15 mg /kg/day
- 14. Practical point #1 If you have a patients with AIH and overlap with PBC: - No follow up changes - You add a comfortable drug (UDCA) which may slow down the progression of the disease and reduces mortality - Possible reduced life expectancy
- 15. Practical point #2 If you have a patients with AIH and overlap with PSC: - substancial follow up changes( the patient more liable to cholangiocarcinoma, hepatoma, or gall bladder carcinoma) - You add a comfortable drug (UDCA) but possibly ineffective to slow down the progression of the disease - Possible reduced life expectancy
- 16. Practical point #3 IF BPC or PSC are preceding , making a diagnosis of AIH overlap syndrome : - You will add a uncomfortable but very effective drug (immunosuppressive) to slow down the progression of the disease and - Dramatically change life expectancy
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