MCQ Pathology Answer 43
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Primary sclerosing cholangitis (PSC)—which is characterized by progressive inflammation, destruction, and fibrosis of intrahepatic and extrahepatic bile ducts—predominantly afflicts young men with inflammatory bowel disease.
Liver biopsy is required for staging disease severity and determining prognosis in PSC. Periductal fibrosis with inflammation, bile duct proliferation alternating with obliteration, and ductopenia constitute the main histological findings. Fibroobliterative cholangiopathy, which is considered the most diagnostic finding on liver biopsy, is present in only 10% of all cases.
PRIMARY SCLEROSING CHOLANGITIS :
- Immune Mediated
- Perinuclear antineutrophil cytoplasmic antibody (pANCA) has been reported in 26% to 85% of PSC subjects and in 70% of UC patients without PSC. The antigenic and subsequent pathogenic role of pANCA, however, is unknown.
- An increased prevalence of HLA-A1, -B8, and -DR3 alleles in PSC has been described but remains nonspecific
- 50-70% of affected individuals are of male gender, with an average age of 40 years at diagnosis.
- Asymptomatic elevations in serum liver biochemistries or the development of symptoms such as fatigue (75%) and pruritus (70%) are observed with the diagnosis of PSC. Jaundice (65%) and weight loss (40%) are less common and should raise the suspicion of advanced disease or a complication related to PSC. The average duration of symptoms before diagnosis is 2 years.
- A previous history of IBD (especially ulcerative colitis) and elevated serum liver biochemistries often prompts investigations to exclude PSC.
- Serum hepatic biochemical parameters usually reflect a cholestatic profile. Alkaline phosphatase values often predominate, with elevations up to three times the upper limit of normal in nearly 95% of cases. Serum alanine and aspartate transaminase levels can also be found between two and three times the upper limit of normal. Of note, serum total bilirubin is normal in 60% of individuals.
- Serum immunoglobulin levels and autoantibodies are associated with PSC. Elevations in IgG and IgM in up to 40% and 60% of individuals are observed. ANA and smooth muscle antibody ASMA can also be detected but are in lower titers compared to AIH. Antimitochondrial antibodies (AMAs) are exceedingly rare in PSC.
Category: Pathology MCQs
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