Case 11
Case submitted by Bilal Hameed, MD
A 34 year old male with left sided ulcerative colitis (UC), diagnosed at the age of 28, is well controlled on delayed release mesalamine. He has had no recent flare ups. The patient denies any fevers, chills, abdominal pain, jaundice, weight loss, worsening diarrhea or bleeding. He denies any alcohol or illicit drug use. He has no new medications or herbal supplements.
On routine labs he was found to have abnormal liver chemistries with total bilirubin 0.8 mg/dL, ALT 20 U/L, AST 18 U/L, alkaline phosphatase of 230 U/L and GGT 140 U/L.
What initial diagnostic work-up will you perform for this patient? (select all that apply)
P-ANCA
P-ANCA can be positive in 26-94% patients with PSC but is not disease specific nor does it reflect prognosis
Liver Biopsy
Liver biopsy is not indicated for the diagnosis of PSC. It may be considered to assess for small duct PSC (patients with clinical suspicion but normal cholangiogram) or to assess for overlap syndromes (aminotransferase levels more than 2-3 times upper limits of normal
ERCP
ERCP is reserved for therapeutic interventions and evaluation of dominant strictures, where there is concern for cholangiocarcinoma.
Anti-Mitochondrial Antibody
To exclude primary biliary cholangitis (PBC), checking for antimitochondrial antibody is recommended in all patients.
MRCP
Cholangiography with MRCP is the modality of choice for the diagnosis of PSC and preferred over ERCP. MRCP has a sensitivity of 86% and specificity of 94% for the diagnosis of PSC.
IgG4 Level
While not used in initial diagnosis, serum IgG4 should be checked in all patients with PSC as 10% can have elevated levels and this can be associated with worse outcomes. This is a separate entity from IgG4 related disease.