How best to manage chronic cholestasis
Here's how to maximize your use of lab work and imaging techniques to identify the source of your patient's cholestasis and provide prompt treatment.
PRACTICE RECOMMENDATIONS
› Suspect intrahepatic cholestasis in a patient with pruritus, normal transaminases, and mildly elevated gamma glutamyl-transpeptidase and alkaline phosphatase levels. A
› Use ultrasonography as a first-line diagnostic tool for cholestasis. A
Strength of recommendation (SOR)
A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series
From The Journal of Family Practice | 2018;67(7):E9-E15.
Other circulating antibodies can help discriminate among cholestatic disorders. In particular, positive tests for perinuclear anti-neutrophil cytoplasmic antibodies (pANCA) are found in 25% to 95% of patients with primary sclerosing cholangitis (PSC), a chronic progressive disorder of unknown etiology that is characterized by inflammation, fibrosis, and stricturing of medium and large ducts of the intrahepatic and extrahepatic biliary tree.24 Anti-smooth muscle antibodies (SMA) can be observed in both PSC and autoimmune hepatitis.
Finally, there are syndromes with serologic and histologic overlap that are characterized by the simultaneous presence of PBC with autoimmune hepatitis or PSC or overlap of PSC with autoimmune hepatitis.
Liver biopsy fills in the rest of the diagnostic picture
Unfortunately, blood tests reveal little about organ integrity and are not useful for disease staging. The decision to perform a liver biopsy should be based on several factors, including the patient’s age, serum parameters, the need to stage the disease, therapy choices, and prognosis.12 One should also consider that biopsy is a costly procedure with potentially serious adverse effects; it should not be repeated frequently. However, when a biopsy is done, it provides critical information, including damage to medium-sized intrahepatic bile ducts with neoductular formation or bile duct scars and strictures.
Treating intrahepatic cholestasis
Although FPs often can provide most—or even all—of the care for patients with stable conditions, a specialist consultation might recommend further testing to identify the underlying disease, which is essential to establish the most appropriate treatment.
Treatment of patients with PBC is based on administering hydrophilic secondary bile salt ursodeoxycholic acid (UDCA) 15 mg/kg/d, which is used to equilibrate the ratio between hydrophilic and hydrophobic bile salts in the liver and bile,25 and is the only treatment approved by the US Food and Drug Administration (FDA) for PBC.4 Tauroursodeoxycholate is better absorbed than UDCA, and, although partially deconjugated and reconjugated with glycine, it undergoes reduced biotransformation to more hydrophobic metabolites and has benefits, including antioxidant, immunomodulation, and neuroprotective effects over UDCA—especially for long-term therapy in PBC.26 However, it is not used often in clinical practice.
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