Cholestasis of pregnancy

Background

• Caused by combination of hormonal, genetic, and environmental factors --> impaired bile flow --> deposition of bile salts in skin and placenta
• May predispose mothers to vitamin K deficiency and increase risk of preterm delivery, IUFD, and neonatal respiratory distress syndrome

Clinical Features

• Typically develops in late second and/or 3rd trimester
• Rapidly resolves after delivery
• Pruritus, starts and predominates on palms and soles
• RUQ pain
• Nausea
• Jaundice

Differential Diagnosis

Jaundice

Differential diagnosis of hyperbilirubinemia.

Indirect Hyperbilirubinemia

• Hemolytic
  • G6PD
  • Drug related
  • Autoimmune hemolytic anemia
• Hematoma resorption
• Ineffective erythropoiesis
• Gilbert's

Direct (Conjugated) Hyperbilirubinemia

• Choledocholithiasis
• Cholecystitis
• Ascending cholangitis
• AIDS cholangiopathy
• Stricture
• Neoplasm
  • Pancreatic head
  • Gallbladder
  • Primary liver (e.g. hepatocellular carcinoma)
  • Metastatic
• Obstructing AAA

Hepatocellular damage

Patient will have severely elevated AST/ALT with often normal Alkaline Phosphatase

• Viral hepatitis
• Fulminant hepatic failure
• alcoholic hepatitis
• Ischemic hepatitis
• Toxins
  • Isoniazid
  • Phenytoin
  • acetaminophen
  • Ritonavir
  • Halothane
  • Sulfonamide
• Autoimmune hepatitis
  • Primary biliary cirrhosis
• HELLP Syndrome
• Congestive Hepatopathy
  • CHF
  • Sepsis (Shock Liver)

Pregnancy Related

• HELLP syndrome
• Acute fatty liver of pregnancy
• Hyperemesis gravidarum
• Cholestasis of pregnancy

Transplant Related

• Transplant rejection
• Graft-vs-host disease

Pediatric Related

• Inborn error of metabolism
• Neonatal jaundice (physiologic)

Additional Differential Diagnosis

• Reye syndrome
• TPN
• Heatstroke
• Budd-Chiari (with acute ascites)
• Wilson's disease
• Sarcoidosis
• Amyloidosis

Masqueraders

Only bilirubin stains the sclera

• Carotenemia
• Quinacrine ingestion
• Dinitrophenol, teryl (explosive chemicals)

Pregnancy-specific pruritus

see also: Rashes of pregnancy

• Pruritus gravidarum
• Atopic eruption of pregnancy
• Polymorphic eruption of pregnancy
• Pemphigoid gestationis
• Prurigo of pregnancy
• Pruritic folliculitis of pregnancy

Evaluation

• CBC (rule out thrombocytopenia)
• LFTs
  • Elevated total bile acid concentration
  • Aminotransferases usually <2x upper limit of normal
  • Alk phos and bili may be elevated
  • GGT normal or modestly elevated
  • RUQ US normal, no biliary duct dilation

Management

• Ursodeoxycholic acid (ursodiol) 300mg TID until delivery
• OBGYN follow-up

Disposition

• Discharge with OBGYN follow-up

References

https://www.uptodate.com/contents/intrahepatic-cholestasis-of-pregnancy