Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis: a Review Featuring a Women's Health Perspective

Abstract

Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are two major types of chronic cholestatic liver disease. Each disorder has distinguishing features and variable progression, but both may ultimately result in cirrhosis and hepatic failure. The following offers a review of PBC and PSC, beginning with a general overview of disease etiology, pathogenesis, diagnosis, clinical features, natural course, and treatment. In addition to commonly associated manifestations of fatigue, pruritus, and fat-soluble vitamin deficiency, select disease-related topics pertaining to women's health are discussed including metabolic bone disease, hyperlipidemia and cardiovascular risk, and pregnancy-related issues influencing maternal disease course and birth outcomes. This comprehensive review of PBC and PSC highlights some unique clinical considerations in the care of female patients with cholestatic liver disease.

Keywords: Cholestasis; Fat-soluble vitamin deficiency; Fatigue; Hyperlipidemia; Metabolic bone disease; Pregnancy; Primary biliary cirrhosis; Primary sclerosing cholangitis; Pruritus; Women's health.

Fig. 1. H&E, X100; Liver biopsy.

The classic appearance of primary biliary cirrhosis in its early portal inflammatory stage demonstrating granulomatous features with interface hepatitis. The inflammatory infiltrate is typically composed of lymphocytes and plasma cells. The lymphocytes may form lymphoid nodules, occasionally with germinal centers. Inflammation typically surrounds the interlobular bile ducts that might show evidence of injury. Abbreviation: H&E (Hematoxylin & Eosin).

Fig. 2. H&E, X200; Liver biopsy.

An absence/paucity of bile ducts is seen with focal chronic inflammation in a portal area consistent with late-stage primary biliary cirrhosis.

Fig. 3. Trichrome, X40; Liver Biopsy.

Low power view demonstrating a focal lesion typical for primary sclerosing cholangitis. Periductular layered fibrosis (characteristic “onion skin” pattern) is seen with edema and inflammation surrounding the interlobular bile ducts in the center of the field. A normal portal area with bile ducts is seen in the upper right corner of the image.

Fig. 4. Trichrome, X400; Liver biopsy.

Typical focal lesion of primary sclerosing cholangitis demonstrating “onion skin” concentric fibrosis and chronic periductular inflammation. Notice significant edema in this relatively early phase of fibrosis.

Fig. 5

Magnetic resonance cholangiogram in a patient with primary sclerosing cholangitis demonstrating characteristic findings of multifocal biliary strictures with intervening dilation and areas of sacculation.