Primary Biliary Cirrhosis, An Issue of Clinics in Liver Disease

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Primary biliary cirrhosis (PBC) is a chronic, slowly progressive, cholestatic disorder first described >150 years ago. The name of this liver disorder is itself misleading because the majority of patients do not have cirrhosis at the time of diagnosis. However, an alternative term proposed for this hepatic disorder—pleomorphic destructive nonsuppurative cholangitis—is quite a mouthful to enunciate and it is obvious that it has not been embraced by the hepatology community. PBC is increasingly recognized worldwide, although there is significant geographic variation in its incidence and apparent marked clustering in certain regions within a country or even in certain urban areas in a county or a state.

The May 2008 issue of Clinics in Liver Disease is devoted to PBC. There are 15 chapters contributed by an august international faculty. The chapters include topics such as “Presentation and Diagnosis of Primary Biliary Cirrhosis in the 21st Century,” “Antimitochondrial Antibodies,” “Natural History,” and “Recent Advances in the Epidemiology and Pathogenesis of PBC.” There are also specific chapters devoted to animal models and genetics and genomics. The clinical hallmarks of this disorder—fatigue, pruritus, and osteoporosis—are singled out in distinctive chapters and are covered with exceptional clarity and depth. The figures, pie charts, and algorithmic approach in the chapter on fatigue are outstanding and the author offers a pragmatic approach to treating this vexing and sometimes very debilitating symptom, after excluding associated and coexisting conditions such as hypothyroidism and celiac sprue. The potential contribution of autonomic dysfunction and sleep disturbances to the fatigue that these patients suffer from is something that many a reader may not be aware of. Similarly, in the figure depicting the prevention and treatment of osteoporosis in the corresponding chapter, the author provides a lucid suggested protocol of general measures and specific strategies.

Bottom Line: There is a wealth of interesting and useful information that has been provided in this volume. The chapters are extensively referenced, updated, well illustrated, and the graphics are of excellent quality. Virtually all of the chapters have a pithy but well-written summary paragraph. This book is intended for all those interested in advancing the understanding of this disorder and optimizing the care of patients with PBC. I congratulate the editor, Dr. Heathcote, and the authors for a job well done.