Autoimmune diseases of the liver

This book is a state-of-the-art review compiling all the key aspects of autoimmune liver diseases (AILD). It not only covers the spectrum of autoimmune hepatitis (AIH) but also gives an in depth review of the primary cholestatic diseases of the liver.

In the current era, AILD contributes to a significant cause of liver related disorders all over the world and constitutes a major chunk of “difficult-to-treat” patients encountered in the field of Hepatology. The emerging intricacies associated with the pathogenesis, diagnostic challenges and management options of patients with AILD demand for a book of this kind, which gives a lucid, concise and ready to use information regarding the key aspects of this disease. The first chapter of the book starts with a snapshot of the existing burden of AILD and depicts a picture of its worldwide incidence by quoting extensive literature of various population-based studies from all over the world giving the reader a sense of insight of the existing problem associated with these diseases.

The first part of the book emphasizes predominantly on AIH, covering in depth the genetic risk, pathogenesis, diagnostic criteria and management strategies of the disease. This book also elucidates the underlying immunobiology of the development of AILD and the chapter on regulatory T cells lucidly explains the emerging concept of immune tolerance in patients with AILD with the help of figures which aid in understanding this complex concept with ease. The various diagnostic criteria for AIH have been clearly explained and a mention of the limitations of these diagnostic scores has also been made which helps the reader attain adequate knowledge on their strengths and weaknesses. The chapter on the role of histopathology gives a crisp idea on the various histologic changes encountered in patients with AIH, however, the text on ‘many histological faces of AIH’ deserves special attention. It explains histological changes at different stages of AIH like fulminant AIH, AIH presenting with cirrhosis and post treatment AIH, benefiting not only clinicians but also histopathologists who are actively involved in reporting these diseases.

This book clears several doubts pertaining to the treatment of AIH by giving latest review of literature on the current aspects of managing patients with AIH, with special emphasis on various steroid regimens to be used and compiling all vital data regarding the endpoints of treatment, thus enabling the reader to understand the complexities of various treatment protocols with ease. Other than the conventional methods of treatment of AIH, this book also discusses various alternative regimens and drugs available for difficult to treat AIH patients.

The spectrum of paediatric AIH is covered in detail which may be of help to practicing paediatricians in understanding the complex problems associated with the diagnostic dilemmas and disease severity. Additionally, alternative treatment options including treatment of refractory cases and role of liver transplantation in children are also discussed. Autoimmune sclerosing cholangitis (AISC), which classically affects children and young adults and imposes not only a diagnostic difficulty but also a therapeutic challenge in management of these patients has also been discussed.

The second part of the book is devoted to primary cholestatic diseases of the liver including both primary biliary cholangitis (PBC) and primary sclerosing cholangitis (PSC). The pathogenesis of PBC has been explained in detail and the genetic risk factors of PBC has been mentioned in depth citing various genome-wide association studies (GWAS). Clinicians treating patients with PBC are well aware of the frustrating symptom of fatigue reported by several patients suffering with this disease. This book not only explains the pathogenesis of fatigue in such patients but also suggests relevant points in managing the same. The ‘TRACE’ approach which is a structured and systematic approach to manage fatigue in patients of PBC and makes it extremely simple for readers to follow the same in their clinical practice is also discussed. The role of ursodeoxycholic acid (UDCA) in treating patients with PBC has met several landmarks in history and this book not only gives the latest literature evidence supporting its role in patients with PBC but also mentions the treatment options for patients with suboptimal response to UDCA. Role of various drugs which are in the pipeline for managing PBC like budesonide, fibrates, obeticholic acid and rituximab have been mentioned citing relevant references.

The book covers the various aspects of epidemiology and diagnostic aspects of PSC. The chapter specially written to encompass all the malignancies associated with PSC deserves special attention. Detailed mechanisms of the malignant transformation in patients with PSC including epithelial-mesenchymal transition has been explained in simple and lucid language which will help the readers imbibe the intricate mechanisms and also help them learn various treatment modalities to manage the same. Various therapeutic approaches to manage PSC including the chapter on ‘therapy tomorrow’ form the essential highlights of this book as it describes and compiles all the recent literature to manage patients with PSC and what is likely to be available in future. The book also has a separate chapter on the pathogenesis and management of pruritis in patients with primary cholestatic liver diseases which discusses the role of various drugs and describes a stepwise approach for treating this symptom in patients suffering from PBC and PSC. Separate chapters written on diseases like IgG4 associated cholangitis and various overlap syndromes will be of immense help to practicing clinicians in the field of hepatology as these diseases impose serious diagnostic challenges.

There are a few quibbles about the book. The recurrence of AILD after liver transplantation (LT), pathogenesis and management of de novo AIH after LT have not been covered. Though the medical management of patients with PSC has been exhaustively covered, endoscopic strategies to treat complications like dominant strictures have not been discussed in detail. Overall, this book accomplishes the daunting task of covering most of the important aspects of AILD and is a must read for those dealing with these patients in the field of hepatology and gastroenterology. It would also cater to the general physicians and internists who actively take part in the management and care of patients with autoimmune liver diseases.