Cryptogenic Cirrhosis: What Are We Missing?

Caldwell, Stephen H.

doi:10.1007/s11894-009-0082-7

Cited by 45 publications

(33 citation statements)

References 74 publications

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“…Prevalence of cryptogenic cirrhosis varies from 5% to 30%. [28][29][30] Determining etiology is challenging task. Neither imaging nor serology is helpful in such cases.…”

Section: Discussionmentioning

confidence: 99%

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Amarapurkar

Amarapurkar²

2015

Journal of Clinical and Experimental Hepatology

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Background: Liver biopsy (LB) has been used as diagnostic modality in liver diseases (LD). Over last two decades, there has been remarkable improvement in understanding of natural history, molecular diagnostics of viral hepatitis, genetic of LD, and also limitations of LB. There is current trend in avoiding LB in the management of various LDs. Aim: To determine utility of LB in clinical practice. Material and Methods: In a prospective study, 2413 patients of LD were followed up, 219 (9%) were acute, and remaining 2194 (90.9%) were chronic LD. Patients were evaluated by biochemical parameters, virological studies, and imaging endoscopy as and when required. LB was performed in 176 (7.2%) patients when no conclusion could be drawn from the noninvasive workup. Patients with platelet count <50,000/cm 2 , ascites, and overt bleeding were excluded. Patients with international normalization ratio (INR) more than 1.5 were not excluded. No prophylactic use of fresh frozen plasma and platelet transfusion was done. There was no major complication related to the procedure. Indications for LB were as follows: cryptogenic LD 38 cases, hepatitis B infection 35, suspected autoimmune hepatitis 30, mass lesion in the liver and lymphoma 29, evaluation of portal hypertension 15, elevated liver enzymes 11, hepatitis C infection 9, and drug-induced LD 4, and miscellaneous 5 cases which were primary biliary cholangitis, primary sclerosing cholangitis, cholestatic LD, sarcoidosis, and amyloidosis. Results: LB changed the diagnosis in 55 (31.2%). These were cryptogenic LD in 24 cases, portal hypertension 15, elevated liver enzymes 11, and 5 others. In remaining, LB confirmed clinical diagnosis and helped in making management decisions. Conclusion: LB was required in 7.2% of patients with chronic LD. In 31.2% cases, LB changed the diagnosis. LB was a safe procedure even in presence of low platelet count and abnormal INR. ( J CLIN EXP HEPATOL 2015;5:314-319)

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“…Prevalence of cryptogenic cirrhosis varies from 5% to 30%. [28][29][30] Determining etiology is challenging task. Neither imaging nor serology is helpful in such cases.…”

Section: Discussionmentioning

confidence: 99%

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Amarapurkar

Amarapurkar²

2015

Journal of Clinical and Experimental Hepatology

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“…Both autoimmune hepatitis and cryptogenic chronic hepatitis are diagnosed mainly by the exclusion of diseases that have a precise etiology [10,13,82]. The important distinction between autoimmune hepatitis and cryptogenic chronic hepatitis is that autoimmune hepatitis has a characteristic clinical phenotype and treatment response [84] (Table 1).…”

Section: Cryptogenic Chronic Hepatitis and Autoimmune Hepatitismentioning

confidence: 99%

“…Institutions that apply nonstandard serological tests, such as antibodies to soluble liver antigen (anti-SLA) [3][4][5], liver cytosol type 1 (anti-LC1) [6], asialoglycoprotein receptor (anti-ASGPR) [7,8] or uridine diphosphate glucuronosyltransferases (anti-UGT) [9] or that have focused expertise in metabolic disorders (diabetes and metabolic syndrome) [10][11][12][13], hereditary conditions (Wilson disease, hemochromatosis, and alpha-1 anti-trypsin deficiency) [14][15][16][17][18][19][20][21][22][23][24][25][26], nonstandard viral infections (hepatitis E virus, hepatitis G virus, and immunodeficiency virus) [27][28][29][30][31][32][33][34][35] and celiac disease [36][37][38][39][40][41] may have different concepts about the true nature of cryptogenic chronic hepatitis. Similarly, experiences from liver transplantation centers may differ from those that assess patients with active precirrhotic disease.…”

Section: Introductionmentioning

confidence: 99%

“…This shift in focus in part reflects better exclusionary tests and the increased frequency of obesity and the metabolic syndrome worldwide [12,[55][56][57][58][59]. Interspersed within this changing spectrum of cryptogenic chronic hepatitis have also been re-classifications that have emphasized an autoimmune nature in some cases [2,[60][61][62][63] and an unsuspected drug or alcohol exposure in others [11,13,54,64].…”

Section: Introductionmentioning

confidence: 99%

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Cryptogenic Chronic Hepatitis and Its Changing Guise in Adults

Czaja

2011

Dig Dis Sci

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Cryptogenic chronic hepatitis is a disease that is unexplained by conventional clinical, laboratory and histological findings, and it can progress to cirrhosis, develop hepatocellular carcinoma, and require liver transplantation. The goals of this review are to describe the changing phenotype of cryptogenic chronic hepatitis in adults, develop a diagnostic algorithm appropriate to current practice, and suggest treatment options. The frequency of cryptogenic hepatitis is estimated at 5.4%. Cryptogenic cirrhosis is diagnosed in 5-30% of patients with cirrhosis, and it is present in 3-14% of adults awaiting liver transplantation. Nonalcoholic fatty liver disease has been implicated in 21-63% of patients, and autoimmune hepatitis is a likely diagnosis in 10-54% of individuals. Viral infections, hereditary liver diseases, celiac disease, and unsuspected alcohol or drug-induced liver injury are recognized infrequently in the current cryptogenic population. Manifestations of the metabolic syndrome heighten the suspicion of nonalcoholic fatty liver disease, and the absence of hepatic steatosis does not discount this possibility. The diagnostic scoring system of the International Autoimmune Hepatitis Group can support the diagnosis of autoimmune hepatitis in some patients. Certain genetic mutations may have disease-specificity, and they suggest that some patients may have an independent and uncharacterized disease. Corticosteroid therapy is effective in patients with autoimmune features, and life-style changes and specific therapies for manifestations of the metabolic syndrome are appropriate for all obese patients. The 1- and 5-year survivals after liver transplantation have ranged from 72-85% to 58-73%, respectively.

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“…Patients with NAFLD are also at an increased risk of endstage liver disease, CVD and diabetes, which explain their overall increased mortality rate [Ekstedt et al 2006;Adams et al 2005b]. NASH can progress to cirrhosis in up to 515% of patients and is now recognized as the most common cause of cryptogenic cirrhosis [Caldwell, 2010;Bugianesi et al 2007;Harrison, 2006]. Obesity and T2DM are present in a large portion of patients who develop cryptogenic cirrhosis, an association not seen in hepatitis-C-related cirrhosis or primary biliary cirrhosis.…”

Section: Magnitude Of the Problem: Prevalence And Natural Historymentioning

confidence: 99%

An endocrine perspective of nonalcoholic fatty liver disease (NAFLD)

Lomonaco

Chen

Cusi

2011

Therapeutic Advances in Endocrinology

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Abstract:Endocrinologists are encountering patients with obesity-related complications such as metabolic syndrome (MetS) and type 2 diabetes mellitus (T2DM) on a daily basis. Nonalcoholic fatty liver disease (NAFLD) is a liver condition characterized by insulin resistance, hepatic steatosis and frequently T2DM. This is now the most common chronic liver condition in adults and is present in the majority of obese subjects. Liver fat accumulation may range from simple steatosis to severe steatohepatitis with hepatocyte necroinflammation (or nonalcoholic steatohepatitis [NASH]). Although the natural history is incompletely understood, NAFLD may lead to serious medical consequences ranging from cirrhosis and hepatocellular carcinoma to earlier onset of T2DM and cardiovascular disease (CVD). The diagnosis of NAFLD may be challenging because signs and symptoms are frequently absent or nonspecific, and thus easily missed. Liver aminotransferases may be helpful if elevated, but most times are normal in the presence of the disease. Liver imaging may assist in the diagnosis (ultrasound or MRI and spectroscopy) but a definitive diagnosis of NASH still requires a liver biopsy. This may change in the near future as novel biomarkers become available. Treatment of NAFLD includes aggressive management of associated cardiovascular risk factors and many times control of T2DM. Pioglitazone and vitamin E appear promising for patients with NASH, although longterm studies are unavailable. In summary, this review hopes to address the common clinical dilemmas that endocrinologists face in the diagnosis and management of NAFLD and increase awareness of a potentially serious medical condition.

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Cryptogenic Cirrhosis: What Are We Missing?

Cited by 45 publications

References 74 publications

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Indications of Liver Biopsy in the Era of Noninvasive Assessment of Liver Fibrosis

Cryptogenic Chronic Hepatitis and Its Changing Guise in Adults

An endocrine perspective of nonalcoholic fatty liver disease (NAFLD)

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