Extrahepatic Autoimmune Conditions Associated with Primary Biliary Cirrhosis

Floreani, Annarosa; Franceschet, I.; Cazzagon, Nora; Spinazzè, Alice; Buja, Alessandra; Furlan, Patrizia; Baldo, Vincenzo; Gershwin, M. Eric

doi:10.1007/s12016-014-8427-x

Cited by 173 publications

(168 citation statements)

References 35 publications

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“…Patients mainly died from nonhepatic primary cancers or hematological malignancies, whereas there is classically no such increased risk of extrahepatic malignant conditions in PBC. (25)(26)(27) Quite rightly, it may be argued that such a link between AMAs and non-liver-related mortality likely results from complications of concomitant diseases and/or treatments (immunosuppressive drugs) rather than from the consequences of AMAs. Unfortunately, we could not obtain data from the patients who were tested AMA negative during the same census period, which precludes any conclusions.…”

Section: Discussionmentioning

confidence: 99%

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

et al. 2017

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The prevalence, clinical characteristics, and outcomes of patients with antimitochondrial antibodies (AMAs), but no clinical evidence of primary biliary cholangitis (PBC), are largely unknown. A prospective study of AMA incidence was conducted through a nation-wide network of 63 French immunology laboratories. Clinical data from 720 of 1,318 AMApositive patients identified in 1 year were collected. Patients were categorized as either newly diagnosed with PBC (n 5 275), previously diagnosed with PBC (n 5 216), or with nonestablished diagnosis of PBC (n 5 229). The latter group was specifically evaluated. Follow-up data were collected for up to 7 years after detection of AMAs. Prevalence of AMApositive patients without evidence of PBC was 16.1 per 100,000. These patients had the following characteristics: 78% female; median age 58 years; median AMA titer 1:160; extrahepatic autoimmune disorders 46%; normal serum alkaline phosphatases (ALP) 74%; ALP above 1.5 times the upper limit of normal 13%; and cirrhosis 6%. Compared to those newly diagnosed with PBC, the patients were slightly younger, had lower AMA titers, and lower sex-ratio imbalance. Among the patients with normal ALP and no evidence of cirrhosis, the 5-year incidence rate of PBC was 16%. Whereas no patients died from PBC, the 5-year survival rate was 75%, as compared to 90% in a control, standardized population matched for age and sex (P < 0.05). Conclusion: Nearly half of the newly detected AMAs in clinical practice does not lead to a diagnosis of PBC. PBC is unrecognized in 13% of those cases. Only 1 in 6 patients with AMAs and normal ALP will develop PBC after 5 years. The mortality of AMA-positive patients without PBC is increased irrespective of the risk of PBC development. (HEPATOLOGY 2017;65:152-163).

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Section: Discussionmentioning

confidence: 99%

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

et al. 2017

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“…Table 1 shows the clinical features of the five relevant cases and the present one. In a prospective study of 361 PBC patients, Floreani et al reported on seven patients with PBC associated with Graves' disease, although detailed information about those patients was not shown [4]. The five reported cases were all females.…”

Section: Discussionmentioning

confidence: 99%

“…Clinically, it seems there are some differences in PBC patients between the sexes, with females tending to present with pruritus more often than males, and with males at a higher risk of developing gastrointestinal bleeding and hepatocellular carcinoma [17,18]. It is noteworthy that males are less likely to suffer concomitant autoimmune disease than females [4,18].…”

Section: Discussionmentioning

confidence: 99%

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Primary biliary cirrhosis associated with Graves’ disease in a male patient

Suzuki

Ishida

Takahashi

et al. 2016

Clin J Gastroenterol

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Primary biliary cirrhosis (PBC), which predominantly affects women, has been associated with various autoimmune diseases. Although hypothyroidism accompanying PBC is well documented, the concomitance of PBC and hyperthyroidism is rare. Herein, we report the case of a 62-year-old man who was diagnosed with PBC several years after the development of Graves' disease. This is the first case of a male patient developing PBC with Graves' disease. Both serum alanine aminotransferase levels and serum thyroid hormone levels were normalized after the administration of thiamazole for Graves' disease. However, the cholestatic liver enzyme abnormalities continued, indicating that the PBC was actualized by the administration of thiamazole. After starting ursodeoxycholic acid treatment, cholestatic liver enzyme abnormalities improved. Taken together, when a cholestatic pattern of liver enzymes is observed during follow-up for Graves' disease, an association between Graves' disease and PBC should be considered as a differential diagnosis.

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“…Similarly, HT, rheumatoid arthritis (RA), systemic sclerosis (SS), and Raynaud's disease may coexist with PBC [21][22][23].…”

Section: Gd Complicated By Pbcmentioning

confidence: 99%

A Literature Review of Concomitant Primary Biliary Cirrhosis and GravesÂ’ Disease

Shizuma¹

2015

J Gastrointest Dig Syst

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Although Hashimoto's thyroiditis (HT) is commonly characterized by extrahepatic manifestations of primary biliary cirrhosis (PBC), coexistence of PBC and Graves' disease (GD) is uncommon. This is a review of the English and Japanese scientific literature, comprising 7 cases of PBC and GD coexistence. All patients were female. In 4 cases, both diseases were almost simultaneously diagnosed, whereas PBC preceded GD in the remaining 3 cases. One fatality was observed on account of sepsis and liver failure caused by progressing PBC.

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Extrahepatic Autoimmune Conditions Associated with Primary Biliary Cirrhosis

Cited by 173 publications

References 35 publications

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitis

Primary biliary cirrhosis associated with Graves’ disease in a male patient

A Literature Review of Concomitant Primary Biliary Cirrhosis and GravesÂ’ Disease

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