Connective tissue diseases in primary biliary cirrhosis: A population-based cohort study

Wang, Li; Zhang, Fengchun; Chen, Hua; Zhang, Xuan; Xu, Dong; Li, Yongzhe; Wang, Qian; Gao, Lixia; Yang, Yun; Kong, Fang; Wang, Ke

doi:10.3748/wjg.v19.i31.5131

Cited by 68 publications

(49 citation statements)

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“…A recent study that evaluated 322 Chinese patients with PBC revealed a PBC-PM coexistence rate of 3.1% [7]. European-based studies demonstrated a much lower incidence, for instance, no cases were reported in a cohort of 160 PBC patients [8].…”

Section: Discussionmentioning

confidence: 98%

“…This perspective also applies to autoimmune liver diseases, characterized by a high percentage of disease associations. Floreani et al reports a 61.2% concurrence of PBC and extrahepatic autoimmune conditions [6], out of which up to 46.6% are represented by one or more connective tissue diseases [7]. Autoimmune thyroid disease has been observed in 23% of patients with PBC [8] and 18.3% of patients with PBC-AIH overlap syndrome [9].…”

Section: Discussionmentioning

confidence: 99%

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Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Pamfil

Candrea

Berki³

et al. 2015

JGLD

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Autoimmune liver diseases may be associated with extrahepatic autoimmune pathology. We report the case of a 52-year old woman who initially presented to the gastroenterology department for extreme fatigue, pale stools, dark urine and pruritus. Laboratory tests showed significant cholestasis and elevation of aminotransferase levels. Immunological tests revealed positive antinuclear (ANA=1:320) and antimitochondrial antibodies (AMA=1:40) with negative anti-smooth muscle and liver kidney microsomal type 1 antibodies. The biopsy was compatible with overlap syndrome type 1. The patient was commenced on immunosuppressive therapy according to standard of care (azathioprine 50mg, ursodeoxycholic acid and prednisone 0.5mg/kg), with moderate biochemical improvement. She subsequently developed proximal symmetrical weakness and cutaneous involvement and was diagnosed with biopsy-proven dermatomyositis. The immunosuppressive regimen was intensified to 150 mg azathioprine. At the three-month follow-up, her symptoms subsided and aminotransferases and muscle enzymes normalized. Upon further investigation the patient was diagnosed with autoimmune thyroiditis and antiphospholipid syndrome. To our knowledge, this is the first case of primary biliary cirrhosis - autoimmune hepatitis overlap syndrome associated with dermatomyositis, autoimmune thyroiditis and antiphospholipid syndrome.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Pamfil

Candrea

Berki³

et al. 2015

JGLD

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“…Furthermore, Muratori et al [47] showed no differences between subgroups of PBC patients with and without concomitant autoimmune disorder in terms of age at diagnosis, serum level of antibodies and long-term complications of liver disease. On the other hand, Rigamonti et al [48] reported a slower progression of liver disease in PBC patients with coexisting systemic sclerosis, whereas Wang et al [49] observed certain differences in the laboratory features between Chinese PBC patients with and without overlapping connective tissue disease. These interesting results should be verified in future studies concerning presence of extrahepatic autoimmune conditions and progression of chronic cholestatic disorders.…”

Section: Discussionmentioning

confidence: 98%

Genetic Risk Factors for Autoimmune Thyroid Disease might Affect the Susceptibility to and Modulate the Progression of Primary Biliary Cholangitis

Kuś

Arłukowicz-Grabowska

Szymański

et al. 2017

JGLD

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Background & Aims: Patients with primary biliary cholangitis (PBC) frequently suffer from extrahepatic autoimmune conditions, of which autoimmune thyroid disease (AITD) is one of the most common. Previous studies identified several genetic variants increasing the odds of developing AITD. Here we investigate whether AITD-associated polymorphisms might also play a role in the development and clinical course of PBC and PBC associated with AITD (PBC-AITD).Methods: To this end, we prospectively recruited 230 patients with PBC and 421 healthy controls. Among recruited patients, 64 (30.9%) had PBC-AITD as diagnosed by elevated serum TPO-antibodies. In all subjects we genotyped 10 variants previously associated with AITD.Results: We detected significant associations between the PTPN22 polymorphism and risk of developing PBC (rs2476601, OR=1.43, P=0.035) as well as PBC-AITD (OR=1.74, P=0.028). The IL2RA polymorphism was associated with liver cirrhosis (rs41295061, OR=1.76, P=0.033) whereas the MMEL1 polymorphism increased the risk of requiring liver transplantation (rs2843403, OR=1.70, P=0.023). Although no significant differences in clinical or biochemical characteristics between patients with PBC and PBC-AITD were seen (all P>0.05), liver function tests and metabolic traits in PBC patients were significantly (all P<0.05) affected by the CTLA4 (rs3087243), MMEL1 (rs2843403), PTPN22 (rs2476601) and RNASET2 (rs9355610) variants.Conclusion: Our study demonstrates the existence of a genetic overlap between PBC and AITD. Apparently, genetic variants known to increase the AITD risk might affect the clinical course of PBC. On the other hand, AITD per se does not seem to significantly influence the natural history of PBC.Abbreviations: AITD: autoimmune thyroid disease; ALP: alkaline phosphatase; ALT: alanine transaminase; AMA: anti-mitochondrial autoantibodies; AST: aspartate aminotransferase; DM1: diabetes mellitus type 1; ELISA: enzyme-linked immunosorbent assay method; GGT: gamma-glutamyl transpeptidase; GD: Graves’ disease; GWAS: genome-wide association studies; HT: Hashimoto thyroiditis; HWE: Hardy-Weinberg equilibrium; Lyp: lymphoid-specific protein tyrosine phosphatase non-receptor type 22; OR: odds ratio; PBC: primary biliary cholangitis; RA: rheumatoid arthritis; SLE: systemic lupus erythematosus; TPOAb: thyroid peroxidase antibody; UDCA: ursodeoxycholic acid.

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“…Several reports indicate that the incidence of coexisting PBC in patients with SLE ranges from 0% to 2.7% [3,7,8,10,11,13,19,26,37]. The frequency of PBC in patients with concomitant SLE who have abnormal levels of liver enzymes or liver dysfunction reportedly is 0%-7.5% [3,8,10,11,13].…”

Section: Pbc In Patients With Slementioning

confidence: 99%

“…Large multicenter studies of mortality in patients with SLE have revealed that liver diseases do not influence morbidity or mortality [18]. One of the possible explanations for this may be that end-stage liver diseases with concomitant SLE are uncommon [4,5,11,19]. However, the complications of chronic liver diseases such as viral hepatitis caused by Hepatitis B Virus (HBV) and Hepatitis C Virus (HCV), autoimmune liver diseases, and nonalcoholic steatohepatitis in patients with SLE may exacerbate Liver Cirrhosis (LC) and liver cancer.…”

Section: Liver Dysfunction In Patients With Slementioning

confidence: 99%

Liver Complications Associated with Systemic Lupus Erythematosus

Shizuma

2015

Rheumatology (Sunnyvale)

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Although liver dysfunction is not considered the main organ pathology or prognostic factor in patients with Systemic Lupus Erythematosus (SLE), it is not uncommon during the course of SLE. Liver complications in patients with SLE may be caused by lupus hepatitis (SLE-related hepatitis); autoimmune liver diseases, such as Autoimmune Hepatitis (AIH) and Primary Biliary Cirrhosis (PBC); viral hepatitis; and drug-induced liver injury. Here, liver complications in patients with SLE are reviewed.

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Connective tissue diseases in primary biliary cirrhosis: A population-based cohort study

Abstract: Besides the common liver manifestation of PBC, systemic involvement and overlaps with other CTDs are not infrequent in Chinese patients. When overlapping with other CTDs, PBC patients manifested some special clinical and laboratory features which may have effect on the prognosis.

Cited by 68 publications

References 0 publications

Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Primary Biliary Cirrhosis – Autoimmune Hepatitis Overlap Syndrome Associated with Dermatomyositis, Autoimmune Thyroiditis and Antiphospholipid Syndrome

Genetic Risk Factors for Autoimmune Thyroid Disease might Affect the Susceptibility to and Modulate the Progression of Primary Biliary Cholangitis

Liver Complications Associated with Systemic Lupus Erythematosus

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