Autoimmune serology of primary sclerosing cholangitis

Zauli, Daniela; Grassi, Alberto; Cassani, F.; Ballardini, G.; Bortolotti, Roberto; Muratori, L.; Fusconi, M.; Bianchi, F.B.

doi:10.1016/s1590-8658(01)80099-2

Cited by 6 publications

(6 citation statements)

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“…Further studies of anti-TBB5 are necessary to characterise the clinical and pathogenetic relevance of these findings. Other nuclear antigens have also been [137] 72 IIF (33/46) - 9 Hardarson et al [145] 69 [99] 67 IIF (46/69) 1:40 Bansi et al [146] 66 proposed as nuclear targets of pANCA in AIH and UC, notably the high mobility group (HMG) non-histone chromosomal proteins HMG1 and HMG2 [66][67][68] and Histone H1 [69] . These have not been studied in patients with PSC.…”

Section: Hov Jr Et Al Autoantibodies In Pscmentioning

confidence: 99%

Autoantibodies in primary sclerosing cholangitis

Hov¹,

Boberg²,

Karlsen³

2008

WJG

150

100

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The aetiology of primary sclerosing cholangitis (PSC) is not known and controversy exists as to whether PSC should be denominated an autoimmune disease. A large number of autoantibodies have been detected in PSC patients, but the specificity of these antibodies is generally low, and the frequencies vary largely between different studies. The presence of autoantibodies in PSC may be the result of a nonspecific dysregulation of the immune system, but the literature in PSC points to the possible presence of specific antibody targets in the biliary epithelium and in neutrophil granulocytes. The present review aims to give an overview of the studies of autoantibodies in PSC, with a particular emphasis on the prevalence, clinical relevance and possible pathogenetic importance of each individual marker.

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Section: Hov Jr Et Al Autoantibodies In Pscmentioning

confidence: 99%

Autoantibodies in primary sclerosing cholangitis

Hov¹,

Boberg²,

Karlsen³

2008

WJG

150

100

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“…[26–11] Clinical and serological findings of concomitant autoimmunity have been described in 25 to 70% of PSC patients and in up to 20% of MG patients, but a coexistence of these two conditions, as seen in our patient, has never been documented. [25–11]…”

Section: Discussionmentioning

confidence: 78%

“…[24] The hypothesis of PSC as an autoimmune disease is supported by the high frequency of inflammatory bowel disease in PSC patients, the increased incidence of other coexisting autoimmune diseases, and the presence of multiple autoantibodies. [2–49–11] No antibody is specific for PSC. [1011] As many as 90% of patients with PSC have at least one detectable autoantibody; however, the presence of multiple antibodies does not correlate with disease activity.…”

Section: Discussionmentioning

confidence: 99%

“…[2–49–11] No antibody is specific for PSC. [1011] As many as 90% of patients with PSC have at least one detectable autoantibody; however, the presence of multiple antibodies does not correlate with disease activity. [1011] ANA and SMA antibodies are found in up to 60% of patients with PSC[1011] The main autoimmune disease associated with PSC is inflammatory bowel disease, especially ulcerative colitis, which occurs in up to 75% of PSC patients, but several other autoimmune disorders have been reported to be associated in up to 25% of cases.…”

Section: Discussionmentioning

confidence: 99%

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Coexistence of primary sclerosing cholangitis in a patient with myasthenia gravis

Lorenzoni

Scola

Kay

et al. 2011

Ann Indian Acad Neurol

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Myasthenia gravis (MG) is an immune-mediated disease that compromises the postsynaptic membrane of the neuromuscular junction. Primary sclerosing cholangitis (PSC) is considered an immune-mediated cholestatic liver disease. Both MG and PSC include an autoimmune pathogenesis, so there is some evidence that patients with MG or PSC have a higher risk of developing autoantibodies and other immune disorders than normal controls, but the coexistence of these two disorders has never been documented. We report a 40-year-old woman who presented with MG when she was 20 years old and developed PSC 20 years after a thymectomy. Liver biochemistry revealed cholestasis. Magnetic resonance imaging showed multifocal strictures and beads involving the intrahepatic bile ducts. A liver biopsy confirmed sclerosing cholangitis. Serological analysis demonstrated positive autoantibodies (Anti-nuclear antibodies, anti-smooth muscle antibodies). Repetitive stimulation had a decremental response, and antibodies to acetylcholine receptors were detectable. To our knowledge, this is the first case of PSC in a patient with MG. The main characteristics of both MG and PSC combination are discussed.

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“…Other autoantibodies such as ANA, SMA and anti-lactoferrin antibodies are often reported in PSC (63%, 61% and 37%, respectively) but they do not seem to have clinical relevance (Zauli et al, 2001).…”

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confidence: 99%