A 33-Year-Old Woman with an Autoimmune Syndrome

A comparative study of antibody expressions in primary biliary cirrhosis and autoimmune cholangitis using phage display

“…PBC was diagnosed according to the description by Kaplan. 21 AIC was diagnosed according to published descriptions, [3][4][5] namely a chronic hepatobiliary disease with an obstructive pattern of biochemical functional indices, no evidence of extrahepatic biliary obstruction, histopathologic features of intrahepatic inflammatory biliary ductular lesions, and a serologic test pattern of negativity for AMA, and positivity for ANA. By indirect immunofluorescence, the patients with PBC were positive for AMA and those with AIC were negative for AMA by both immunofluorescence and immunoblot, but positive for ANA, particularly anti-Sp-100.…”

“…A variant syndrome, autoimmune cholangitis (AIC), has clinical and histopathologic features of PBC, but AMA are lacking and ANA are present with specificities similar to those seen in ANA-positive cases of PBC. 2 An earlier opinion that AIC might be a distinct disease [3][4][5] has given way to aligning it with PBC, 2,6-11 but with recommendations that its status be protected pending explanation of serologic and other differences. 12 The provocation of either PBC or AIC is unknown.…”

Long term outcome and response to therapy of primary biliary cirrhosis—autoimmune hepatitis overlap syndrome

“…However, patients presenting with clinical, biochemical, serological, and/or histological features reminiscent of both diseases, either simultaneously or consecutively, have been repeatedly recognised [1][2][3][4][5]. The term overlap syndrome is used to describe these settings [6][7][8][9][10].…”

Diagnosing clinical subsets of autoimmune liver diseases based on a multivariable model

“…19 Similarly, no histological features, including chronic non-suppurative destructive cholangitis, are 100% specific for each type of AILD. 21,22 We found seven independent explanatory variables by multiple logistic regression analysis with which we could predict the diagnosis of AILD. Interestingly, the seven factors were common in both AIH and PBC, indicating that both physicians and pathologists diagnose AIH and PBC by using the same diagnostic factors.…”