{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,28]],"date-time":"2026-02-28T03:27:15Z","timestamp":1772249235604,"version":"3.50.1"},"reference-count":38,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":11566,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[1984,1]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Total complement activity was normal in 18 patients with primary biliary cirrhosis using two hemolytic assays capable of distinguishing between defects in classical and alternative pathways. Activation of the classical pathway was demonstrated in all patients by formation of complexes between C1r, C1s, and C1 inactivator. Large amounts of free C1q, not in complex with C1r and C1s, were demonstrated in the majority of patient sera. Furthermore, C4 levels were within the normal range or slightly subnormal. No evidence for alternative pathway activation was found. Increased mean levels of several complement components, in particular C1 inactivator, C2, C3, factor B, factor H, were noted. A significant correlation between these complement factors, derived mainly from the liver, and ceruloplasmin suggests that this elevation might be secondary to cholestasis. In contrast, no significant correlations with levels of early reacting acute phase reactants, immunoglobulins, or circulating immune complex-like material were observed. It is concluded that activation of the complement system by the classical pathway is common in patients with primary biliary cirrhosis.<\/jats:p>\n          <\/jats:sec>","DOI":"10.1002\/hep.1840040102","type":"journal-article","created":{"date-parts":[[2008,8,22]],"date-time":"2008-08-22T13:52:03Z","timestamp":1219413123000},"page":"9-14","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":35,"title":["Complement Components and Activation in Primary Biliary Cirrhosis"],"prefix":"10.1097","volume":"4","author":[{"given":"Stefan","family":"Lindgren","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna-Brita","family":"Laurell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sten","family":"Eriksson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"10.1002\/hep.1840040102-BIB1","doi-asserted-by":"crossref","first-page":"322","DOI":"10.1055\/s-2008-1040735","article-title":"The role of the immune response in the pathogenesis of primary biliary cirrhosis","volume":"1","author":"James","year":"1981","journal-title":"Semin Liver Disease"},{"key":"10.1002\/hep.1840040102-BIB2","doi-asserted-by":"crossref","first-page":"451","DOI":"10.1136\/gut.14.6.451","article-title":"Serum complement in chronic liver disease","volume":"14","author":"Potter","year":"1973","journal-title":"Gut"},{"key":"10.1002\/hep.1840040102-BIB3","doi-asserted-by":"crossref","first-page":"371","DOI":"10.1159\/000198223","article-title":"Profiles of serum complement in patients with hepatobiliary diseases","volume":"18","author":"Potter","year":"1978","journal-title":"Digestion"},{"key":"10.1002\/hep.1840040102-BIB4","first-page":"509","article-title":"Circulating conversion products of C3 in liver disease","volume":"14","author":"Teisberg","year":"1973","journal-title":"Clin Exp Immunol"},{"key":"10.1002\/hep.1840040102-BIB5","first-page":"427","article-title":"Hypercatabolism of the third component of complement in patients with primary biliary cirrhosis","volume":"88","author":"Potter","year":"1976","journal-title":"J Lab Clin Med"},{"key":"10.1002\/hep.1840040102-BIB6","doi-asserted-by":"crossref","first-page":"1034","DOI":"10.1016\/0016-5085(80)90788-X","article-title":"Complement metabolism in chronic liver disease: catabolism of Clq in chronic active liver disease and primary biliary cirrhosis","volume":"78","author":"Potter","year":"1980","journal-title":"Gastroenterology"},{"key":"10.1002\/hep.1840040102-BIB7","doi-asserted-by":"crossref","first-page":"233","DOI":"10.1056\/NEJM197802022980502","article-title":"Circulating immune complexes and complement activation in primary biliary cirrhosis","volume":"298","author":"Wands","year":"1978","journal-title":"N Engl J Med"},{"key":"10.1002\/hep.1840040102-BIB8","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1016\/S0140-6736(77)92666-6","article-title":"Is primary biliary cirrhosis an immune complex disease?","volume":"2","author":"Thomas","year":"1977","journal-title":"Lancet"},{"key":"10.1002\/hep.1840040102-BIB9","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1084\/jem.156.3.756","article-title":"Modulation of human lymphocyte function by C3a and C3a(70-77)","volume":"156","author":"Payan","year":"1982","journal-title":"J Exp Med"},{"key":"10.1002\/hep.1840040102-BIB10","doi-asserted-by":"crossref","first-page":"1069","DOI":"10.1172\/JCI109212","article-title":"Receptor specific clearance by the reticuloendothelial system in chronic liver diseases; 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