{"corpus_id":24408004,"paper_sha":"4bbc5ec42f97c4787ee3096288210e420984d34b","doi":"10.1016/j.semarthrit.2017.10.004","arxiv_id":null,"pmid":29246416,"pmcid":null,"mag_id":2764116959,"dblp_id":null,"acl_id":null,"title":"Hepatobiliary involvement in systemic sclerosis and the cutaneous subsets: Characteristics and survival of patients from the Spanish RESCLE Registry.","year":2017,"publication_date":"2017-10-06","venue":"Seminars in Arthritis & Rheumatism","journal":{"name":"Seminars in arthritis and rheumatism","pages":"\n          849-857\n        ","volume":"47 6"},"journal_issn":null,"journal_title":null,"publication_types":["JournalArticle"],"pubmed_pub_types":["Journal Article","Research Support, Non-U.S. Gov't"],"s2_fields_of_study":["Medicine"],"reference_count":61,"citation_count":23,"influential_citation_count":0,"is_open_access":false,"arxiv_categories":null,"arxiv_license":null,"arxiv_journal_ref":null,"mesh_headings":[{"d":"Adult","mj":false,"ui":"D000328"},{"d":"Aged","mj":false,"ui":"D000368"},{"d":"Cholangitis","mj":false,"qs":[{"q":"etiology","mj":true,"ui":"Q000209"},{"q":"mortality","mj":false,"ui":"Q000401"}],"ui":"D002761"},{"d":"Female","mj":false,"ui":"D005260"},{"d":"Hepatitis, Autoimmune","mj":false,"qs":[{"q":"etiology","mj":true,"ui":"Q000209"},{"q":"mortality","mj":false,"ui":"Q000401"}],"ui":"D019693"},{"d":"Humans","mj":false,"ui":"D006801"},{"d":"Male","mj":false,"ui":"D008297"},{"d":"Middle Aged","mj":false,"ui":"D008875"},{"d":"Prognosis","mj":false,"ui":"D011379"},{"d":"Registries","mj":false,"ui":"D012042"},{"d":"Scleroderma, Systemic","mj":false,"qs":[{"q":"complications","mj":true,"ui":"Q000150"},{"q":"mortality","mj":false,"ui":"Q000401"}],"ui":"D012595"},{"d":"Sjogren's Syndrome","mj":false,"qs":[{"q":"complications","mj":false,"ui":"Q000150"},{"q":"mortality","mj":false,"ui":"Q000401"}],"ui":"D012859"},{"d":"Spain","mj":false,"ui":"D013030"},{"d":"Survival Rate","mj":false,"ui":"D015996"}],"chemicals":null,"comments_corrections":null,"source_flags":5,"s2_open_access_pdf_url":null,"s2_open_access_landing_url":null,"s2_open_access_license":null,"s2_open_access_status":null,"pmc_open_access_pdf_url":null,"pmc_open_access_landing_url":null,"pmc_open_access_license":null,"pmc_open_access_status":null,"unpaywall_open_access_pdf_url":null,"unpaywall_open_access_landing_url":null,"unpaywall_open_access_license":null,"unpaywall_open_access_status":null,"abstract":"OBJECTIVE\nTo assess the prevalence and causes of hepatobiliary involvement (HBI) in systemic sclerosis (SSc), to investigate the clinical characteristics and prognosis of SSc patients with HBI (SSc-HBI) and without HBI (SSc-non-HBI), and to compare both groups according to the cutaneous SSc subsets.\n\n\nMETHODS\nIn all, 1572 SSc patients were collected in the RESCLE registry up to January 2015, and all hepatobiliary disturbances were recorded. We investigated the HBI-related characteristics and survival from the entire SSc cohort and according to the following cutaneous subsets: diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc sine scleroderma (ssSSc).\n\n\nRESULTS\nOut of 1572, 118 (7.5%) patients had HBI. Primary biliary cholangitis (PBC) was largely the main cause (n = 67, 4.3%), followed by autoimmune hepatitis (n = 19, 1.2%), and anti-mitochondrial negative PBC (n = 6, 0.4%). Other causes of HBI were as follows: secondary liver diseases (n = 11, 0.7%), SSc-related HBI (n = 7, 0.4%), nodular regenerative hyperplasia (n = 3, 0.2%), liver cirrhosis (n = 3, 0.2%), and HBI of unknown origin (n = 2, 0.1%). In multivariate analysis, HBI was independently associated to lesser risk of dcSSc (5.1% vs. 24.4%), and higher frequency of calcinosis (26% vs. 18%), left ventricular diastolic dysfunction (46% vs. 27%), sicca syndrome (51% vs. 29%), and anti-centromere antibodies (ACA, 73% vs. 44%). According to the cutaneous subsets, HBI was associated (1) in lcSSc, to longer time from SSc onset to diagnosis (10.8 ± 12.5 vs. 7.2 ± 9.3 years), sicca syndrome (54% vs. 33%), and ACA (80% vs. 56%); (2) in ssSSc, to sicca syndrome (44% vs. 19%), and (3) in dcSSc, no associations were found. HBI was the cause of death in 2.3% patients but the cumulative survival according to the presence or absence of HBI showed no differences.\n\n\nCONCLUSIONS\nHBI prevalence in SSc is 7.5% and dcSSc is the least involved subset. PBC is the main cause of HBI. Patients with SSc-HBI exhibited specific clinical and immunologic profile. Survival is similar for SSc patients with HBI.","claims":[{"public_id":"cl_7f79dc1984c475dd848e7ab05b615ab1","status":"active","text":"Among limited cutaneous systemic sclerosis, hepatobiliary involvement is associated with longer time from systemic sclerosis onset to diagnosis, sicca syndrome, and anti-centromere antibodies; among systemic sclerosis sine scleroderma, it is associated with sicca syndrome; and no associations are found in diffuse cutaneous systemic sclerosis.","confidence":0.93,"contributors":[{"id":1,"public_id":"12632b8b5f","public_label":"Anonymous (12632b8b5f)","roles":["extraction"],"url":"https://sah.borca.ai/u/12632b8b5f"}],"url":"https://sah.borca.ai/claims/cl_7f79dc1984c475dd848e7ab05b615ab1"},{"public_id":"cl_60cb177eec086c3853c026fb7c37ffe8","status":"active","text":"Hepatobiliary involvement is independently associated with a lower likelihood of diffuse cutaneous systemic sclerosis and with higher frequencies of calcinosis, left ventricular diastolic dysfunction, sicca syndrome, and anti-centromere antibodies.","confidence":0.94,"contributors":[{"id":1,"public_id":"12632b8b5f","public_label":"Anonymous (12632b8b5f)","roles":["extraction"],"url":"https://sah.borca.ai/u/12632b8b5f"}],"url":"https://sah.borca.ai/claims/cl_60cb177eec086c3853c026fb7c37ffe8"},{"public_id":"cl_f35966a395ad27d7c5fbc4a2d2a5582e","status":"active","text":"Hepatobiliary involvement occurs in 7.5% of patients with systemic sclerosis in the RESCLE registry.","confidence":0.99,"contributors":[{"id":1,"public_id":"12632b8b5f","public_label":"Anonymous (12632b8b5f)","roles":["extraction"],"url":"https://sah.borca.ai/u/12632b8b5f"}],"url":"https://sah.borca.ai/claims/cl_f35966a395ad27d7c5fbc4a2d2a5582e"},{"public_id":"cl_20df9d28246c0f9d4a59aab05909b3e7","status":"active","text":"Primary biliary cholangitis is the main cause of hepatobiliary involvement, followed by autoimmune hepatitis.","confidence":0.98,"contributors":[{"id":1,"public_id":"12632b8b5f","public_label":"Anonymous (12632b8b5f)","roles":["extraction"],"url":"https://sah.borca.ai/u/12632b8b5f"}],"url":"https://sah.borca.ai/claims/cl_20df9d28246c0f9d4a59aab05909b3e7"},{"public_id":"cl_a67966894dc703bf262c1ab49c6a84d9","status":"active","text":"Survival does not differ between patients with and without hepatobiliary involvement, although hepatobiliary involvement accounts for a small proportion of deaths.","confidence":0.91,"contributors":[{"id":1,"public_id":"12632b8b5f","public_label":"Anonymous (12632b8b5f)","roles":["extraction"],"url":"https://sah.borca.ai/u/12632b8b5f"}],"url":"https://sah.borca.ai/claims/cl_a67966894dc703bf262c1ab49c6a84d9"}],"concepts":[{"public_id":"co_19aead28180f7980200bf5a47b1d3a69","status":"active","name":"autoimmune hepatitis","description":"An autoimmune inflammatory liver 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