{"corpus_id":24131897,"paper_sha":"a40c708fd32967f706e8a20de166e0bf3ecbc71e","doi":"10.3748/wjg.v19.i31.5131","arxiv_id":null,"pmid":23964148,"pmcid":"PMC3746386","mag_id":2128644179,"dblp_id":null,"acl_id":null,"title":"Connective tissue diseases in primary biliary cirrhosis: a population-based cohort study.","year":2013,"publication_date":"2013-08-21","venue":"World Journal of Gastroenterology","journal":{"name":"World journal of gastroenterology","pages":"\n          5131-7\n        ","volume":"19 31"},"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":52,"citation_count":73,"influential_citation_count":6,"is_open_access":false,"arxiv_categories":null,"arxiv_license":null,"arxiv_journal_ref":null,"mesh_headings":[{"d":"Chi-Square Distribution","mj":false,"ui":"D016009"},{"d":"China","mj":false,"qs":[{"q":"epidemiology","mj":false,"ui":"Q000453"}],"ui":"D002681"},{"d":"Connective Tissue Diseases","mj":false,"qs":[{"q":"diagnosis","mj":false,"ui":"Q000175"},{"q":"epidemiology","mj":true,"ui":"Q000453"}],"ui":"D003240"},{"d":"Humans","mj":false,"ui":"D006801"},{"d":"Incidence","mj":false,"ui":"D015994"},{"d":"Liver Cirrhosis, Biliary","mj":false,"qs":[{"q":"diagnosis","mj":false,"ui":"Q000175"},{"q":"epidemiology","mj":true,"ui":"Q000453"}],"ui":"D008105"},{"d":"Retrospective Studies","mj":false,"ui":"D012189"}],"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":"AIM\nTo establish the frequency and clinical features of connective tissue diseases (CTDs) in a cohort of Chinese patients with primary biliary cirrhosis (PBC).\n\n\nMETHODS\nThree-hundred and twenty-two Chinese PBC patients were screened for the presence of CTD, and the systemic involvement was assessed. The differences in clinical features and laboratory findings between PBC patients with and without CTD were documented. The diversity of incidence of CTDs in PBC of different countries and areas was discussed. For the comparison of normally distributed data, Student's t test was used, while non-parametric test (Wilcoxon test) for the non-normally distributed data and 2 × 2 χ(2) or Fisher's exact tests for the ratio.\n\n\nRESULTS\nOne-hundred and fifty (46.6%) PBC patients had one or more CTDs. The most common CTD was Sjögren's syndrome (SS, 121 cases, 36.2%). There were nine cases of systemic sclerosis (SSc, 2.8%), 12 of systemic lupus erythematosus (SLE, 3.7%), nine of rheumatoid arthritis (RA, 2.8%), and 10 of polymyositis (PM, 3.1%) in this cohort. Compared to patients with PBC only, the PBC + SS patients were more likely to have fever and elevated erythrocyte sedimentation rate (ESR), higher serum immunoglobulin G (IgG) levels and more frequent rheumatoid factor (RF) and interstitial lung disease (ILD) incidences; PBC + SSc patients had higher frequency of ILD; PBC + SLE patients had lower white blood cell (WBC) count, hemoglobin (Hb), platelet count, γ-glutamyl transpeptidase and immunoglobulin M levels, but higher frequency of renal involvement; PBC + RA patients had lower Hb, higher serum IgG, alkaline phosphatase, faster ESR and a higher ratio of RF positivity; PBC + PM patients had higher WBC count and a tendency towards myocardial involvement.\n\n\nCONCLUSION\nBesides 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.","claims":[{"public_id":"cl_230167ce368dcb65491a23424d863231","status":"active","text":"Forty-six point six percent of the 322 Chinese patients with primary biliary cirrhosis had one or more connective tissue diseases.","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_230167ce368dcb65491a23424d863231"},{"public_id":"cl_d65657d0b877c5f72befc3b1007e0d5e","status":"active","text":"Sjögren's syndrome was the most common connective tissue disease in the cohort, occurring in 36.2% of patients.","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_d65657d0b877c5f72befc3b1007e0d5e"},{"public_id":"cl_14db89c095391f23526c04fe61574f89","status":"active","text":"Specific overlap patterns were associated with distinct clinical and laboratory features, including fever, elevated erythrocyte sedimentation rate, higher IgG, rheumatoid factor positivity, interstitial lung disease, renal involvement, and myocardial involvement depending on the coexisting connective tissue disease.","confidence":0.96,"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_14db89c095391f23526c04fe61574f89"},{"public_id":"cl_77c68dfaa0729cd767f54cf146f084f3","status":"active","text":"Systemic involvement and overlap with other connective tissue diseases are not infrequent in Chinese patients with primary biliary cirrhosis and may affect prognosis.","confidence":0.92,"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_77c68dfaa0729cd767f54cf146f084f3"},{"public_id":"cl_61901de8be12c429e9909169a549cc0d","status":"active","text":"The cohort also included systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis, and polymyositis at lower frequencies.","confidence":0.95,"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_61901de8be12c429e9909169a549cc0d"}],"concepts":[{"public_id":"co_084236cf5a7989e1fda57da23d1f94a2","status":"active","name":"systemic sclerosis","description":"An autoimmune connective tissue disease marked by skin thickening and internal organ 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