Prognostic implications of extra-hepatic clinical manifestations, autoimmunity and microscopic nail capillaroscopy in patients with primary biliary cirrhosis
| Autor: | María José Amengual-Guedan, B. Marí-Alfonso, Mercè Vergara-Gómez, Carles Tolosa-Vilella, Esther Jove-Buxeda, Carmen Pilar Simeón-Aznar, Juan Oliva-Morera, V. Fonollosa-Pla |
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| Rok vydání: | 2016 |
| Předmět: |
Adult
Male medicine.medical_specialty Pathology Physical examination medicine.disease_cause behavioral disciplines and activities Gastroenterology Autoimmune Diseases Microscopic Angioscopy Autoimmunity Systemic autoimmune disease 03 medical and health sciences 0302 clinical medicine Primary biliary cirrhosis Internal medicine mental disorders Prevalence medicine Humans In patient skin and connective tissue diseases Aged Retrospective Studies Aged 80 and over 030203 arthritis & rheumatology Anamnesis Scleroderma Systemic integumentary system medicine.diagnostic_test Liver Cirrhosis Biliary business.industry Autoantibody Middle Aged Prognosis medicine.disease digestive system diseases Cohort Female 030211 gastroenterology & hepatology business |
| Zdroj: | Medicina Clínica (English Edition). 146:8-15 |
| ISSN: | 2387-0206 |
| Popis: | Primary biliary cirrhosis (PBC) is associated to any systemic autoimmune disease (SAD), in particular systemic sclerosis (SSc). To investigate the prevalence of SAD in a cohort of patients with PBC, specifically the prevalence of SSc and its clinical subtypes, and determining the clinical and biological profile of patients with associated PBC and SSc.Observational study of 62 patients with PBC following a protocol that included an anamnesis and physical examination to detect the presence of SAD as well as a nailfold capillaroscopy and an immunological study with specific SSc autoantibodies. A comparative analysis was conducted between patients with isolated PBC and patients with PBC and an associated SAD.SAD was associated to PBC in 22 patients (35,4%), and SSc was the most frequent illness, identified in 13 cases (21%). Five patients (8%) without previous diagnosis of SAD fulfilled pre-scleroderma criteria, according to LeRoy and Medsger criteria. The presence of anticentromere antibodies (54,5% vs. 5%, P.001) was the unique immunological determination identified more frequently in patients with PBC-SAD. The SSc suggestive capillary pattern was visualized in 11 patients (20,4%), mainly the slow pattern. No factors associated with greater morbi-mortality were identified in the PBC-SAD group.It does exist a subgroup of patients with PBC and clinical-biological features suggestive of an SAD, which advise a protocolized study to detect early the association to an SAD. |
| Databáze: | OpenAIRE |
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