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Primary biliary cirrhosis in a rheumatoid arthritis patient treated with rituximab, a case-based review

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Abstract

Primary biliary cirrhosis (PBC) is an autoimmune disease in which intrahepatic bile ducts are targeted by an immune-mediated injury. This disease tends to progress to fibrosis and cirrhosis with hepatic failure. The authors report a case of a 50-year-old rheumatoid arthritis (RA) patient, with erosions and seropositive for rheumatoid factor and anti-citrullinated peptide antibodies, with 18 years disease duration refractory to prednisolone and several disease-modifying antirheumatic drugs, either conventional or biological (adalimumab and etanercept). In April 2007, she started therapy with rituximab (RTX) with good European League Against Rheumatism response achieved 9 months later. In June 2008, she was admitted with intrahepatic cholestasis, steatorrhea, and spontaneous fractures of various ribs. After excluding cholelitiasis, as well as infectious and neoplastic diseases a liver biopsy was performed that was compatible with the diagnosis of PBC. The antinuclear antibodies (1/160) were positive as well as the antimitochondrial antibodies (1/640). Other antibodies were negative such as anti-SSA and anti-SSB. Afterwards, the patient started ursodesoxycholic acid 15 mg kg-1 day-1 with progressive improvement of cholestatic markers. A labial salivary gland biopsy was performed and showed findings compatible with the concomitant diagnosis of Sjögren’s syndrome. Based on this clinical report, a detailed review of the clinical aspects of PBC is presented as well as its association with other immune-mediated inflammatory diseases, particularly, with RA.

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Acknowledgments

The authors thank Dr. Ana Afonso and Dr. Maria Adelaide Milheiro from the Pathology Department of Hospital Curry Cabral, Lisbon, for the clinical discussion and for sharing the pathology images.

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Authors and Affiliations

  1. Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Lisbon, Portugal

    Joaquim Polido-Pereira, Ana Maria Rodrigues, Helena Canhão & João Eurico Fonseca

  2. Rheumatology Department, Centro Hospitalar de Lisboa Norte, EPE, Hospital de Santa Maria, Lisbon, Portugal

    Joaquim Polido-Pereira, Ana Maria Rodrigues, Helena Canhão, Fernando Saraiva, José Alberto Pereira da Silva & João Eurico Fonseca

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  1. Joaquim Polido-Pereira
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  2. Ana Maria Rodrigues
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  6. João Eurico Fonseca
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Correspondence to Joaquim Polido-Pereira.

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Polido-Pereira, J., Rodrigues, A.M., Canhão, H. et al. Primary biliary cirrhosis in a rheumatoid arthritis patient treated with rituximab, a case-based review. Clin Rheumatol 31, 385–389 (2012). https://doi.org/10.1007/s10067-011-1879-y

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  • DOI: https://doi.org/10.1007/s10067-011-1879-y

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