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

• Published in: Clinical rheumatology 2012-02, Vol.31 (2), p.385-389
• Main Authors: Polido-Pereira, Joaquim, Rodrigues, Ana Maria, Canhão, Helena, Saraiva, Fernando, da Silva, José Alberto Pereira, Fonseca, João Eurico
• Format: Article
• Language: English
• Subjects: Antibodies, Monoclonal, Murine-Derived - therapeutic use; Antinuclear antibodies; Antirheumatic Agents - therapeutic use; Arthritis, Rheumatoid - complications; Arthritis, Rheumatoid - drug therapy; Autoimmune diseases; Bile duct; Biopsy; Case Based Review; Case reports; Cholestasis; Cirrhosis; Clinical aspects; Drugs; etanercept; Female; Fibrosis; Fractures; Humans; Inflammatory diseases; Injuries; Liver Cirrhosis, Biliary - complications; Liver Cirrhosis, Biliary - surgery; Liver diseases; Medicine; Medicine & Public Health; Middle Aged; Monoclonal antibodies; Prednisolone; primary biliary cirrhosis; Reviews; Rheumatism; Rheumatoid arthritis; Rheumatoid factor; Rheumatology; Rib; Rituximab; Salivary gland; Sjogren's syndrome; Steatorrhea; Treatment Outcome
• ISSN: 0770-3198; 1434-9949
• DOI: 10.1007/s10067-011-1879-y

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.