J Rheum Dis.  2014 Dec;21(6):322-325. 10.4078/jrd.2014.21.6.322.

Successful Treatment of Primary Biliary Cirrhosis with Adalimumab in a Patient with Overlap Syndrome

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, Gachon University Gil Medical Center, Incheon, Korea. hjchoi@gilhospital.com

Abstract

Primary biliary cirrhosis (PBC) is occasionally developed in patients with rheumatic diseases, such as systemic sclerosis or Sjogren's syndrome. However, there are a few reports of overlap syndrome with PBC. The authors report a case of a 36 year-old female with PBC and overlap syndrome. Systemic sclerosis was diagnosed in 2007, and rheumatoid arthritis in 2010. Adalimumab stopped because of her pregnancy plan in January 2012. One month after delivery, she felt increased hand joint pain and fatigue. Laboratory findings were as follows: elevated AST, ALT, ALP, r-GTP and positive anti-mitochondrial antibody. Histology of a liver biopsy revealed moderate porto-periportal and mild lobular inflammation with bile duct inflammation, which was consistent with PBC. She was treated with prednisolone and UDCA (urosodeoxycholic acid), but her disease was not controlled. From May 2013, she has been treated with adalimumab. Her arthritis was improved and liver function test normalized up until now.

Keyword

Primary biliary cirrhosis; Overlap syndrome; Rheumatoid arthritis; Systemic sclerosis; Adalimumab

MeSH Terms

Adalimumab
Arthralgia
Arthritis
Arthritis, Rheumatoid
Bile Ducts
Biopsy
Fatigue
Female
Glycogen Storage Disease Type VI
Hand
Humans
Inflammation
Liver
Liver Cirrhosis, Biliary*
Liver Function Tests
Prednisolone
Pregnancy
Rheumatic Diseases
Scleroderma, Systemic
Sjogren's Syndrome
Prednisolone

Figure

  • Figure 1. Skin thickening on face.

  • Figure 2. High resonance computed tomography revealed subpleural ground glass opacity and reticular densities in both lower lung fields.

  • Figure 3. There are multiple erosions and joint space narrowing on both intercarpal, radiocarpal and left metacaophalangeal joints.

  • Figure 4. Moderate porto-periportal and mild lobular inflammation with bile duct inflammation/damage, consistent with primary biliary cirrhosis (×400).


Reference

1. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005; 353:1261–73.
Article
2. Watt FE, James OF, Jones DE. Patterns of autoimmunity in primary biliary cirrhosis patients and their families: a population-based cohort study. QJM. 2004; 97:397–406.
Article
3. Assassi S, Fritzler MJ, Arnett FC, Norman GL, Shah KR, Gourh P, et al. Primary biliary cirrhosis (PBC), PBC autoantibodies, and hepatic parameter abnormalities in a large population of systemic sclerosis patients. J Rheumatol. 2009; 36:2250–6.
Article
4. Maekawa S, Yano E, Shintani S. A case of rheumatoid arthritis associated with progressive systemic sclerosis and primary biliary cirrhosis in the presence of various autoantibodies. Ryumachi. 1992; 32:515–21.
5. Larrea E, Garcia N, Qian C, Civeira MP, Prieto J. Enhanced expression of TNFa in patients with primary biliary cirrhosis. Int Hepatol Commun. 1994; 2:6–13.
6. Neuman M, Angulo P, Malkiewicz I, Jorgensen R, Shear N, Dickson ER, et al. Tumor necrosis factor-alpha and transforming growth factor-beta reflect severity of liver damage in primary biliary cirrhosis. J Gastroenterol Hepatol. 2002; 17:196–202.
7. Sherlock S, Scheuer PJ. The presentation and diagnosis of 100 patients with primary biliary cirrhosis. N Engl J Med. 1973; 289:674–8.
Article
8. Polido-Pereira J, Rodrigues AM, Canhão H, Saraiva F, da Silva JA, Fonseca JE. Primary biliary cirrhosis in a rheumatoid arthritis patient treated with rituximab, a case-based review. Clin Rheumatol. 2012; 31:385–9.
Article
9. Spadaro A, Scrivo R, Riccieri V, Valesini G. Effect of tumor necrosis factor alpha antagonists in a patient with rheumatoid arthritis and primary biliary cirrhosis. Joint Bone Spine. 2008; 75:87–9.
Article
10. Ogata A, Terabe F, Nakanishi K, Kawai M, Kuwahara Y, Hirano T, et al. Etanercept improved primary biliary cirrhosis associated with rheumatoid arthritis. Joint Bone Spine. 2009; 76:105–7.
Article
11. Kubo S, Iwata S, Saito K, Tanaka Y. Successful treatment of primary biliary cirrhosis with etanercept in a patient with rheumatoid arthritis. Joint Bone Spine. 2011; 78:535–6.
Article
12. Bernstein RM, Callender ME, Neuberger JM, Hughes GR, Williams R. Anticentromere antibody in primary biliary cirrhosis. Ann Rheum Dis. 1982; 41:612–4.
Article
13. Liberal R, Grant CR, Sakkas L, Bizzaro N, Bogdanos DP. Diagnostic and clinical significance of anti-centromere antibodies in primary biliary cirrhosis. Clin Res Hepatol Gastroenterol. 2013; 37:572–85.
Article
14. Gupta RC, Seibold JR, Krishnan MR, Steigerwald JC. Precipitating autoantibodies to mitochondrial proteins in progressive systemic sclerosis. Clin Exp Immunol. 1984; 58:68–76.
15. Rigamonti C, Shand LM, Feudjo M, Bunn CC, Black CM, Denton CP, et al. Clinical features and prognosis of primary biliary cirrhosis associated with systemic sclerosis. Gut. 2006; 55:388–94.
Article
Full Text Links
  • JRD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr