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

• Han JY
• Lee KH
• Seo MR
• Choi HJ

• KMID: 2222938
• DOI: http://doi.org/10.4078/jrd.2014.21.6.322

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

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  Figure 1. Skin thickening on face.

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

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

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

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