A Case of Primary Biliary Cirrhosis in Association with an Overlap Syndrome of Systemic Sclerosis and Systemic Lupus Erythematosus

Bo Young Yoon; Hwa Young Song; Ju Hyun Lee; Kyung Ah Kim

doi:10.4078/jkra.2009.16.4.338

Journal List > J Korean Rheum Assoc > v.16(4) > 1003697

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Yoon, Song, Lee, and Kim: A Case of Primary Biliary Cirrhosis in Association with an Overlap Syndrome of Systemic Sclerosis and Systemic Lupus Erythematosus

Original Article

J Korean Rheum Assoc 2009;16(4):338-343.

Published online: 20 January 2009

DOI: https://doi.org/10.4078/jkra.2009.16.4.338

A Case of Primary Biliary Cirrhosis in Association with an Overlap Syndrome of Systemic Sclerosis and Systemic Lupus Erythematosus

Bo Young Yoon, Hwa Young Song, Ju Hyun Lee, Kyung Ah Kim

Department of Internal Medicine, Inje University College of Medicine, Goyang, Korea

Received 26 August 2009 Revised 10 September 2009 Accepted 10 September 2009

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Primary biliary cirrhosis (PBC) is a slowly progressive autoimmune disease of the liver that is related to anti-mitochondria antibody and the disease is characterized by portal inflammation and immune-mediated destruction of the intrahepatic bile ducts. Several autoimmune diseases, such as hypothyroidism, Sjogren syndrome and systemic sclerosis (SSc), occur with increased frequency in patients with PBC. However, there are a few reports of a possible connection between systemic lupus erythematosus (SLE) or autoimmune hemolytic anemia (AIHA) and PBC. A 52-year-old female was admitted with fatigue and dyspnea that she had suffered with for the past month. She had suffered from jaundice for 2 weeks before admission. Many of the clinical manifestations and laboratory findings suggested the diagnosis of PBC with SSc of the limited type and AIHA. She was treated with methylprednisolone pulse therapy and ursodeoxycholic acid. We consequently diagnosed her as having SLE, as she satisfied the 4 relative diagnostic criteria-arthritis, AIHA, positive antinuclear antibody and positive antiphospholipid antibodies.

Keywords: Primary biliary cirrhosis, Systemic sclerosis, Autoimmune hemolytic anemia, Systemic lupus erythematosus

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Fig. 1.

Spherocytosis and polychromasia are shown on the peripheral blood smear (Wright-Giemsa stain, ×400).

Fig. 2.

Bone marrow aspiration smear (Wright-Giemsa stain, ×400) (A) & biopsy (H-E stain, ×400) (B) Revealed hypercellularity with normoblastic hyperplasia.

Fig. 3.

The endoscopic findings show esophageal varix (A) and fundal varices (B) in the stomach.

Fig. 4.

The abdominal CT scan shows characteristics of early liver cirrhosis, including enlarged left and caudate lobes and the minimal surface nodularity of the liver.

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