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

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

• Yoon BY
• Song HY
• Lee JH
• Kim KA

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.

Keyword

Primary biliary cirrhosis; Systemic sclerosis; Autoimmune hemolytic anemia; Systemic lupus erythematosus

MeSH Terms

Anemia, Hemolytic, Autoimmune
Antibodies, Antinuclear
Antibodies, Antiphospholipid
Autoimmune Diseases
Bile Ducts, Intrahepatic
Dyspnea
Fatigue
Female
Humans
Hypothyroidism
Inflammation
Jaundice
Liver
Liver Cirrhosis, Biliary
Lupus Erythematosus, Systemic
Methylprednisolone
Middle Aged
Scleroderma, Systemic
Sjogren's Syndrome
Ursodeoxycholic Acid
Antibodies, Antinuclear
Antibodies, Antiphospholipid
Methylprednisolone
Ursodeoxycholic Acid

Figure

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  Fig. 1. Spherocytosis and polychromasia are shown on the peripheral blood smear (Wright-Giemsa stain, ×400).

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  Fig. 2. Bone marrow aspiration smear (Wright-Giemsa stain, ×400) (A) & biopsy (H-E stain, ×400) (B) Revealed hypercellularity with normoblastic hyperplasia.

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  Fig. 3. The endoscopic findings show esophageal varix (A) and fundal varices (B) in the stomach.

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  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.

Reference

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