A Case of Primary Biliary Cirrhosis in Association with an Overlap Syndrome of Systemic Sclerosis and Systemic Lupus Erythematosus.
10.4078/jkra.2009.16.4.338
- Author:
Bo Young YOON
1
;
Hwa Young SONG
;
Ju Hyun LEE
;
Kyung Ah KIM
Author Information
1. Department of Internal Medicine, Inje University College of Medicine, Goyang, Korea. byyoon@paik.ac.kr
- Publication Type:Case Report
- Keywords:
Primary biliary cirrhosis;
Systemic sclerosis;
Autoimmune hemolytic anemia;
Systemic lupus erythematosus
- MeSH:
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
- From:The Journal of the Korean Rheumatism Association
2009;16(4):338-343
- CountryRepublic of Korea
- Language:Korean
-
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.
