Case Report

The Journal of the Korean Rheumatism Association 2009; 16(4): 338-343

Published online December 30, 2009

© Korean College of Rheumatology

원발담도경화증에 병발된 전신경화증, 전신홍반루푸스 중복증후군 1예

윤보영ㆍ송화영ㆍ이주현ㆍ김경아

인제대학교 의과대학 내과학교실

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

Correspondence to : Bo Young Yoon

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

Article

Case Report

The Journal of the Korean Rheumatism Association 2009; 16(4): 338-343

Published online December 30, 2009

Copyright © Korean College of Rheumatology.

원발담도경화증에 병발된 전신경화증, 전신홍반루푸스 중복증후군 1예

윤보영ㆍ송화영ㆍ이주현ㆍ김경아

인제대학교 의과대학 내과학교실

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

Correspondence to:Bo Young Yoon

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

JRD
Jan 01, 2025 Vol.32 No.1, pp. 1~7
COVER PICTURE
Cumulative growth of rheumatology members and specialists (1980~2024). Cumulative distribution of the number of the (A) Korean College of Rheumatology members and (B) rheumatology specialists. (J Rheum Dis 2025;32:63-65)

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