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학술연구/단체지원/교육 등 연구자 활동을 지속하도록 DBpia가 지원하고 있어요.
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논문 기본 정보
- 자료유형
- 학술저널
- 저자정보
- 발행연도
- 2020.1
- 수록면
- 69 - 73 (5page)
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Systemic sclerosis (SSc) is a chronic systemic disease of unknown etiology characterized by vasculopathy, excessive accumulation of extracellular matrix, and fibrosis of the skin and other internal organs. Although its etiology remains elusive, approximately one third of SSc patients presents with additional autoimmune disease, which suggests that an autoimmune mechanism is a major component of the underlying pathophysiology. On the other hand, primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two main autoimmune liver diseases. A 41-year-old female previously diagnosed with PBC/AIH overlap syndrome presented with multiple, painful brownish to erythematous firm patches on the hands, arms, axillae, neck, abdomen, and thighs. Laboratory work-up yielded positive results for anti-nuclear antibody, anti-Ro/Sjögren’s-syndrome-related antigen A autoantibodies, and perinuclear anti-neutrophil cytoplasmic antibodies while punch biopsy of her left hand showed characteristics that are consistent with scleroderma. Herein, we report the first case of a patient with diffuse cutaneous SSc and concurrent PBC/AIH overlap syndrome and suggest that this coexistence of multiple autoimmune diseases is not a coincidence but rather that a common autoimmune pathogenesis may exist.
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참고문헌 (18)
참고문헌 신청
Efe C / 2010 / The development of autoimmune hepatitis and primary biliary cirrhosis overlap syndrome during the course of connective tissue diseases : report of three cases and review of the literature / Dig Dis Sci 55:2417~2421
Toyoda M / 2009 / Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome concomitant with systemic sclerosis, immune thrombocytopenic purpura / Intern Med 48:2019~2023
West M / 2006 / The development of connective tissue diseases in patients with autoimmune hepatitis : a case series / Semin Arthritis Rheum 35:344~348
Lindor KD / 2009 / Primary biliary cirrhosis / Hepatology 50:291~308
Manns MP / 2010 / Diagnosis and management of autoimmune hepatitis / Hepatology 51:2193~2213
Toyoda M / 2009 / Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome concomitant with systemic sclerosis, immune thrombocytopenic purpura / Intern Med 48:2019~2023
West M / 2006 / The development of connective tissue diseases in patients with autoimmune hepatitis : a case series / Semin Arthritis Rheum 35:344~348
Lindor KD / 2009 / Primary biliary cirrhosis / Hepatology 50:291~308
Manns MP / 2010 / Diagnosis and management of autoimmune hepatitis / Hepatology 51:2193~2213
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