Clin Rheumatol DOI 10.1007/s10067-010-1390-x
CASE REPORT
Diffuse systemic sclerosis and autoimmune hepatitis: a unique association Carlos Ewerton Maia Rodrigues & Cláudia Lobato Borges & Jozélio Freire de Carvalho
Received: 3 November 2009 / Revised: 8 January 2010 / Accepted: 13 January 2010 # Clinical Rheumatology 2010
Abstract Autoimmune hepatitis (AIH) is a chronic hepatitis of unknown etiology characterized by continuing hepatocellular necrosis and inflammation that afflicts 100,000 to 200,000 persons in the United States. It is a rare manifestation of systemic sclerosis. Only about nine reports of this association have been previously reported in the literature. Importantly, all cases had the limited clinical form of systemic. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs. We also briefly review the literature regarding this issue. Keywords Autoimmune hepatitis . Hepatitis . Scleroderma . Systemic sclerosis
Introduction Systemic sclerosis (SSc) is a chronic autoimmune disease characterized by fibrosis of the skin and internal organs, especially the gastrointestinal tract, occurring in as many as
50% to 90% of patients [1]. The major involved organ of this system is the esophagus; however, the other gastrointestinal organs have also been found to be involved. In a number of cases, the liver may be attacked by scleroderma. Primary biliary cirrhosis is a cholestatic liver disorder that is frequently observed in scleroderma patients [2]. Autoimmune hepatitis has been reported in patients with various diseases including insulin-dependent diabetes, vitiligo, glomerulonefritis, and autoimmune hemolytic anemia [3]. However, autoimmune hepatitis related to connective tissue disorders has been described as occurring rarely, for example, during the course of systemic lupus erythematosus, Sjögren’s syndrome, and mixed connective tissue diseases [2]. Regarding autoimmune hepatitis (AIH), only nine cases of systemic sclerosis patients with this liver disorder have been previously reported in the literature. Importantly, all these cases had the limited clinical form of systemic sclerosis [2, 4–10]. The authors describe herein the first report of a patient with diffuse systemic sclerosis who was diagnosed with AIH with a positive antimitochondrial antibody and had an excellent response to immunosuppressive drugs.
Case report JF Carvalho received a grant from Federico Foundation. C. E. M. Rodrigues : C. L. Borges : J. F. de Carvalho Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil J. F. de Carvalho (*) Disciplina de Reumatologia da Faculdade de Medicina da USP, Av. Dr. Arnaldo, 455, 3º andar, sala 3190, Cerqueira César, São Paulo, São Paulo, Brazil 01246-903 e-mail:
[email protected]
We describe the case of a 47-year-old female patient with diffuse systemic sclerosis, diagnosed 14 years ago and characterized by proximal and distal skin thickness, Raynaud’s phenomenon, polyarthralgia, esophageal, and positive anticentromere antibodies. Secondary Sjögren’s syndrome was also diagnosed based on xerophthalmia, xerostomia, bilateral Schirmer’s test
