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Diffuse systemic sclerosis and autoimmune hepatitis: a unique association

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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.

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References

  1. Abu-Shakra M, Guillemin F, Lee P (1994) Gastrointestinal manifestations of systemic sclerosis. Semin Arthritis Rheum 24:29–39

    Article  CAS  PubMed  Google Scholar 

  2. Marie I, Levesque H, Tranvouez JL, François A, Riachi G, Cailleux N, Courtois H (2001) Autoimmune hepatitis and systemic sclerosis: a new overlap syndrome? Rheumatology (Oxford) 40:102–106

    Article  CAS  Google Scholar 

  3. McFarlane IG (1998) The relationship between autoimmune markers and different clinical syndromes in autoimmune hepatitis. Gut 42:599–602

    CAS  PubMed  Google Scholar 

  4. Khalifa M, Ben Jazia E, Hachfi W, Sriha B, Bahri F, Letaief A (2006) Autoimmune hepatitis and morphea: a rare association. Gastroenterol Clin Biol 30:917–918

    Article  PubMed  Google Scholar 

  5. West M, Jasin HE, Medhekar S (2006) The development of connective tissue diseases in patients with autoimmune hepatitis: a case series. Semin Arthritis Rheum 35:344–348

    Article  CAS  PubMed  Google Scholar 

  6. Narbutt J, Lesiak A, Kwiecień A, Sysa-Jedrzejowska A, Kuydowicz J (2005) Scleroderma-like lesions in the course of autoimmune hepatitis. Przegl Lek 62:314–316

    PubMed  Google Scholar 

  7. Lis-Swiety A, Brzezinska-Wcislo L, Pierzchala E, Wcislo-Dziadecka D (2006) Systemic sclerosis–polymyositis overlap syndrome accompanied by autoimmune hepatitis and sarcoidosis of mediastinal lymph nodes. J Eur Acad Dermatol Venereol 20:107–108

    Article  CAS  PubMed  Google Scholar 

  8. Ngo Mandag N, Van Gossum M, Rickaert F, Golstein M (2007) Autoimmune hepatitis and CREST syndrome. Rev Med Brux 28:528–531

    CAS  PubMed  Google Scholar 

  9. Ishikawa M, Okada J, Shibuya A, Kondo H (1995) CRST syndrome (calcinosis cutis, Raynaud’s phenomenon, sclerodactyly, and telangiectasia) associated with autoimmune hepatitis. Intern Med 34:6–9

    Article  CAS  PubMed  Google Scholar 

  10. Yabe H, Noma K, Tada N, Mochizuki S, Nagano M (1992) A case of CREST syndrome with rapidly progressive liver damage. Intern Med 31:69–73

    Article  CAS  PubMed  Google Scholar 

  11. Montano-Loza AJ, Carpenter HA, Czaja AJ (2008) Frequency, behavior, and prognostic implications of antimitochondrial antibodies in type 1 autoimmune hepatitis. J Clin Gastroenterol 42:1047–1053

    Article  PubMed  Google Scholar 

  12. D’Angelo WA, Fries JF, Masi AT, Schulman LE (1969) Pathologic observations in systemic sclerosis(scleroderma): a study of fifty-eight autopsy cases and fifty-eight matched controls. Am J Med 46:428–440

    Article  PubMed  Google Scholar 

  13. White B (2001) Systemic sclerosis, epidemiology, pathology and pathogenesis. In Primer on the rheumatic diseases. 2nd ed, Canada, Arthritis Foundation, 353–356

  14. Steen VD, Medsger TA Jr (1998) Case-control study of corticosteroids and other drugs that either precipitate or protect from the development of scleroderma renal crisis. Arthritis Rheum 41:1613–1619

    Article  CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Rheumatology Division, Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, Brazil

    Carlos Ewerton Maia Rodrigues, Cláudia Lobato Borges & Jozélio Freire de Carvalho

  2. 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

    Jozélio Freire de Carvalho

Authors
  1. Carlos Ewerton Maia Rodrigues
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  2. Cláudia Lobato Borges
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  3. Jozélio Freire de Carvalho
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Corresponding author

Correspondence to Jozélio Freire de Carvalho.

Additional information

JF Carvalho received a grant from Federico Foundation.

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Rodrigues, C.E.M., Borges, C.L. & de Carvalho, J.F. Diffuse systemic sclerosis and autoimmune hepatitis: a unique association. Clin Rheumatol 29, 799–801 (2010). https://doi.org/10.1007/s10067-010-1390-x

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  • DOI: https://doi.org/10.1007/s10067-010-1390-x

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