Autoimmune Hepatitis and Systemic Sclerosis: a Rare Association

  • Elisabete Pereira Coelho Unidade de Cuidados Intensivos Polivalente, Hospital de Braga, Braga, Portugal
  • Ana Rita Matos Serviço de Medicina Interna, Hospital de Braga, Braga, Portugal
  • Sofia Caridade Serviço de Medicina Interna, Hospital de Braga, Braga, Portugal

Keywords

Autoimmune hepatitis, systemic sclerosis, alcoholic hepatitis

Abstract

A woman in her early 40s, with a history of excessive alcohol intake, presented with purpuric, ulcerative lesions on the lower limbs. On examination, hirsutism and generalized stiffening and thickening of the skin were noted. Laboratory investigations revealed hyperbilirubinemia, hypergammaglobulinemia and positive anti-smooth muscle antibodies. Histologic examination of the skin was compatible with scleroderma. Histologic examination of the liver was suggestive of autoimmune and alcoholic hepatitis.

VIEW THE ENTIRE ARTICLE

References

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

  • Toyoda M, Yokomori H, Kaneko F, Yoshida H, Hoshi K, Takeuchi H, et al. Primary biliary cirrhosis-autoimmune hepatitis overlap syndrome concomitant with systemic sclerosis, immune thrombocytopenic purpura. Intern Med 2009;48:2019–2023.

  • Assandri R, Monari M, Montanelli A. Development of systemic sclerosis in patients with autoimmune hepatitis: an emerging overlap syndrome. Gastroenterol Hepatol Bed Bench 2016;9:211-9.

  • West M, Jasin HE, Medhekar S. The development of connective tissue diseases in patients with autoimmune hepatitis: a case series. Semin Arthritis Rheum 2006;35:344–348.
  • Liberal R, Krawitt EL, Vierling JM, Manns MP, Mieli-Vergani G, Vergani D. Cutting edge issues in autoimmune hepatitis. J Autoimmun 2016;75:6–19.

  • Rodrigues CE, Borges CL, de Carvalho JF. Diffuse systemic sclerosis and autoimmune hepatitis: a unique association. Clin Rheumatol 2010;29:799–801.

  • You BC, Jeong SW, Jang JY, Goo SM, Kim SG, Kim YS, et al. Liver cirrhosis due to autoimmune hepatitis combined with systemic sclerosis. Korean J Gastroenterol 2012;59:48–52.

  • Johnson SR. New ACR EULAR guidelines for systemic sclerosis classification. Curr Rheumatol Rep 2015;17:32.

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

  • Hennes EM, Zeniya M, Czaja AJ, Pares A, Dalekos GN, Krawitt EL, et al. Simplified criteria for the diagnosis of autoimmune hepatitis. Hepatology 2008;48:169–176.
  • Published: 2019-12-13

    Issue: Vol 6 No 12 (view)

    Section: Articles

    How to cite:
    Coelho, E., Matos, A., & Caridade, S. (2019). Autoimmune Hepatitis and Systemic Sclerosis: a Rare Association. European Journal of Case Reports in Internal Medicine, 6(12). https://doi.org/https://doi.org/10.12890/2019_001352

    Most read articles by the same author(s)