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Cryoglobulinemic vasculitis in systemic sclerosis successfully treated with mycophenolate mofetil

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Abstract

Cryoglobulinemic vasculitis is extremely rare in patients with systemic sclerosis (SSc). So far, only two cases of cryoglobulinemic vasculitis in SSc were described in the literature. This report is about a patient with SSc and secondary Sjőgren’s syndrome, who developed typical clinical features of small-vessel vasculitis, including arthritis, purpura, microhaematuria, gangrene of fingers, and toes and myocardial ischemia, in the presence of mixed cryoglobulinemia, ANA, rheumatoid factor, and anti-SSA/Ro antibodies. Symptoms and signs of vasculitis worsened despite initial treatment with corticosteroids and cyclophosphamide, but improved significantly when mycophenolate mofetil was used instead cyclophosphamide.

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References

  1. Cutolo M, Sulli A, Smith V (2011) Evaluating micropangiopathy in systemic sclerosis: what have we learnt and what is left to discover. Expert Rev Clin Immunol 7(4):395–397

    Article  PubMed  Google Scholar 

  2. Wigley F (2009) Vascular disease in scleroderma. Clinic Rev Allerg Immunol 36(2–3):150–175

    Article  Google Scholar 

  3. Blain H, Cacoub P, Musset L et al (2000) Cryofibrinogenaemia: a study of 49 patients. Clin Exp Immunol 120(2):253–260

    Article  CAS  PubMed Central  PubMed  Google Scholar 

  4. Husson JM, Druet P, Contet A (1976) Systemic sclerosis and cryoglobulinemia. Clin Immunol Immunopathol 6(1):77–82

    Article  CAS  PubMed  Google Scholar 

  5. Jiménez López A, García Marcos MA, Fernández-Roldán CC et al (1980) Sindrome CRST y crioglobulinemia mixta. Medicina Clinica 74(1):31–33

    PubMed  Google Scholar 

  6. Barrett MC, Prendiville JS, Pardy BJ et al (1986) Cryofibrinogenaemia and acute gangrene in systemic sclerosis. Postgrad Med J 62(732):935–936

    Article  CAS  PubMed  Google Scholar 

  7. Oddis CV, Eisenbeis CH Jr, Reidbord HE et al (1987) Vasculitis in systemic sclerosis: association with Sjőgren’s syndrome and the CREST syndrome variant. J Rheumatol 14(5):942–948

    CAS  PubMed  Google Scholar 

  8. Ginzler EM, Dooley MA, Aranow C et al (2005) Mycophenolate mofetil or intravenous cyclophosphamide for lupus nephritis. N Engl J Med 353:2219–2228

    Article  CAS  PubMed  Google Scholar 

  9. Joy MS, Hogan SL, Jennette JC et al (2005) A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 20:2725–2732

    Article  CAS  PubMed  Google Scholar 

  10. Ramos-Casalas M, Font J (2005) Mycophenolate mofetil in patients with hepatitis C virus infection. Lupus 14(Suppl 1):64–72

    Article  Google Scholar 

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Correspondence to Predrag Ostojic.

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Ostojic, P. Cryoglobulinemic vasculitis in systemic sclerosis successfully treated with mycophenolate mofetil. Rheumatol Int 34, 145–147 (2014). https://doi.org/10.1007/s00296-012-2640-9

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  • DOI: https://doi.org/10.1007/s00296-012-2640-9

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