Cryoglobulinemic vasculitis in pregnancy.

Int J Gynaecol Obstet

Department of Hematology, Faculty of Pathology, Mubarak Al Kabeer Hospital, Kuwait University, Kuwait.

Published: November 2008

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http://dx.doi.org/10.1016/j.ijgo.2008.05.027DOI Listing

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Cryoglobulinemic vasculitis is a rare small-vessel vasculitis leading to multi-organ dysfunction, often associated with chronic infections like hepatitis C virus (HCV), and autoimmune disorders. Most cases involve mixed monoclonal or polyclonal immunoglobulins, presenting symptoms such as purpura, arthralgias, and weakness. Severe organ involvement, particularly cardiac, is rare but potentially life-threatening.

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  • Type I and mixed cryoglobulinemic vasculitis have different causes, symptoms, and treatment responses, with a reported case of refractory vasculitis linked to ischemic non-obstructive coronary artery disease.
  • The patient exhibited severe symptoms including dyspnea, abdominal pain, purpura, and renal failure requiring dialysis, with diagnostic markers suggesting mixed cryoglobulinemia.
  • Despite various treatments failing initially, bortezomib and dexamethasone successfully led to clinical improvement and cryoglobulin negativity, indicating bortezomib's potential as an effective therapy for this challenging condition.
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AP-HP, Université Paris Saclay, department of internal medicine and clinical immunology, Bicêtre Hospital, Le Kremlin Bicêtre, France.

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Cryoglobulinemia is a rare disease characterized by the presence of cryoglobulins in the blood serum. It is usually caused by autoimmune, lymphoproliferative, or infectious factors. The pathogenesis of cryoglobulinemia is not well understood, therefore, genetic testing is very important.

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  • - A case study of a 52-year-old woman with Sjögren's syndrome revealed she developed cryoglobulinemic vasculitis, causing severe complications like lower extremity weakness and renal issues.
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