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CASE-BASED REVIEW
Year : 2016  |  Volume : 11  |  Issue : 3  |  Page : 167-170

Uncommon clinical presentation of cryoglobulinemia vasculitis successfully treated with rituximab and mycophenolate mofetil


1 Department of Rheumatology, Central Defense University Hospital Gómez Ulla, Madrid, Spain
2 Department of Neurophysiology, Central Defense University Hospital Gómez Ulla, Madrid, Spain
3 Department of Pathology, University Hospital 12 de Octubre, Madrid, Spain
4 Department of Neurology, Central Defense University Hospital Gómez Ulla, Madrid, Spain

Correspondence Address:
María Ahijón Lana
Glorieta del Ejercito, 1. 28047, Madrid
Spain
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-3698.187416

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We report the case of a 80-year-old woman with a severe mononeuritis multiplex over bilateral lower limb, with no other clinical signs or symptoms of vasculitis. Laboratory test showed elevated erythrocyte sedimentation rate and C reactive protein, low C4 levels, positive rheumatoid factor and polyclonal IgG and IgA cryoglobulins at a high cryocrit. Malignancies disorders and infectious diseases screening was negative. Nerve biopsy revealed signs of vasculitis with inflammatory infiltrate of epineural vessels, axonal degeneration and moderate loss of myelinated fibres. The diagnosis of essential mixed cryoglobulinemia vasculitis was established. She received treatment with plasmapheresis and high doses of steroids with progression of the symptoms to the upper limbs and persistence of positive cryoglobulins, low C4 levels and high acute phase reactants. She required treatment with rituximab and mycophenolate mofetil to control the disease. In conclusion we report an uncommon clinical presentation of a cryoglobulinemia vasculitis successfully treated with two B- cell depleting therapies.


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