Vasculitis crioglobulinémica con compromiso renal en pacientes con crioglobulinemia mixta esencial sin relación con infección por virus de la hepatitis C. Descripción de una serie de casos y revisión de la bibliografía

Abstract

Cryoglobulinemic vasculitis is a small vessel vasculitis associated with the presence of cryoglobulins. It compromises skin, joints, peripheral nervous system and kidneys. In the kidney the predominant compromise is glomerular, with histopathological manifestations characterized by a membranoproliferative pattern associated with immune complex deposition. The objective of this paper is to describe the clinical presentation, histopathological characteristics, treatment response and prognosis of a series of patients with renal cryoglobulinemic vasculitis associated with mixed cryoglobulinemia, confirmed involvement (nephritic syndrome, rapidly progressive renal failure). The most frequent histopathological pattern was membranoproliferative glomerulonephritis with immune complex deposition. In 2 cases underlying disease (autoimmune, infectious) was found. All received initial treatment based on glucocorticoids, in two therapeutic plasma replacement was performed and in three cyclophosphamide it was administered. As maintenance, the whole received corticosteroids associated with mofetil mycophenolate or rituximab. One patient died shortly after diagnosis. A 24.2-month follow-up was complet-ed, at the end of it, 3 presented partial response and 2 complete response.