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Management of Small Vessel Vasculitides

  • Vasculitis (LR Espinoza, Section Editor)
  • Published:
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Abstract

Inflammation mediated by cells of the immune system and necrosis are the most striking features observed at the histologic level in patients with vasculitides, clinical entities classified according to pathologic findings involving different organs, to etiology, or to size of vessels involved. Small vessel vasculitides (SVV) are a peculiar group of systemic disorders electively involving small intraparenchymal arteries, arterioles, capillaries, or venules and leading to different levels of vascular obstruction, tissue ischemia and risk of infarction; they can be divided into anti-neutrophil cytoplasmic antibody-associated vasculitides and immune complex vasculitides. Despite the significant advances in understanding the whole disease process and pathophysiology of SVV, strong efforts are still needed to draft, share and spread guidelines in the therapeutic management of these protean disorders. After an accurate evaluation of different open or double-blind trials and cohort studies in this review, we analyze the actual medical tools suggested for treating granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, Henoch-Schönlein purpura, cryoglobulinemic vasculitis, anti-glomerular basement membrane disease and hypocomplementemic urticarial vasculitis.

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    Authors and Affiliations

    1. Interdisciplinary Department of Medicine, Rheumatology Unit, University of Bari, Bari, Italy

      Giuseppe Lopalco, Vincenzo Venerito, Maria Grazia Anelli, Giovanni Lapadula & Florenzo Iannone

    2. Institute of Pediatrics, Fondazione Policlinico Universitario A. Gemelli, Università Cattolica Sacro Cuore, Rome, Italy

      Donato Rigante

    3. Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy

      Giacomo Emmi

    4. Research Center of Systemic Autoinflammatory Diseases and Behçet’s Disease Clinic, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Siena, Italy

      Luca Cantarini

    5. Rheumatology Unit, Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Policlinico Le Scotte, Viale Bracci 1, 53100, Siena, Italy

      Luca Cantarini

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    1. Giuseppe Lopalco
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    2. Donato Rigante
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    3. Vincenzo Venerito
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    5. Maria Grazia Anelli
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    6. Giovanni Lapadula
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    7. Florenzo Iannone
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    8. Luca Cantarini
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    Correspondence to Luca Cantarini.

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    GLO, DR, VV, GE, MGA, GLA, FI, and LC declare that they have no conflicts of interest.

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    This article does not contain any studies with human or animal subjects performed by any of the authors.

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    This article is part of the Topical Collection on Vasculitis

    Giuseppe Lopalco and Donato Rigante contributed equally to this work.

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    Lopalco, G., Rigante, D., Venerito, V. et al. Management of Small Vessel Vasculitides. Curr Rheumatol Rep 18, 36 (2016). https://doi.org/10.1007/s11926-016-0580-1

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