**Author details**

IL-6, TNF-α, tumor necrosis factor-like weak inducer of apoptosis (TWEAK), IL-8, TGF-β, and

Tissue samples of affected skin areas from patients with HSP show epidermal staining with IL-6 [155]. Serum levels of IL-6 were significantly higher in patients with HSP during the acute phase of disease than in controls and also higher in patients with HSN than HSP without renal affection [153]. IL-6 displays a wide variety of pro-inflammatory properties and promotes the

IL-6 displays, besides its various pro-inflammatory effects, even anti-inflammatory effects by

TNF-α is produced by macrophages and T cells in affected skin areas during HSP. Serum levels of TNF-α were higher in patients with HSN than HSP without renal engagement [153].

It stimulates the presentation of adhesion molecules and receptors on leukocytes and endo‐ thelial cells thereby directing inflammatory events. Furthermore, endothelial cells stimulated with TNF-α were shown to bind IgA with higher affinity [155]. These findings suggest, that TNF-α could be involved in the accumulation of granulocytes and endothelial sequestration

TWEAK, a member of the TNF superfamily, which binds to specific receptors on endothelial cells, is involved in the regulation of cell growth, angiogenesis, apoptosis, and inflammation. In vitro evidence suggests that TWEAK may induce cytokine production by human micro‐ vascular endothelial cells via up-regulation of the production of IL-8 and CCL-5 leading to a leucocyte migration into affected vessels [158,159] which are common aspects of the HSP

Sera and IgA from patients with HSP induce the secretion of IL-8 from endothelial cells invitro

IL-8 is a potent chemoattractant for polymorphonuclear neutrophilic granulocytes (PMNs). Levels of leukotriene B4, also a potent chemo-attractant and activator of PMNs, are elevated

Furthermore, the levels of leukotriene A4, which counter-balance the effects of leukotriene B4 and inhibit the synthesis of proinflammatory cytokines (e.g. IL-6, IL-8, TNF- α), are decreased

The role of VEGF in HSP is not clear-cut. Serum levels of VEGF were significantly higher during the acute phase of HSP than during remission. However tissue staining for VEGF showed more intense staining for VEGF in the epidermis and vascular bed during the resolution phase than during the acute phase of HSP [163]. High serum levels of VEGF could influence endothelial permeability, which may enhance capillary leakage and facilitate the extravasation and perivascular deposition of immune complexes. The increased tissue staining during the resolution phase, on the other hand, suggests a possible function of VEGF in the resolution of

both in serum and urine in patients with HSN compared to those with HSP.

VEGF have been found to be up-regulated during the acute stage of HSP [153,154].

inhibiting TNF-α and IL-1 and activating IL-1ra as well as IL-10 [157].

secretion of IgA [153,156].

16 Updates in the Diagnosis and Treatment of Vasculitis

lesion.

[160,161].

in patients with HSN [162].

vascular damage.

of IgA as seen in affected tissues in HSP [153].

Mohamed Abdgawad\*

Address all correspondence to: mohamed.abdgawad@med.lu.se

The Department of Medicine, Blekinge Hospital, Karlshamn, Sweden

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**Chapter 2**

**The Pathogenesis of Antineutrophil Cytoplasmic**

The vasculitides comprise a heterogeneous group of diseases characterized by inflammation and destruction of blood vessels. Vessels of any size can be involved which explains the diverse spectrum of clinical diseases attributed to vasculitis. While the immunological basis of disease for vasculitis was recognized over thirty years ago,[1] a standardized classification system was only adopted nearly twenty years later. The initial classification system proposed by the American College of Rheumatology attempted to classify vasculitis according to standardized criteria.[2] The subsequent system described by the Chapel Hill Conference on the Nomen‐ clature of Systemic Vasculitis[3] introduced a system which coupled contemporary commonly

Necrotizing arteritis is common to many forms of vasculitis, but involvement of vessels smaller than arteries is unique to small vessel vasculitis.[4] A clinical report of 'Vasculitis' originated from the mid-nineteenth century[5] and clinical descriptions of these diseases were published in the 1930s,[6] however it was not until the 1950s that Wegener's Granulo‐ matosis, Churg Strauss Syndrome and Microscopic polyangiitis were identified as unique clinical entities.[7] In the 1980s it was appreciated that the small vessel vasculitides repre‐ sented a clinically distinct form of disease.[8] These small vessel vasculitides will be the pri‐

> © 2013 Ford et al.; licensee InTech. This is an open access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use,

© 2013 The Author(s). Licensee InTech. This chapter is distributed under the terms of the Creative Commons Attribution License http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution,

distribution, and reproduction in any medium, provided the original work is properly cited.

and reproduction in any medium, provided the original work is properly cited.

**Antibody Renal Vasculitis**

Shaun Andrew Summers

http://dx.doi.org/10.5772/54637

**1. Introduction**

**1.1. Small vessel vasculitis**

mary focus of this chapter.

Sharon Lee Ford, Stephen Roger Holdsworth and

Additional information is available at the end of the chapter

used disease names and the size of vessel(s) involved.
