Preface

Chapter 9 **Treatment of ANCA-Negative Small Vessel Vasculitis 217**

Chapter 10 **Recent Advances in the Management of Refractory**

Reem Hamdy A. Mohammed

**Vasculitis 239**

**VI** Contents

Christina G. Katsiari, Theodora Simopoulou and Lazaros I. Sakkas

Vasculitis, an inflammation of blood vessels, can be idiopathic or secondary to other conditions. Infections may also mimic idiopathic vasculitis and the differential diagnosis is of paramount importance for the practicing physician. Vasculitides are not uncommon diseases. In fact, some vasculitides, such as giant cell arteritis, cutaneous vasculitis, and ANCA-associated vasculitis, are relatively common in everyday practice. Vasculitis may rapidly lead to organ failure, and put patient's life in danger. Therefore, physicians of different specialties must diagnose system‐ ic vasculitis early, because early treatment is crucial for the favorable outcome.

In recent years progress has been made in the pathophysiology and treatment of vasculitis. Understanding molecular mechanisms in the pathogenesis of vasculitis helps in the rational development of new treatments. Trials with biological agents have been published, and EU‐ LAR and ACR have issued guidelines for the treatment of various types of vasculitis. This book reflects new advances in pathogenetic mechanisms, diagnosis, and treatment of differ‐ ent types of vasculitis. The international panel of authors helps in achieving a balanced view on different aspects of vasculitis.

We hope that this book will be an enjoyable and useful reading.

#### **Lazaros I. Sakkas, MD, DM, PhD**

Professor and Chairman, School of Medicine, University of Thessaly, Larissa, Greece

#### **Christina Katsiari, MD, DM,**

Lecturer, School of Medicine, University of Thessaly, Larissa, Greece

**Chapter 1**

**History, Classification and Pathophysiology of Small**

Systemic vasculitides are a heterogenous group of disorders characterized by destructive inflammation and fibrinoid necrosis of the blood vessel wall, blood vessel occlusion and ischemia of surrounding tissue. Typical clinical manifestations vary depending on the size of the affected blood vessels, and include fever, weight loss, malaise, arthralgias and arthritis. Vasculitides can be idiopathic, primary, secondary to another disease such as Systemic Lupus Erythematosus (SLE) and Rheumatoid Artritis (RA), or associated with infections, such as infective endocarditis, pharmaceutical drug use, such as propylthiouracil and hydralazine, or other chemical exposures [1]. Vasculitis can be isolated to one organ or vessel and be relatively insignificant clinically or can present as a systemic life-treatening illness involving several

ANCA- associated Systemic Vasculitis (AASV) is the most common primary systemic smallvessel vasculitis that occurs in adults. AASV is a small-vessel vasculitis affecting arterioles, venules, capillaries, and occasionally medium-sized arteries that commonly involves multiple organ systems. Although infrequent, the incidence of AASV is increasing. AASV is also called pauci-immune vasculitis, because no immunoglobulins or complement components are

AASV is associated with significant morbidity and mortality, with almost all patients requiring aggressive immunosuppression. Without treatment, the mortality approaches 100% in 5 years [3]. Based upon the clinical presentation and the predominant organ involvement, AASV cases are classified as Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and Renal Limited Vasculitis (RLV). ANCA are predominantly IgG

> © 2013 Abdgawad; 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.

**Vessel Vasculitis**

Mohamed Abdgawad

http://dx.doi.org/10.5772/55238

**1. Introduction**

organs and vessels [2].

detected in the vasculitic lesions.

Additional information is available at the end of the chapter
