Page 185 - The Vasculitides, Volume 1: General Considerations and Systemic Vasculitis

In: The Vasculitides, Volume 1 ISBN: 978-1-63463-110-5
Editors: David S. Younger © 2015 Nova Science Publishers, Inc.

Chapter 9

The Clinical Approach
to Patients with Vasculitis

Lindsay Lally, M.D. and Stephen A. Paget, M.D.?

Division of Rheumatology, Hospital for Special Surgery, New York, NY, US

Abstract

The systemic vasculitides are a heterogeneous group of disorders. Pathologically
defined by the presence of vascular inflammatory infiltrate, primary vasculitides occur in
the absence of a known cause or associated disease state, while secondary vasculitides
occur in association with known underlying disorders and disease triggers. The vasculitic
process is often systemic however vascular inflammation may be confined to an isolated
organ system such as the kidney or even central nervous system. This chapter provides an
overview of the approach to patients with systemic vasculitis including the clinical
presentation, differential diagnosis, imaging and histopathologic evaluation, and short-
and long-term treatment-related complications.

Introduction

The primary systemic vasculitides are a diverse group of multisystem syndromes
characterized according to the size of the blood vessels involved and the organ systems
affected. Inflammation in the blood vessels can lead to diminished blood flow or vessel
occlusion resulting in ischemia, necrosis and subsequent tissue damage. The blood vessels
themselves can be damaged in vasculitis resulting in permanent stenosis, aneurysmal change
and vessel rupture. Any combination of vessels from large arteries to tiny venules and
capillaries may be involved in the inflammatory process. The 2012 Revised Chapel Hill
Consensus Conference (CHCC) [1] provides useful nosology for the primary vasculitides
based upon the caliber of the vessels, both arteries and veins, involves. Small vessel vasculitis

? Corresponding author: Stephen A. Paget, M.D. Division of Rheumatology, Hospital for Special Surgery, 535 E.
70th Street, New York, NY 10021.E-mail: [email protected].

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