Pathophysiology and therapy of systemic vasculitides

Abstract

Systemic vasculitides represent uncommon conditions characterized by the inflammation of blood vessels that can lead to different complex disorders limited to one organ or potentially involving multiple organs and systems. Systemic vasculitides are classified according to the diameter of the vessel that they mainly affect (small, medium, large, or variable). The pathogenetic mechanisms of systemic vasculitides are still partly unknown, as well as their genetic basis. For most of the primary systemic vasculitides, a single gold standard test is not available, and diagnosis is often made after having ruled out other mimicking conditions. Current research has focused on new management protocol and therapeutic strategies aimed at improving long-term patient outcomes and avoiding progression to multiorgan failure with irreversible damage. In this narrative review, authors describe different forms of systemic vasculitides through a review of the literature, with the aim of highlighting the current knowledge and recent findings on etiopathogenesis, diagnosis and therapy.

Keywords: Behcet's disease; Giant cell arteritis; Kawasaki disease; Polyarteritis nodosa; Systemic vasculitides; Takayasu arteritis.

Table 1. Chapel Hill Consensus Conference 2012 Classification criteria

Table 2. Different types of vessel involvement based on conventional angiographic findings published by the International Conference on Takayasu Arteritis in 1994

Table 3. The 1990 American College of Rheumatology Classification criteria for Takayasu arteritis. For classification purposes, a patient is said to have Takayasu arteritis if at least three of the six criteria are present (from Arend et al., 1990).

Table 4. European League Against Rheumatism (EULAR) / Paediatric Rheumatology European Society (PRES) / Paediatric Rheumatology International Trials Organization (PRINTO) classification criteria for childhood IgA vasculitis

Table 5. International Study Group (ISG) criteria for the diagnosis of Behcet's disease

Figure 1. Algorithm for syndromic approach of symptoms in vasculitis (from Sangolli and Lakshmi, 2019; available from: http://www.idoj.in/text.asp?2019/10/6/617/270204)

Figure 2. Classification of primary vasculitis based on revised Chapel Hill criteria (2012) and the European League Against Rheumatism (EULAR) / Paediatric Rheumatology European Society (PRES) classification (from Schnabel and Hedrich, 2018; available from: https://doi.org/10.3389/fped.2018.00421)