Vasculitides secondary to systemic diseases (original) (raw)

Pathophysiology and therapy of systemic vasculitides

EXCLI Journal, 2020

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 th...

Pathophysiological Relationship between Infections and Systemic Vasculitis

Autoimmune Diseases, 2015

The development of autoimmune disorders requires a combination of genetic, immunological, and environmental factors. Infectious agents, such as viruses and bacteria, can trigger autoimmunity through different mechanisms, and for systemic vasculitis in particular, microbial agents have been suggested to be involved in its pathogenesis. Although the exact mechanisms have not been fully elucidated, different theories have been postulated. This review considers the role of infections in the etiology of primary vasculitis, emphasizing their related immunological events.

Systemic vasculitis: a difficult diagnosis

Sao Paulo Medical Journal, 1997

N othingtriggers a,n internist's diagnostic interests more than a potential case of systemic vasculitis. The ubiquitous nature of the target tissue, the variations in the rate and severity of disease progression, and its ability to mimic other diseases mandate an intense and focused approach to diagnosis and treatment. Blood vessels have limited ways of responding to injury: increased vascular permeability, weakening of vessel wall with resultant aneurysm formation or hemorrhage or both, and intimaI proliferation and thrombosis which can result in obstruction and ischemia. I Vasculitis is a heterogeneous group of disorders characterized by inflammation, often with necrosis and occlusion of blood vessels. These disorders may be generalized or localized, primary (Le., the essential disorder affecting blood vessels) or secondary (associated with a variety of different underlyng diseases). The clinicaI spectrum of vasculitis represents one of the most interesting and perplexing group of diseases in internaI medicine. 2 Since the first classification of vasculitis by Zeek in 1952,3 many other classification systems have been proposed,4-7 but the lack of pathognomonic clinicaI and laboratory features, uniform histopathological expression, and unknown or incompletely understood etiology and pathogenesis for the majority of the disorders make it

The “other” vasculitis syndromes and kidney involvement

Pediatric Nephrology, 2010

There are a number of vasculitides that are not confined to a specific vessel size, do not have characteristic features, and/or are not secondary to another disease. Most of these vasculitides are rare in childhood. Behçet disease is representative of this group as it involves vessels of any size on both the arterial and venous side. In addition to renal vascular involvement, Behçet disease may involve the kidney through glomerulonephritis, secondary amyloidosis and, rarely, tubulointerstital involvement. Vasculitis secondary to infections, malignancy, and drugs are not common among children. However, vasculitis may be associated with a number of rheumatic diseases in childhood and the auto-inflammatory syndromes (periodic fever syndromes). Auto-inflammatory syndromes are diseases characterized by periodic attacks of clinical and laboratory inflammation. Studies carried out during the past decade have provided valuable information on the mechanism of inflammation and innate immunity in general. This group of vasculitides is associated with secondary amyloidosis of the kidney if not treated. Hypocomplementemic urticarial vasculitis is an interesting vasculitic disease with frequent kidney involvement. Here, we introduce the reader to the wide scope of these diseases; although rare, such diseases represent a challenge to the nephrologist.

Expert perspectives on pathological findings in vasculitis

Modern Rheumatology, 2022

Pathological findings are important in the diagnosis of vasculitis. However, due to the rarity of the disease, standard textbooks usually devote only a few pages to this topic, and this makes it difficult for clinicians not specializing in vasculitis to fully understand the pathological findings in vasculitis. To address the paucity of information, we present representative pathological findings in vasculitis classified in the 2012 Revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides (CHCC2012). The CHCC2012 classifies 26 vasculitides into seven categories: (1) large-vessel vasculitis, (2) medium-vessel vasculitis, (3) small-vessel vasculitis, including antineutrophil cytoplasmic antibodyassociated vasculitis and immune complex small-vessel vasculitis, (4) variable-vessel vasculitis, (5) single-organ vasculitis, (6) vasculitis associated with systemic disease, and (7) vasculitis associated with probable aetiology. Moreover, representative pathological findings of vasculitis-related diseases and non-inflammatory vasculopathy not mentioned in the CHCC2012 are also presented. This will be useful for clinicians to refer to typical pathological findings of vasculitis in daily practice.

Clinical characteristics and outcome of vasculitides

Journal of Patan Academy of Health Sciences, 2016

Introductions: Vasculitides can cause significant morbidity and mortality if not treated on time. There is lack of data locally. This study aims to define the pattern, clinical characteristics, and outcome of vasculitides.Methods: This was a cross sectional study between January 2011 and December 2015 at Patan Hospital, Patan Academy of Health Sciences, Lalitpur, Nepal. The medical records of patients diagnosed with vasculitides in adults rheumatology service of the hospital were reviewed.Results: Ninety six patients were diagnosed with vasculitides during the study period. The mean age was 42.2 years. Sixty nine (71.8%) patient had small vessel, 20 (20.8%) large vessel and five (5.2%) had variable vessel vasculitides. Seventy five patients (78.1%) had primary and 21 (21.8%) secondary vasculitides. Cutaneous leucocytoclasticangitis was seen in 27 (28.1%), Takayasu arteritis in 17 (17.7), Henoch-Schonlein purpura in 11 (11.4%) and Rheumatoid arthritis associated vasculitis in nine p...