Vasculitis and Its Classification

Petty, Ross E.; Cabral, David A

doi:10.1016/b978-0-323-24145-8.00032-6

Cited by 7 publications

(4 citation statements)

References 17 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Vasculitis is group of disorders characterized by inflammation of blood vessels leading to organ dysfunction. Given the ubiquitous presence of blood vessels, vasculitis can result in a wide array of clinical manifestations from the pathologic consequences of inflammation, hemorrhage, and/or ischemia ( 1 ). Vasculitides are traditionally classified based on the size of affected vessels, involvement of specific organs, findings on microscopic examination, and presence of other associated etiologies ( 2 , 3 ).…”

Section: Introductionmentioning

confidence: 99%

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2

Lee

2018

Front. Pediatr.

View full text Add to dashboard Cite

Deficiency of adenosine deaminase 2 (DADA2) is a monogenic form of systemic vasculopathy that often presents during early childhood. Linked to biallelic mutations in ADA2 (previously CECR1), DADA2 was initially described as a syndrome of recurrent fever, livedo racemosa, early-onset strokes, and peripheral vasculopathy that resembles polyarteritis nodosum. However, the wide spectrum of clinical findings and heterogeneity of disease, even among family members with identical mutations, is increasingly recognized. Evidence of systemic inflammation and vasculopathy is not uniformly present in DADA2 patients and some can remain asymptomatic through adulthood. Humoral immunodeficiency characterized by low immunoglobulin levels and increased risk of infection is another common feature of DADA2. Variable cytopenias including pure red cell aplasia that mimics Diamond-Blackfan anemia can also be primary manifestations of DADA2. How defects in a single gene translate into these heterogeneous presentations remains to be answered. In this review, we will summarize lessons learned from the pleiotropic clinical manifestations of DADA2.

show abstract

Section: Introductionmentioning

confidence: 99%

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2

Lee

2018

Front. Pediatr.

View full text Add to dashboard Cite

show abstract

“…Biopsy-proven HSP nephritis was also Other concomitant causes (viral, autoimmune, and infectious diseases) that might account for the cardiac involvement were excluded, and both cardiac and renal involvements were treated with intensive immunosuppressive therapy. Cardiac involvement is extremely rare in children with HSP, as well as in adults [1,2]. To date, secondary cardiac involvement in HSP has been reported in only 16 patients, including this patient.…”

Section: Discussionmentioning

confidence: 84%

“…In addition to the typical lesions in the lower extremities, children with Henoch-Schönlein purpura (HSP) often have gastrointestinal system, joint, and kidney involvement, and rarely central nervous system, pulmonary, ocular, and scrotal involvement. Indeed, kidney involvement in patients with HSP determines the prognosis [1,2]. Moreover, cardiac involvement is rare in Henoch-Schönlein purpura, and few case reports have been published in the literature [3][4][5][6][7][8][9][10][11][12][13][14][15][16].…”

Section: Introductionmentioning

confidence: 99%

Myocarditis and intracardiac thrombus due to Henoch-Schönlein purpura: case report and literature review

et al. 2020

View full text Add to dashboard Cite

Cardiac involvement is very rare in patients with Henoch-Schönlein purpura (HSP). In this case study, we present an 8-year-old girl presenting with HSP-induced myocarditis and thrombus in the right atrium and HSP nephritis. To date, 15 cases of HSPrelated cardiac involvement have been reported in the PubMed/MEDLINE, Scopus, and Google Scholar databases. These cases, together with our case, are included in this review. We excluded those patients with other rheumatologic diseases (acute rheumatic fever, acute post-streptococcal glomerulonephritis, Kawasaki disease) accompanied by HSP. Three were children and 13 were adults and all were male except our case. This review revealed tachyarrhythmia, chest pain, dyspnea, murmur, and heart failure as the major signs. Cardiac tests, electrocardiogram (ECG), and imaging methods (echocardiography in all patients, cardiac magnetic resonance imaging (MRI) in three, cardiac biopsy in one, and post-mortem necropsy in three) showed that the cardiac involvements were pericardial effusion, intra-atrial thrombus, myocarditis, coronary artery changes, myocardial ischemia, infarction and necrosis, subendocardial hemorrhage, and left ventricular dilatation. Kidney involvement was not observed in three patients. As the treatment, high-dose prednisolone and cyclophosphamide, oral corticosteroid, azathioprine, nadroparin calcium, ACE inhibitors, calcium antagonists, beta-blockers, and diuretics were used. Eleven patients (all three children and eight of the adults) had a complete cardiac recovery. Cardiac involvement in adults was more likely to be fatal. Death (three patients), ischemia, and infarct have been reported only in adults. We suggested that early and aggressive treatment can be life-saving. MRI examination is effective at identifying cardiac involvement.

show abstract

“…Systemic vasculitis broadly defines a heterogenous group of inflammatory diseases that primarily affect blood vessels ( 1 ). The etiology of most systemic vasculitides remains unknown and the forms of vasculitis in children and adults are not the same.…”

mentioning

confidence: 99%

Editorial: DADA2 and other monogenic vasculitides

2022

View full text Add to dashboard Cite

Editorial on the Research Topic DADA2 and other monogenic vasculitidesSystemic vasculitis broadly defines a heterogenous group of inflammatory diseases that primarily affect blood vessels (1). The etiology of most systemic vasculitides remains unknown and the forms of vasculitis in children and adults are not the same. Among primary vasculitides, IgA vasculitis and Kawasaki disease are common in young children, whereas some such as Takayasu arteritis and anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis are more common in adults. The landmark discoveries of deficiency of adenosine deaminase 2 (DADA2) and STING-associated vasculopathy of infancy (SAVI) redefined the paradigm of systemic vasculitis (2-4). These monogenic forms of secondary vasculitis often manifest early in life and inflict devastating consequences in children and adults (5). While the causal genes for DADA2 and SAVI are defined, we have much more to learn about the complex pathophysiology of these conditions. In the Research Topic "DADA2 and other Monogenic Vasculitides," we aim to advance the pathophysiology, diagnostic evaluation, and treatment for these intriguing yet potentially fatal diseases. An overview of monogenic vasculitidesReview articles by Signa et al. and Wobma et al. provide excellent summaries of DADA2 and SAVI, respectively. Although the precise mechanism linking defective ADA2 function to the plethora of manifestations in DADA2 remains unclear, Signa et al. synthesize the abundant evidence pointing to abnormalities in both innate immunity and adaptive immunity. Aberrant monocyte polarization and neutrophil extracellular traps are potential explanations for the excess tumor necrosis factor (TNF) production (4, 6). Recent studies also suggest dysregulation of type I interferons (IFN-I) in DADA2 (7-9), although the role of these antiviral cytokines awaits further clarification. Wobma et al. review the stimulator of interferon genes (STING) axis of IFN-I production and discuss how dysregulation of this pathway leads to immune activation and progressive Frontiers in Immunology frontiersin.org 01

show abstract

Vasculitis and Its Classification

Cited by 7 publications

References 17 publications

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2

Vasculopathy, Immunodeficiency, and Bone Marrow Failure: The Intriguing Syndrome Caused by Deficiency of Adenosine Deaminase 2

Myocarditis and intracardiac thrombus due to Henoch-Schönlein purpura: case report and literature review

Editorial: DADA2 and other monogenic vasculitides

Contact Info

Product

Resources

About