Complications and causes of mortality in patients with primary necrotizing systemic vasculitis

The article reviews the clinical symptoms, main complications in patients with ANCA-associated systemic vasculitis (granulomatous with polyangiitis, microscopic polyangiitis, eosinophilic granulomatous with polyangiitis) and polyarteritis nodosa during the first three years of the disease. We evaluated the relationship between complications and long-term glucocorticosteroid therapy. Patients with granulomatosis had the most unfavourable course of the disease with a high risk of complications. A high incidence of ischemic strokes and chronic kidney disease in patients with microscopic polyangiitis has been identified. We also analyzed cases of lethal outcomes in patients with primary systemic vasculitis.

A Rare Cause of Young Stroke: What Rheumatologists Should Know about Fabry Disease

Systemic vasculitis is a category of autoinflammatory diseases usually involving multiple organs. Involvement of the central nervous system (CNS) may mimic other disorders, hence resulting in overdiagnosis. This is a case report of a young man who presented with acrodynia, episodic pyrexia, and recurrent strokes. He was treated for systemic vasculitis with glucocorticoids and immunosuppressants, which failed to prevent the stroke. Finally, Fabry disease was diagnosed after [Formula: see text]-galactosidase A gene mutation analysis was done. Rheumatologists should be aware of Fabry disease, which may present with multi-system involvement that mimics vasculitis. To avoid inappropriate treatment, a comprehensive differential diagnosis should be considered before a diagnosis of primary systemic vasculitis is made. Recurrent strokes, especially in males younger than 40, should raise suspicions of Fabry disease.

Cellular and Molecular Characteristics of Vascular Damage in Giant Cell Arteritis, the ‘Unmet Needs’ for Targeted Treatment

Giant cell arteritis (GCA) is a primary systemic vasculitis characterized by systemic inflammation and vascular insufficiency of large and medium blood vessels which may lead to end-organ damage in patients age 50 and older. Standard corticosteroid treatment of GCA significantly improves the intima-media thickness while having less influence on vascular endothelial dysfunction. GCA morbidity may be related to both cardiovascular complications and corticosteroid toxicity. Therefore, we aim to discuss 1) characteristic aspects of vascular damage, 2) several mechanisms that cause vascular dysfunction, intima-media ‘nodular’ thickness, progressive narrowing of the arterial lumen and vascular blockage in the context of systemic inflammation, thrombosis and of the cardiovascular complications in GCA and 3) new therapeutic glucocorticosteroid-sparing (GS) agents which might be a more productive way of avoiding the invalidating or life-threatening cardiovascular complications of GCA.

Trend and Geographic Disparities in the Mortality Rates of Primary Systemic Vasculitis in the United States from 1999 to 2019: A Population-Based Study

The current data on rates and geographic distribution of vasculitis mortality are limited. We aimed to estimate the mortality rates of primary systemic vasculitis and its geographic distribution using recent population data in the United States. The mortality rates of vasculitis from 1999 to 2019 were obtained from the Center for Disease Control (CDC) Wonder Multiple Cause of Death (MCD). The age-adjusted rates per million for vasculitis as MCD and as an underlying cause of death (UCD) were calculated by state using demographics. A joinpoint regression analysis was applied to evaluate trends over time. The age-adjusted mortality rate of vasculitis as MCD was 4.077 (95% CI: 4.029–4.125) and as a UCD was 1.888 per million (95% CI: 1.855–1.921). Since 1999, mortality rates have progressively decreased. The age-adjusted mortality rate was higher in females than in males. The highest mortality rate for vasculitis as MCD was in White patients (4.371; 95% CI: 4.317–4.424). The northern states and areas with lower populations had higher mortality rates. We found a trend of progressive decreases in the mortality rates of vasculitis, as well as gender, racial, and geographic disparities. Further analyses are warranted to better understand the factors associated with these disparities in order to implement targeted public health interventions to decrease them.

Autoantibodies Against Lysosome Associated Membrane Protein-2 (LAMP-2) in Pediatric Chronic Primary Systemic Vasculitis

BackgroundAnti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a small vessel vasculitis in adults and children that commonly affects the kidneys. Although the frequent antigenic, and presumed pathogenic, targets of ANCA in AAV are proteinase-3 (PR3) and myeloperoxidase (MPO), ANCA against lysosome associated membrane protein-2 (LAMP-2), a lesser known ANCA antigen that is expressed on the glomerular endothelium, are present in some adults with AAV-associated renal disease. LAMP-2-ANCA has not been assessed in children with chronic systemic vasculitis, and, if present, would be a potentially valuable biomarker given that treatment decisions for these pediatric patients at diagnosis are largely informed by kidney function.MethodsA custom ELISA, using commercially available reagents, was designed to detect autoantibodies to human LAMP-2 in serum. Sera obtained from 51 pediatric patients at the time of diagnosis of chronic primary systemic vasculitis (predominantly AAV) were screened. LAMP-2-ANCA titers were evaluated for correlation with clinical metrics of disease activity (pediatric vasculitis activity score [pVAS], C-reactive protein [CRP] concentration, and erythrocyte sedimentation rate [ESR]), MPO- and PR3-ANCA titers, and renal function (glomerular filtration rate [GFR], renal-specific pVAS, and serum creatinine concentration).ResultsLAMP-2-ANCA (>1,000 ng/ml) were detected in 35% (n = 18) of pediatric systemic vasculitis patients, of which, 10 (20% of all patients) were found to have high positive titers (>1,500 ng/ml). Undetectable or negative titres (<500 ng/ml) were identified in 12% (n = 6) of patients, those with titers between 500 and 1,000 ng/ml were considered low with unknown clinical relevance (53%, n = 27). Although LAMP-2-ANCA titers did not significantly differ between patients with AAV versus ANCA-negative vasculitis, only AAV patients had high concentrations (>1,500 ng/ml) of LAMP-2-ANCA. LAMP-2-ANCA titers did not correlate with measures of disease activity (pVAS, CRP, or ESR) at the time of diagnosis. In contrast, for patients with 12-month post diagnosis follow-up, a negative correlation was observed between the change in GFR (from diagnosis to 12-month follow-up) and LAMP-2-ANCA titer at diagnosis.ConclusionsModerate to high LAMP-2-ANCA titers were detected in 35% (18/51) of children with chronic systemic vasculitis affecting small-to-medium vessels. Although the highest concentrations of LAMP-2-ANCA in this population were observed in individuals positive for classic ANCA (MPO- or PR3-ANCA), similar to previous reports on adult patients, LAMP-2-ANCA titers do not correlate with classic ANCA titers or with overall disease activity at diagnosis. Renal disease is a common manifestation in systemic small-medium vessel vasculitis (both in adults and children, though more severe in children) and our preliminary data suggest LAMP-2-ANCA at diagnosis may be a risk factor for more severe renal disease.

Takayasu arteritis in pregnancy: A case series

Takayasu arteritis is a disease of unknown etiology which is a primary systemic vasculitis leading to stenotic and occlusive changes. Takayasu arteritis is a chronic inflammatory disease in progressive pattern which chiefly affects the aorta and its main branches. The diagnosis of the disease is mainly based on the clinical suspicion, history, physical examination and vascular imaging. We present here three cases of takayasu arteritis in pregnancy that were treated at our institute in the last 5 years. 1st case had complications like severe preeclampsia, abruption, temporary loss of vision, and intra uterine death of fetus. In 2nd case there was brain sparing effect of fetus, but other than that she did not have any other complications and neonatal outcome was also good without any neonatal complications. In the 3rd case antenatal and postnatal periods were uneventful and with good neonatal outcome. Pregnancy with takayasu arteritis needs to be timely diagnosed and treated for best maternal and fetal outcome.

A CASE REPORT ON TAKAYASU ARTERITIS

Takayasus arteritis primarily affects young women. Takayasu arteritis (TA) is a type of primary systemic vasculitis mainly affecting the medium and large arteries. The signs and symptoms are due to systemic inflammation or ischemia of an organ or limb and include angiodynia, claudication, peripheral pulselessness, murmurs, ischemic stroke, myocardial infarction and severe systemic arterial hypertension. The disease tends to affect more women than men.The current case of Takayasu arteritis type V focuses on a 21 year female who complained of cold, cough, fever, facial puffiness since 2 months, along with bilateral renal artery stenosis, and resistant hypertension.

A CASE REPORT ON TAKAYASU ARTERITIS

Takayasus arteritis primarily affects young women. Takayasu arteritis (TA) is a type of primary systemic vasculitis mainly affecting the medium and large arteries. The signs and symptoms are due to systemic inflammation or ischemia of an organ or limb and include angiodynia, claudication, peripheral pulselessness, murmurs, ischemic stroke, myocardial infarction and severe systemic arterial hypertension. The disease tends to affect more women than men.The current case of Takayasu arteritis type V focuses on a 21 year female who complained of cold, cough, fever, facial puffiness since 2 months, along with bilateral renal artery stenosis, and resistant hypertension.