Form Of Systemic Vasculitis Research Articles

Henoch-Schönlein Purpura (IgA Vasculitis) Chronic Lesions: How Fluorescence Imaging of Bacteria Illuminated the Way Toward Healing

Background/Objectives: IgA vasculitis, formerly Henoch-Schönlein purpura (HSP), is a form of systemic vasculitis that most commonly affects children. We present a case of hard-to-heal lower extremity lesions in a patient with confirmed IgA vasculitis or HSP. Methods: The patient was serially imaged with a handheld, bacterial autofluorescence imaging device across subsequent visits. Results: Due to the identification of bacterial burden presence that was not clinically evident, the patient was prescribed antibiotics and promptly healed. Conclusions: This case illustrates the value of bacterial fluorescence imaging in dermatological cases.

Rheumatoid Purpura in Children: A Retrospective Study.

Introduction Rheumatoid purpura, also referred to as Schönlein-Henoch disease, is a form of systemic vasculitis that is characterized by the presence of IgA deposits within the walls of small vessels. The primary symptoms include skin purpura, arthralgia, abdominal discomfort, and urinary tract abnormalities. While often benign, the disease can result in severe complications, particularly those affecting the digestive and renal systems, which require urgent medical attention. Objectives This study aimsto analyze the epidemiological, clinical, biological, therapeutic, and evolutionary characteristics of rheumatoid purpura in order to enhance understanding of its clinical and therapeutic management. The aim is to expand knowledge of the disease and optimize treatment strategies to prevent serious complications. Methods and results This retrospective study encompasses 39 cases of rheumatoid purpura treated at the General Pediatrics Department of CHU Mohamed VI in Oujda, Morocco, over a period of nine years and six months from January 2015 to June 2024. The patients had a mean age of 6.5 years, and there was a marked male predominance (sex ratio of 2.57). All patients exhibited cutaneous purpura (100%). Approximately 75% of patients exhibited joint involvement, 89% of cases were associated with digestive manifestations, and 28% of cases demonstrated renal involvement. Conclusion Rheumatoid purpura is a pediatric condition often triggered by an upper respiratory infection, characterized by IgA deposits. While typically benign, it can lead to severe complications, particularly in the digestive and renal systems. Corticosteroids are key in managing acute cases, although their preventive efficacy is limited. Early and appropriate management is crucial for improving long-term outcomes.

Incidence of thromboembolic events in patients with a systemic form of vasculitis and cutaneous vasculitis

Incidence of thromboembolic events in patients with a systemic form of vasculitis and cutaneous vasculitis

Idiopathic Eosinophilic Vasculitis: Case Presentation and Literature Review.

Idiopathic eosinophilic vasculitis has been described in previous case series as a possible manifestation of hypereosinophilic syndrome (HES) in asthma-free patients. A rare disease, it can be classified as an eosinophilic-rich, necrotizing, systemic form of vasculitis that affects vessels of various sizes in these patients. This report shares our experience with the treatment of a patient with eosinophilic vasculitis. We present the case of a 45-year-old man who suffered from idiopathic HES manifesting as digital ulcers and peripheral ischemia of both the upper and lower limbs without the involvement of other systems. Diagnosis was made after excluding the primary and secondary causes of eosinophilia. The patient responded well to both corticosteroids and mepolizumab, an interleukin-5 inhibitor, as a corticosteroid-sparing therapy. Our case of HES-associated vasculitis in an asthma-free patient supports previous reports describing this rare diagnosis of idiopathic eosinophilic vasculitis in recent years. We describe a good response to mepolizumab (interleukin-5 inhibitor) in our patient.

Thrombosis risk factors in ANCA-associated vasculitis

The article presents a review of current data on arteriovenous thrombosis risk factors in antineutrophil cytoplasmic antibody-associated vasculitis (AAV). Thrombotic complications are among the most frequent prognostically unfavorable factors in AAV. In general, patients with AAV are at greater risk of mortality from cardiovascular events compared with the general population and patients with other forms of systemic vasculitis. Understanding the mechanisms of thrombosis, as well as pathogenic factors that cause an increased risk of arterial and venous thrombosis in AAV, can improve the quality of management of these patients. Therefore, the question of the need for primary and secondary prevention of thrombosis in AAV is of great practical importance. The presented data show the need to estimate the probability of thrombosis in patients with AAV, taking into account risk factors, as well as the influence of the disease itself and ongoing therapy. Assessment of the risk of thrombosis (both venous and arterial) is necessary for the timely administration of adequate preventive treatment of thrombotic complications in AAV.

Vascular Involvement in Behcet's Disease: An Evaluation of 147 Cases and Literature Review.

Behcet's disease (BD) is characterized by systemic vasculitis with inflammation that can affect various body organs. In BD, vasculitis primarily manifests with venous involvement, distinguishing it from other forms of systemic vasculitis. We retrospectively analyzed the demographic and clinical characteristics of 147 patients diagnosed with vascular BD in our center. Vascular BD cases accounted for 25.0% (147 out of 589) of all BD patients. A statistically significant correlation was found between gender and vascular involvement that was seen predominantly in males (76.9%). In 71 patients, a vascular event developed during follow-up for BD, while in 76 patients the disease was diagnosed after the occurrence of a vascular event (51.7%). The most common vascular event was deep vein thrombosis in the lower extremities (69.4%). Arterial involvement was primarily observed in the pulmonary arteries (12.9%). Patients with lower extremity deep vein thrombosis tended to be younger, while those with pulmonary artery involvement were typically older. Overall, veins were affected 4.5 times more frequently than arteries. The prevalent type of venous involvement was deep vein thrombosis in the lower extremities. Thrombotic events in BD cannot be solely attributed to abnormalities in thrombotic factors. The treatment of thrombotic events in BD remains contentious, with anticoagulant efficacy being debated and immunosuppressive therapy representing the primary treatment approach. Behcet's disease should be considered when a young male patient presents with an arterial or venous vascular event, especially if it is recurrent.

A diagnostic score for eosinophilic granulomatosis with polyangiitis among eosinophilic disorders

Eosinophilic granulomatosis with polyangiitis (EGPA) is a form of systemic vasculitis with eosinophilic inflammation. However, existing classification criteria are all designed to classify EGPA among vasculitis and there is no established method distinguishing EGPA from other eosinophilic disorders. The aim of the present study was to propose a scoring system to differentiate EGPA among eosinophilic disorders. Non-supervised hierarchical clustering using Ward's method and principal component analysis (PCA) were performed for 19 clinical parameters of 58 patients with eosinophilia-related diseases at a tertiary university hospital. The newly proposed scoring system was externally validated in 40 patients at another tertiary institution. Two distinct clusters were identified, and clinical features including peripheral neuropathy, asthma, skin involvement, lung involvement, rheumatoid factor (RF) positivity, myeloperoxidase (MPO)-anti-neutrophil cytoplasmic antibody (ANCA) positivity, IgE elevation, C-reactive protein (CRP) elevation, and vasculitis pathological findings were predominantly observed in one of these clusters (p<0.05). Ten features defining the cluster with a high rate of vasculitis were weighted by PCA to create the E-CASE (EGPA classification among systemic eosinophilia) scoring system, on a 16-point scale. Based on the distribution of scores in the primary cohort, we defined an E-CASE score ≥12 as positive, ≤ 8 as negative, and 9-11 as undeterminable. The sensitivity and specificity of the E-CASE score in the validation cohort were 93.3% and 100%, respectively. We developed and verified a novel scoring system for differentiating EGPA from other types of eosinophilic disorders.

VEXAS Syndrome: A Case Series From a Single-Center Cohort of Italian Patients With Vasculitis.

ObjectiveTo identify patients with VEXAS syndrome (vacuoles, E1 enzyme, X‐linked, autoinflammatory, somatic syndrome) from a single‐center cohort of Italian patients with vasculitis, using a clinically oriented phenotype‐first approach.MethodsWe retrospectively reviewed the clinical records of 147 consecutive male patients followed up in our vasculitis clinic from 2013 to date. All patients with a diagnosis of vasculitis and treatment‐resistant manifestations of inflammation, persistently elevated inflammation markers, and hematologic abnormalities were identified. Bone marrow aspirates were examined for the presence of vacuoles. Sequencing of ubiquitin‐activating enzyme E1 (UBA‐1) was performed using genomic DNA from peripheral blood leukocytes or bone marrow tissue.ResultsSeven patients with vasculitis and concomitant features of VEXAS syndrome were identified. A final diagnosis of VEXAS syndrome was made in 3 of the 5 patients who underwent sequencing of UBA‐1 (diagnosis was made postmortem for 1 patient). In all 3 patients, examination of the bone marrow aspirate revealed vacuoles characteristic of VEXAS syndrome, and all 3 patients met the definitive World Health Organization criteria for myelodysplastic syndrome. Cytogenetic analysis showed normal karyotypes in all 3 patients.ConclusionTo our knowledge, this is the first report of VEXAS syndrome associated with antineutrophil cytoplasmic antibody (ANCA)–associated vasculitis. Our data emphasize the need to consider VEXAS syndrome when evaluating patients with various forms of systemic vasculitis. The novel association between VEXAS syndrome and ANCA‐associated vasculitis reported herein warrants further investigation.

Near Infrared Spectroscopy Detects Change of Tissue Hemoglobin and Water Levelsin Kawasaki Disease and Coronary Artery Lesions.

Background: Kawasaki disease (KD) is a form of systemic vasculitis that mainly affects children under the age of five years old. Limb swelling and redness are among the primary symptoms of KD. Previous studies have reported that wireless optical monitoring systems can identify limb indurations characteristics in patients with KD. Therefore, we conducted this study to monitor the dynamic changes in different stages of KD and the disease outcome of coronary artery lesions (CAL).Methods: KD patients who were admitted for intravenous immunoglobulin (IVIG) treatment and controls with or without fever were enrolled in this study. Near infrared spectroscopy data were collected for KD patients at different stages, including before (within one day before IVIG treatment, KD1) and shortly after IVIG treatment (within three days, KD2), at least 21 days after IVIG (KD3), 6 months later (KD4), 1 year later (KD5), 2 years later (KD6), and 3 years later (KD7).Results: This study included a total of 350 pieces of data, including data from 20 healthy controls, 64 fever controls, 53 KD1, 67 KD2, 58 KD3, 28 KD4, 25 KD5, 15 KD6, and 20 KD7. The relative HbO2 of the KD1 group were significantly lower than those of the healthy group (0.298 ± 0.01 vs. 0.304 ± 0.05, p = 0.028) but no significant differences were found with the fever group. The HbT concentrations of KD1 group showed significantly lower than health group (0.632 ± 0.019 vs. 0.646 ± 0.021, p = 0.001) but no significant difference with fever control. Relative levels of HbO2, HbT and Hb showed significant difference between KD1 and health control while StO2 and H2O showed difference between KD1 and fever control. The relative H2O concentration was significantly higher in KD patients with CAL formation than without (p < 0.005). Conclusion: This report is the first to use near infrared spectroscopy to detect changes in tissue hemoglobin and water levels at different stages of KD in patients and showed that water content was significantly associated with CAL formation. This non-invasive device may benefit physicians by serving for early identification of KD from fever illness.

Features of the lesion of the vascular bed in purulent meningitis in children

Background. Purulent meningitis occupies one of the important places in the structure of neuroinfectious diseases in children and is the top ten places among the causes of death. The relevance of their study is due to the severity of the course, high rates of disability and deaths (839%). Damage to the vascular bed during neuroinfections, including purulent meningitis, is a mandatory component due to the predominantly hematogenous pathway of pathogens. The penetration of microorganisms through the blood-brain barrier into the cranial cavity and their hematogenous intrathecal circulation cause damage to the cerebral veins and arteries, leading to the development of vasculopathies and vasculitis.&#x0D; Aim: To determine the features of vascular disorders in purulent meningitis in children.&#x0D; Materials and methods. 100 children with purulent meningitis were examined, aged from 1 to 17 years 11 months, for the period since 2007 to 2020. All patients underwent neurological monitoring, etiological verification of diagnoses, determination of markers of endothelial dysfunction in the blood (D-dimer and desquamated endothelial cells), as well as MRI of the brain and MRI angiography.&#x0D; Results. A complex lesion of the vascular system in purulent meningitis in children was proved, associated with both structural and functional properties of the vascular wall, as well as damage to the vasomotor function of the endothelium.&#x0D; Conclusions. With purulent meningitis, there is damage to the vascular bed in the form of systemic vasculitis, including cerebral vessels, as evidenced by the presence of both markers of endothelial damage (desquamated endothelial cells and D-dimer) and changes in MRI and MRI angiography.

Multiple intravenous antibiotics usage is associated with intravenous immunoglobulin resistance in Kawasaki disease

Kawasaki disease (KD) is a form of systemic vasculitis that primarily affects children under the age of 5 years old. Antibiotics are often prescribed for KD patients even before a diagnosis is made due to their prolonged fever and elevated inflammatory markers. Therefore, the purpose of this study was to investigate the impact of antibiotics usage on the disease outcome of KD. We carried out a retrospective chart review of children between 2005 and 2017 for initial immunoglobulin (IVIG) treatment of KD. KD children with initial IVIG treatment more than 10 days after the onset of symptoms were excluded. In total, 280 children were eligible for this study, among which 209 had been treated with antibiotic(s) and 71 had not been. The IVIG resistance rates were 5.6% (4/71), 8.9% (10/112), and 21.6% (21/97) in non-users, single-drug users, and multiple-drug users, respectively (r=0.205, p=0.003). The IVIG resistance rate of the multiple antibiotics drug users in KD patients was significantly higher than the other two groups. Furthermore, the likelihood of IVIG resistance was found to increase with elevated C-reactive protein (CRP) values (1.010/unit, p<0.001) but not with total white blood cell (WBC) count (p=0.466). The probability of IVIG resistance increases with elevated CRP values and the use of multiple IV antibiotics, thus indicating that physicians should be prudent in administering multiple IV antibiotics when treating assumed infections in KD children.

A 78-year-old female with severe tongue pain: benefit of modern ultrasound

BackgroundGiant cell arteritis (GCA) is the most common form of systemic vasculitis in persons aged 50 years and older. Medium and large vessels, like the temporal and axillary arteries, are commonly affected. Typical symptoms are headache, scalp tenderness, jaw claudication and ophthalmological symptoms as loss of visual field, diplopia or amaurosis due to optic nerve ischemia. Tongue pain due to vasculitic affection of the deep lingual artery can occur and has so far not been visualized and followed up by modern ultrasound.Case presentationWe report the case of a 78-year-old woman with typical symptoms of GCA, such as scalp tenderness, jaw claudication and loss of visual field, as well as severe tongue pain. Broad vasculitic affection of the extracranial arteries, vasculitis of the central retinal artery and the deep lingual artery could be visualized by ultrasound. Further did we observe a relevant decrease of intima-media thickness (IMT) values of all arteries assessed by ultrasound during follow-up. Especially the left common superficial temporal artery showed a relevant decrease of IMT from 0.49 mm at time of diagnosis to 0.23 mm on 6-months follow-up. This is the first GCA case described in literature, in which vasculitis of the central retinal artery and the lingual artery could be visualized at diagnosis and during follow-up using high-resolution ultrasound.ConclusionHigh-resolution ultrasound can be a useful diagnostic imaging modality in diagnosis and follow-up of GCA, even in small arteries like the lingual artery or central retinal artery. Ultrasound of the central retinal artery could be an important imaging tool in identifying suspected vasculitic affection of the central retinal artery.

The diagnosis and management of Henoch-Schönlein purpura

Henoch-Schönlein purpura is an acute self-limited illness and usually resolves without treatment, but may lead to complications. It is the most common form of systemic vasculitis in children; 90% of cases occur in childhood under the age of 10 years

A Nomogram Model Identifies Eosinophilic Frequencies to Powerfully Discriminate Kawasaki Disease From Febrile Infections.

Background: Kawasaki disease (KD) is a form of systemic vasculitis that occurs primarily in children under the age of 5 years old. No single laboratory data can currently distinguish KD from other febrile infection diseases. The purpose of this study was to establish a laboratory data model that can differentiate between KD and other febrile diseases caused by an infection in order to prevent coronary artery complications in KD.Methods: This study consisted of a total of 800 children (249 KD and 551 age- and gender-matched non-KD febrile infection illness) as a case-control study. Laboratory findings were analyzed using univariable, multivariable logistic regression, and nomogram models.Results: We selected 562 children at random as the model group and 238 as the validation group. The predictive nomogram included high eosinophil percentage (100 points), high C-reactive protein (93 points), high alanine transaminase (84 points), low albumin (79 points), and high white blood cell (64 points), which generated an area under the curve of 0.873 for the model group and 0.905 for the validation group. Eosinophilia showed the highest OR: 5.015 (95% CI:−3.068–8.197) during multiple logistic regression. The sensitivity and specificity in the validation group were 84.1 and 86%, respectively. The calibration curves of the validation group for the probability of KD showed near an agreement to the actual probability.Conclusion: Eosinophilia is a major factor in this nomogram model and had high precision for predicting KD. This report is the first among the existing literature to demonstrate the important role of eosinophil in KD by nomogram.

O05 Active Bechet's with life threatening arterial disease, complicated by concurrent COVID-19 infection at the peak of the COVID-19 pandemic: did immunosuppression help or hinder?

O05 Active Bechet's with life threatening arterial disease, complicated by concurrent COVID-19 infection at the peak of the COVID-19 pandemic: did immunosuppression help or hinder?

A novel nomogram model for differentiating Kawasaki disease from sepsis

Kawasaki disease (KD) is a form of systemic vasculitis that occurs in children under the age of 5 years old. Due to prolonged fever and elevated inflammatory markers that are found in both KD and sepsis, the treatment approach differs for each. We enrolled a total of 420 children (227 KD and 193 sepsis) in this study. Logistic regression and a nomogram model were used to analyze the laboratory markers. We randomly selected 247 children as the training modeling group and 173 as the validation group. After completing a logistic regression analysis, white blood cell (WBC), anemia, procalcitonin (PCT), C-reactive protein (CRP), albumin, and alanine transaminase (ALT) demonstrated a significant difference in differentiating KD from sepsis. The patients were scored according to the nomogram, and patients with scores greater than 175 were placed in the high-risk KD group. The area under the curve of the receiver operating characteristic curve (ROC curve) of the modeling group was 0.873, sensitivity was 0.893, and specificity was 0.746, and the ROC curve in the validation group was 0.831, sensitivity was 0.709, and specificity was 0.795. A novel nomogram prediction model may help clinicians differentiate KD from sepsis with high accuracy.

Cardiovascular Disease in the Systemic Vasculitides.

The vasculitides are a heterogeneous group of disorders, characterized by inflammatory cell infiltration and necrosis of blood vessels that cause vascular obstruction or aneurysm formation, affecting various organs such as lungs, kidneys, skin and joints. Cardiac involvement is commonly encountered in primary systemic vasculitis and it is associated with increased morbidity and mortality. Depending on the dominant pathophysiological mechanism, heart complications may manifest in different ways, including myocardial ischemia due to impaired micro- or macrovascular circulation, progressive heart failure following valvular heart disease and myocardial dysfunction, (sub) clinical myocarditis, pericarditis, pulmonary hypertension as well as arteritis of coronary vessels. Beyond cardioprotective regimens, aggressive immunosuppression reduces the inflammatory burden and modulates the progression of cardiovascular complications. Perioperative management of inflammation, when surgical treatment is indicated, improves surgical success rates and postoperative long-term prognosis. We aim to provide an overview of the pathogenetic, diagnostic and therapeutic principles of cardiovascular involvement disease in the various forms of systemic vasculitis.

Pathophysiology and therapy of systemic vasculitides.

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.

Mixed cryoglobulinemia associated with chronic HCV infection in the era of modern antiviral therapy: Can the problem be considered solved?

Mixed cryoglobulinemia is the most common extrahepatic manifestation of chronic HCV infection. The disease can occur both asymptomatically and in the form of systemic vasculitis with a wide range of clinical manifestations. The development of cryoglobulinemia is based on relatively benign lymphoproliferation, which, as pathological clones increase and their malignancy can lead to the appearance of B-cell non-Hodgkin's lymphomas. The new treatment of HCV infection with direct acting antivirals allows to achieve an increase in the rate of virus elimination. However, the clinical and, especially, immunological responses associated with the disappearance of cryoglobulins, as well as the normalization of the rheumatoid factor and C4 complement components, were significantly weaker compared to the virological response. Recently, it became known about the possible positive dynamics of these parameters in the future course, but the timing and volume of such observations are clearly insufficient. The persistence of elevated rheumatoid factor values, hypocomplementemia, and, especially, cryoglobulinemia after achieving a sustained virologic response as a result of antiviral therapy raises a number of serious questions relevant for clinical practice. Among the main issues are how often and how quickly normalization of these immunological disorders is possible? What are the consequences of their persistence? Under what conditions is it appropriate to consider alternative approaches to antiviral therapy and to use immunobiological drugs aimed at suppressing B-lymphocyte proliferation? These problems, as well as some aspects of the pathogenesis, clinical course, and treatment of extrahepatic manifestations of chronic HCV infection based on virus-induced lymphoproliferation are discussed in this literature review.

A rare case of granulomatosis with polyangiitis (Wegener’s) presenting with tracheo-bronchial involvement

Granulomatosis with polyangiitis (Wegener’s) (GPA) is a form of systemic vasculitis that involves primarily the upper and lower airways and the kidneys. Although the pulmonary involvement of GPA is well described, the lower airway findings are rarely involved, and only various descriptions have been reported. The most frequent airway manifestations include subglottic stenosis and inflammation, and tracheal and bronchial stenoses. We, hereby, report a case of GPA presenting with fever, cough and haemoptysis with bronchoscopy showing markedly inflamed bronchial mucosa.