Systemic Vasculitis Research Articles

The Expression of High Mobility Group Box-1 (HMG1) in the Peripheral Blood and Its Relation with Systemic Vasculitis Patients.

We aimed to explore the expression of high mobility group box-1 (HMG1) in the peripheral blood of systemic vasculitis (SV) patients. The peripheral blood were collected from 35 healthy controls and 35 SV patients, and the expressions of HMGB1 and pyroptosis-related markers in the samples were detected by ELISA. They were admitted to the Department of Rheumatology and Immunology of the Third Affiliated Hospital of Qiqihar Medical University, China in 2022. The severity of diseases was graded according to the diagnosis and treatment norms of SV. The correlation between HMGB1 expression level and disease-related indicators and grades were explored through Pearson correlation analysis. The specific mechanism of HMGB1 mediating the occurrence and development of diseases through the regulation of endothelial pyroptosis was clarified. HMGB1 expression significantly increased in the peripheral blood of SV patients compared with healthy controls (P<0.0001). Pearson correlation analysis indicated that HMGB1 expression level in serum gradually increased with the aggravation in SV patients. The expression levels of ASC (P<0.0001), IL-1β (P=0.004) and IL-18 (P<0.0001) in peripheral blood of SV patients were significantly increased, which were significantly positively correlated with HMGB1 in peripheral blood (P<0.0001). Recombinant HMGB1 significantly promoted the expression of ASC, IL-1β and IL-18 in vascular endothelial cells. Recombinant HMGB1 stimulation significantly activated NLRP3 inflammasome, and the additional addition of NLRP3 inhibitor significantly inhibited HMGB1-mediated endothelial pyroptosis. HMGB1 expression was significantly high in the peripheral blood of SV patients, which was positively correlated with the severity of diseases. HMGB1 could mediate pyroptosis through activating TLR4/NF-κB/NLRP3 signaling pathway.

Single-cell transcriptomic profiling reveals a pathogenic role of cytotoxic CD4+ T cells in giant cell arteritis

Giant cell arteritis (GCA) is a systemic vasculitis mediated by an aberrant immunological response against the blood vessel wall. Although the pathogenic mechanisms that drive GCA have not yet been elucidated, there is strong evidence that CD4+ T cells are key drivers of the inflammatory process occurring in this vasculitis. The aim of this study was to further delineate the role of CD4+ T cells in GCA by applying single-cell RNA sequencing and T cell receptor (TCR) repertoire profiling to 114.799 circulating CD4+ T cells from eight GCA patients in two different clinical states, active and in remission, and eight healthy controls. Our results revealed an expansion of cytotoxic CD4+ T lymphocytes (CTLs) in active GCA patients, which expressed higher levels of cytotoxic and chemotactic genes when compared to patients in remission and controls. Accordingly, differentially expressed genes in CTLs of active patients were enriched in pathways related to granzyme-mediated apoptosis, inflammation, and the recruitment of different immune cells, suggesting a role of this cell type in the inflammatory and vascular remodelling processes occurring in GCA. CTLs also exhibited a higher clonal expansion in active patients with respect to those in remission. Drug repurposing analysis prioritized maraviroc, which targeted CTLs, as potentially repositionable for this vasculitis. In addition, effector regulatory T cells (Tregs) were decreased in GCA and showed lower expression of genes involved in their suppressive activity. These findings provide further insights into the pathogenic role of CD4+ T cells in GCA and suggest targeting CTLs as a potential therapeutic option.

Vascularite systémique sévère induite par l’ibrutinib

IntroductionThe specific cutaneous toxicity of Bruton's tyrosine kinase inhibitors is poorly described. We report a case of severe systemic vasculitis induced by ibrutinib. ObservationA 73-year-old woman with chronic lymphocytic leukemia was treated with ibrutinib. Eighteen months after treatment onset, ulceronecrotic lesions on toes and tongue occurred. Skin biopsy found vasculitis of small and medium vessels. Biologic tests were negative. This vasculitis was refractory to systemic corticosteroid therapy and azathioprine. Ibrutinib was stopped on the hypothesis of drug-induced vasculitis. Skin lesions improved after discontinuation of ibrutinib. ConclusionThe mechanism of action of ibrutinib does not explain the occurrence of vasculitis and an immunoallergic mechanism is suspected.

Mycophenolate mofetil in giant cell arteritis.

Giant cell arteritis (GCA) is a systemic granulomatous vasculitis affecting the large arteries. Abnormal lymphocyte function has been noted as a pathogenic factor in GCA. Mycophenolate mofetil (MMF) inhibits inosine monophosphate dehydrogenase and is therefore a highly lymphocyte-specific immunosuppressive therapy. We aimed to assess the efficacy of MMF for inducing remission in GCA. Seven patients (5 female, 2 male) with GCA under therapy with MMF and who were treated at the outpatient clinic for rare inflammatory systemic diseases at Hannover Medical School between 2010 and 2023 were retrospectively included in the study. All patients underwent duplex sonography, 18F-fluorodeoxyglucose positron emission tomography (18F-FDG PET), magnetic resonance imaging (MRI), and/or biopsy to confirm the diagnosis. The primary endpoints were the number of recurrences, CRP levels at 3-6 and 6-12 months, and the period of remission. All patients in this case series showed inflammatory activity of the arterial vessels in at least one of the imaging modalities: duplex sonography (n = 5), 18F-FDG PET (n = 5), MRI (n = 6), and/or biopsy (n = 5). CRP levels of all patients decreased at the measurement time points 3-6 months, and 6-9 months after initiation of therapy with MMF compared with CRP levels before MMF therapy. All patients with GCA in this case series achieved disease remission. The results of the present case series indicate that MMF is an effective therapy in controlling disease activity in GCA, which should be investigated in future randomized controlled trials.

Bruton’s tyrosine kinase is a possible therapeutic target in microscopic polyangiitis

BackgroundBruton’s tyrosine kinase (Btk) is an enzyme expressed in leukocytes other than T lymphocytes and plasma cells and involved in B-cell receptor- and Fcγ receptor (FcγR)-mediated signal transduction. Btk inhibitors potentially suppress autoantibody production due to the expected inhibitory ability of B lymphocyte differentiation into antibody-producing plasma cells and reduce FcγR-mediated neutrophil activation, including the release of neutrophil extracellular traps (NETs). Microscopic polyangiitis (MPA) is a systemic small-vessel vasculitis characterized by the pathogenic autoantibody, antineutrophil cytoplasmic antibody (ANCA) that reacts with myeloperoxidase (MPO). MPO and MPO-ANCA immune complex (IC)-induced FcγR-mediated NETs are critically involved in MPA pathogenesis. This study aimed to demonstrate the therapeutic efficacy of the Btk inhibitor tirabrutinib on MPA.MethodsVarious doses of tirabrutinib or vehicle were orally administered to Sprague–Dawley rats daily. Four weeks later, the number of peripheral B lymphocytes was counted, and Btk phosphorylation in B lymphocytes was evaluated by flow cytometry. Human peripheral blood neutrophils were stimulated by MPO and anti-MPO antibody ICs (MPO and anti-MPO-ICs), and Btk and its downstream Vav phosphorylation were assessed by western blotting. The effects of tirabrutinib on MPO and anti-MPO-IC-induced NET formation were examined in vitro. Wistar Kyoto rats were immunized with human MPO to induce experimental MPA and given drug-free or tirabrutinib-containing feed (0.0037% or 0.012%) from day 0 or 28. All rats were euthanized on day 42 for serological and histological evaluation.ResultsTirabrutinib inhibited Btk phosphorylation without decreasing B lymphocytes in vivo. Neutrophil Btk and Vav were phosphorylated when stimulated with MPO and anti-MPO-ICs. Tirabrutinib suppressed MPO and anti-MPO-IC-induced NET formation in vitro and ameliorated experimental MPA in a dose-dependent manner in vivo. Although MPO-ANCA production was not affected, NET-forming neutrophils in the blood were significantly reduced by tirabrutinib.ConclusionsThe Btk inhibitor tirabrutinib suppressed MPO and anti-MPO-IC-induced NET formation in vitro and ameliorated experimental MPA by reducing NET-forming neutrophils but not decreasing MPO-ANCA titer in vivo. This study suggests that Btk is a possible therapeutic target in MPA.

Symposium 10: Spotting EGPA from Asthma Populations and How Co-Care Works Between Rheumatology and Respiratory Physicians: A Case Sharing

Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss syndrome, is a rare systemic vasculitis characterized by asthma, eosinophilia, and vasculitic organ involvement. This rare disease poses diagnostic challenges within the domain of severe asthma. Recognizing EGPA within the asthmatic cohort is therefore crucial for appropriate management and improved patient outcomes. Moreover, effective collaboration between rheumatologists and respiratory physicians is vital for the comprehensive care of EGPA patients. This scientific lecture aims to discuss the identification of EGPA in individuals with asthma and highlight the importance of co-care between rheumatology and respiratory medicine. This lecture will begin by providing an overview of EGPA, its clinical manifestations, and the challenges associated with differentiating it from severe asthma. The crucial identification of distinctive clinical features, such as refractory asthma, peripheral eosinophilia, and systemic symptoms, which serve as beacons for the presence of EGPA, will be discussed. Diagnostic tools encompassing imaging, laboratory tests, and biopsy techniques are explored, focusing on their utility in distinguishing EGPA from asthma. Concomitantly, the lecture will then shift its focus to the collaborative efforts required from rheumatologists and respiratory physicians in managing EGPA patients. The complementary roles of both specialties in achieving optimal patient outcomes will be discussed. Rheumatologists play a crucial role in the diagnosis and treatment of systemic vasculitis, including EGPA, while respiratory physicians provide expertise in managing asthma and respiratory complications. The case will highlight the importance of open communication, shared decision-making, and coordinated treatment plans to ensure comprehensive care for EGPA patients, addressing both their rheumatologic and respiratory needs. Real-life case will be shared to illustrate successful co-management models between rheumatology and respiratory medicine. These examples will underscore the benefits of a multidisciplinary approach, including improved disease management, reduced treatment-related complications, and enhanced patient satisfaction. In conclusion, identifying EGPA within the asthma population is crucial for appropriate management, and effective collaboration between rheumatology and respiratory physicians is essential for comprehensive care. This scientific lecture aims to provide insights into spotting EGPA among asthma patients and emphasize the significance of co-care between these specialties, ultimately improving patient outcomes and quality of life.

Postoperative acute kidney injury after on-pump cardiac surgery in patients with connective tissue disease.

Patients with connective tissue disease have a poor prognosis after receiving cardiac surgery. This study described the clinical scenarios and investigated factors correlated with acute kidney injury (AKI) after on-pump cardiac surgery in patients with systemic lupus erythematosus (SLE) or vasculitis. Patients with SLE or vasculitis who underwent on-pump cardiac surgery from March 2002 to March 2022 were enrolled, while patients with preoperative renal dysfunction were excluded. AKI was defined according to the Kidney Disease: Improving Global Outcomes (KDIGO) criteria. Uni- and multivariable analyses were performed to identify potential factors associated with postoperative AKI. Among 123 patients enrolled, 39 (31.7%) developed AKI within seven days after receiving on-pump cardiac surgery. Four patients died in the hospital, resulting in an overall in-hospital mortality of 3.3%, and all deaths occurred in the AKI group. Patients in the AKI group also had longer ICU stays (median difference 3.0 day, 95% CI: 1.0-4.0, P < 0.001) and extubation time (median difference 1.0 days, 95% CI: 0-2.0, P < 0.001) than those in the non-AKI group. Multivariable logistic regression revealed that BMI over 24 kg/m2 (OR: 3.00, 95% CI: 1.24-7.28) and comorbid SLE (OR: 4.73, 95% CI: 1.73-12.93) were independently correlated with postoperative AKI. Factors potentially correlated with AKI following on-pump cardiac surgery in patients with connective tissue disease were explored. Clinicians should pay more attention to preoperative evaluation and intraoperative management in patients with risk factors.

Cardiac Mass and Cerebrovascular Accident as Primary Manifestations of Churg-Strauss Syndrome.

Churg-Strauss syndrome (CSS), recently named eosinophilic granulomatosis with polyangiitis (EGPA), is a rare form of systemic vasculitis with extravascular granulomas occurring in patients with asthma and tissue eosinophilia. We represent a large left ventricular granuloma, confirmed by histopathologic evaluation, detected as a ventricular mass by echocardiography in a 45-year-old asthmatic male who was admitted for a cerebrovascular accident. Paraclinical and histopathologic findings confirmed the diagnosis of EGPA. As cardiac involvement in patients with EGPA is associated with poor prognosis, routine echocardiographic evaluation of these patients is suggested.

Dilemma in diagnosing incomplete Kawasaki disease in a resource limited setting

Introduction and importance: Kawasaki disease (KD) is an acute febrile systemic vasculitis that predominantly affects small to medium sized vessels and mostly occurs in children below 5 years of age. The morbidity and mortality mostly occur due to cardiac involvement. Case presentation: The authors present a case of a 5-year-old male child from hilly region of Nepal who presented with fever for 7 days along with strawberry tongue and non-exudative conjunctivitis without rashes, extremity changes or lymphadenopathy. A suspicion of incomplete KD (IKD) was made. The notable investigation findings were increased erythrocyte sedimentation rate, C-reactive protein, leucocyte count and platelets. Echocardiography showed normal findings. Based on the clinical features and supplemental laboratory findings, a diagnosis of IKD was made. The patient improved after intravenous immunoglobulin and Aspirin. Clinical discussion: The main learning objective that the authors get from this case is the challenges in the diagnosis of IKD in the resource limited setting like Nepal. Whether or not to start intravenous immunoglobulin is a dilemma for the physician in most of the cases of suspected IKD, due to the high cost and poor availability of intravenous immunoglobulin in this setting. Hence, the use of inflammatory markers, supplemental laboratory findings together with the few diagnostic criteria met by the patient helps in making a diagnosis and institute timely treatment with intravenous immunoglobulin and aspirin. Conclusion: Diagnosis of KD in difficult in resource limited setting.

Risk factors, clinical features and treatment of Behçet's disease uveitis.

Behçet's disease is a systemic vasculitis frequently associated with intraocular inflammation. Recent findings identified independent clinical clusters in Behçet's disease, each involving distinct combinations of affected organs. Ocular Behçet's disease, mainly manifested as uveitis, is characterized as an independent cluster with a low likelihood of association with other system involvements, such as intestinal, cardiovascular, or central nervous system. A prevailing theory suggests that the pathogenesis of the disease is multifactorial, where a variety of genetic and infectious agents may interact with each other to cause the disease. Among sporadic cases, the human leukocyte antigen (HLA) genes, including HLA-B51, HLA-A26, HLA-B15, and HLA-B5701, have been found to be a key component conferring genetic susceptibility. Outside the HLA region, a set of susceptibility variants are identified, closely related to interleukin (IL)-23/IL-17 pathway, tumor necrosis factor (TNF) signaling, and pattern recognition receptor systems. Microbial infections, such as Streptococcus sanguinis, Mycobacterium tuberculosis, and Herpes simplex virus (HSV), are linked to play the triggering of disease in immunogenetically predisposed individuals. Clinically, due to the notable relapsing-remitting course of ocular Behçet's disease, the prevention of recurrent attack would be the primary treatment goal. Combination of corticosteroids and immunomodulatory drugs, such as anti-TNF agents, interferon, and conventional immunosuppressants (e.g. cyclosporine, azathioprine), have been the mainstream regimen for the disease. Future research may focus on comparing the effectiveness of immunomodulatory drugs and identifying the most suitable subgroups for a specific drug on the basis of the knowledge of the molecular heterogeneity of the disease.

Incidental Detection of Systemic Vasculitis in A Sarcoidosis Patient on FDG PET/CT scan: A Case Report

Sarcoidosis is a rare disease with variable clinical presentation as it can involve different organ systems. Systemic vasculitis is a rare presentation of extra pulmonary sarcoidosis that can be fatal if undiagnosed. FDG PET/CT scan is a great modality in detecting both pulmonary and extrapulmonary manifestation of sarcoidosis. In this case report, we present a case of a 47-year-old lady with underlying pulmonary sarcoidosis stage 2 with sick sinus syndrome, who underwent FDG PET/CT scan to rule out cardiac sarcoidosis. The 18F-FDG PET/CT scan showed FDG avid disease in the lungs, lymph nodes and the vessels, suggestive of systemic vasculitis. If undiagnosed and untreated, it may lead to development of aneurysm and dissection. Immunosuppressive treatment using steroids and cytotoxic therapy can be life-saving in this condition and may lead to remission in some of the patients.

Особливості сучасного перебігу гранулематозу з поліангіїтом (гранулематозу Вегенера) у дітей на прикладі клінічного випадку в пацієнта підліткового віку

The article introduces readers to a rare disease from the group of systemic vasculitis - Wegener’s granulomatosis. The prevalence of this disease in European countries ranges from 4.9 to 10.5 cases per 1 million adult population. A feature of the disease is the pathology of the upper respiratory tract at the beginning of the inflammatory process, which leads to long and difficult treatment of patients by otolaryngologists, as a result of which the correct diagnosis is delayed and the prognosis of the disease worsens. The disease is especially rare among children. Purpose - to share the experience of diagnosis and treatment of a teenager with this extremely rare pathology. Complications of drug therapy and ways to solve them are considered in detail. The conclusion of this experience is an understanding of the severity of the course of the disease and the need to use aggressive therapy already in its early stages in order to save the patient’s life. The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent of the parents and the child was obtained for the study. No conflict of interests was declared by the authors.

Beyond the symptoms: Personalizing giant cell arteritis care through multidimensional patient reported outcome measure

BackgroundGiant Cell Arteritis (GCA) is the commonest form of systemic vasculitis in people over the age of 50. Published research highlighted the lack of a disease-specific patient reported outcomes (PROMs) for GCA. ObjectivesTo assess the validity, reliability and responsiveness to change of a devised disease specific patient self-reported outcome measures questionnaire for Giant Cell Arteritis (GCA). MethodsThe GCA-PROMs was conceptualized based on frameworks outlined in the OMERACT developed core set of Outcome Measures for Large-Vessel Vasculitis and the guiding principles of the FDA guidance. Initially, cognitive interviews were conducted to identify item pool of questions. Item selection and reduction was achieved based on patients as well as an interdisciplinary group of specialists. Rasch and internal consistency reliability analyses were implemented. ResultsA total of 54 GCA patients completed the questionnaire. The GCA-PROMs questionnaire was reliable as demonstrated by a high standardized alpha (0.878–0.983). Content construct assessment of the GCA-PROMs functional disability and QoL revealed significant correlation (p< 0.01) with both HAQ and EQ-5D. Changes in functional disability, QoL showed significant (p< 0.01) variation with diseases activity status in response to therapy. ConclusionsThe developed GCA-PROMs questionnaire is a reliable and valid instrument for assessment of GCA patients. A stratified treatment regimen depending on the individual patient's risk factors as well as preferences and associated comorbidities is the best approach to tailored patient management.

Refractory drug-induced systemic small-vessel vasculitis with two varied extracutaneous manifestations: a case report and review of the literature

BackgroundClopidogrel and ticagrelor are rarely reported to cause vasculitis via drug hypersensitivity reaction, largely mediated by T cells and immunoglobulin E (IgE). Despite therapeutic advances, the etiology of refractory vasculitides remains incompletely understood. Recently, (non)immunological mechanisms bypassing T cells and IgE have been proposed to explain resistance to standard immunosuppressants. Herein, we report a case of refractory drug-induced systemic small-vessel vasculitis with varied extracutaneous manifestations and incorporate multiple sources of data to provide detailed accounts of complex (non)immunological phenomena involved in this case. Study objectives are to provide an insight about rare presentations of commonly used drugs, upgrade the pathophysiological concepts of drug-induced vasculitis, raise need for further investigation to define causes and risk factors for refractory vasculitis, and discuss most of the current knowledge suggesting novel therapeutic approaches to treat this vasculitis. To our knowledge, this is the first case of the two flares of systemic small-vessel vasculitis in a single patient in response to clopidogrel and ticagrelor exposure, respectively. However, this report is limited by attribution/observer bias.Case presentationWe herein report a 24-year-old Caucasian male student with a medical history of mild seasonal allergic rhinoconjunctivitis, tension-type headaches, posttraumatic arterial stenosis, and previous exposure to ibuprofen, acetylsalicylic acid, and mRNA coronavirus disease 2019 (COVID-19) vaccine who suffered largely from acute urticaria and dyspnea after 20 days of acetylsalicylic acid and clopidogrel introduction. A skin punch biopsy confirmed leukocytoclastic vasculitis. Serologic antibody testing, complement analysis, microbiologic testing, and cancer biomarkers revealed no abnormalities. Regarding the patient’s medical history, both acetylsalicylic acid and clopidogrel were exchanged for ticagrelor. Furthermore, the addition of naproxen, cyclosporine, bilastine, prednisolone, and montelukast resulted in complete recovery. After 7 days, diarrhea and hematuria occurred. Urinalysis and computed tomography showed reversible proteinuria with gross hematuria and hypodense changes in kidney medulla, respectively, associated with discontinuation of ticagrelor and naproxen. In addition, the patient recovered completely without any immunosuppression up-titration.ConclusionsThis case highlights the role of clopidogrel and ticagrelor as possible triggering agents for systemic small-vessel vasculitis and offers an insight into novel therapeutic strategies for refractory vasculitides. Further research is needed to build on the findings of a current report.

Frequency and course of COVID-19 in patients with rheumatic diseases (according to the data of V.A. Nasonova Research Institute of Rheumatology)

The aim of the study was to assess frequency and severity of COVID-19 in patients with rheumatic diseases (RD) who were on inpatient treatment at the V.A. Nasonova Research Institute of Rheumatology. Patients and methods. The study included information on the presence or absence of COVID-19 in the medical history of 6911 patients with immunoinflammatory RD (IIRD) and 362 patients with osteoarthritis (OA) who were on inpatient treatment at the V.A. Nasonova Research Institute of Rheumatology from September 21, 2021 to April 28, 2023. Results. The incidence of COVID-19 in the analyzed IIRD was significantly higher compared to OA (p&lt;0.001). All IIRD included in the analysis are characterized by an increased risk of COVID-19 incidence when compared with OA by 2.7–6.3 times. Patients with rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, microcrystalline arthritis, Sjögren’s disease, systemic sclerosis, systemic lupus erythematosus, and ANCA-associated vasculitis were significantly more likely (p&lt;0.02) to receive COVID-19 therapy compared with the control group. Patients with these diseases are characterized by an increased risk of treatment for COVID-19 by 1.9–3.7 times compared with OA. Also, patients with inflammatory joint diseases (IJD), connective tissue diseases (CTDs) and systemic vasculitis (SV) were hospitalized with COVID-19 more often than patients with OA (p=0.01, p=0.007 and p=0.024, respectively). Patients with IJD, CTDs and SV are characterized by an increased risk of hospitalization with COVID-19 by 4.3– 4.7 times compared with OA. In addition, elderly patients with IIRD are characterized by an increasing risk of treatment, hospitalization and use of biologics or targeted synthetic disease-modifying drugs for COVID-19. Conclusion. According to the results obtained, the problem of COVID-19 is significant for patients with RD, which dictates need for further research on vaccination against SARS-CoV-2 among this cohort of patients.

Rituximab in the treatment of Graves’ disease and endocrine ophthalmopathy. Opportunities and limitations

Rituximab is a chimeric (mouse and human) monoclonal antibody against B-lymphocytes (CD20). This drug is widely used in rheumatology in the treatment of rheumatoid arthritis, Sjogren’s syndrome, some systemic connective tissue diseases and vasculitis, as well as in hematology in lymphoproliferative diseases. Administration of rituximab leads to depletion of B-lymphocytes through various mechanisms, including antibody-dependent and complementdependent cytotoxic effects, as well as the regulation of apoptosis. Considering the mechanism of autoimmune damage in Graves’ disease, an autoimmune thyroid disease accompanied by thyrotoxicosis and endocrine ophthalmopathy (an autoimmune lesion of the accessory apparatus of the eye), the use of rituximab may be effective in these diseases. The review considers the currently available results of studies on the use of various doses of rituximab in these diseases.

New ACR/EULAR 2022 classification criteria for ANCA-associated vasculitis

The new 2022 classification criteria for antineutrophil cytoplasmic antibodies associated vasculitis (AAV), proposed by the American College of Rheumatology (ACR) and the European Alliance of Associations for Rheumatology (EULAR), was an important stage in the development of the doctrine of systemic vasculitis. Based on the analysis of large international cohorts, the criteria for granulomatosis with polyangiitis and eosinophilic granulomatosis with polyangiitis were revised, and classification criteria for microscopic polyangiitis were proposed for the first time. The creation’s history of the AAV classification criteria is discussed over the past three decades, and their limitations are considered. It should be noted, the ACR/EULAR 2022 criteria were developed for creating homogeneous groups of patients for scientific studies and cannot be widely used in clinical practice for the various AAV nosological forms diagnosis, primarily at early stage of the disease or mono organ damage (for example, kidneys). As before, decisive importance in AVV diagnostic belong to a detailed clinical analysis and a complete examination of patients to identify pathognomonic symptoms, including asymptomatic ones.

Primary central nervous system vasculitis: Description based on Chapel Hill Conference Consensus 2012 and a case report

Primary central nervous system vasculitis: Description based on Chapel Hill Conference Consensus 2012 and a case report

Zone 2/3 lesion and emergency repair as potential mortality predictors of TEVAR for thoracic aortic pseudoaneurysm

ObjectiveThoracic aortic pseudoaneurysm (TAP) is an uncommon but life-threatening condition. The present study aimed to investigate the early and midterm clinical outcome of TAP patients following TEVAR and identify potential mortality predictors.MethodsWe retrospectively reviewed a series of 37 eligible patients with TAP admitted to our hospital from July 2010 to July 2020. We explored their baseline, perioperative and follow-up data. Fisher exact test and Kaplan–Meier method were applied for comparing difference between groups.ResultsThere were 29 men and 12 women, with the mean age as 59.5 ± 13.0 years (range 30–82). The mean follow-up period was 30.7 ± 28.3 months (range 1–89). For early outcome (≤ 30 days), mortality happened in 3 (8.1%) zone 3 TAP patients versus 0 in zone 4 (p = 0.028); postoperative acute arterial embolism of lower extremity and type II endoleak respectively occurred in 1(2.7%) case. For midterm outcome, survival at 3 months, 1 year and 5 years was 88.8%, 75.9% and 68.3%, which showed significant difference between zone 2/3 versus zone 4 group (56.3% vs. 72.9%, p = 0.013) and emergent versus elective TEVAR group (0.0% versus 80.1%, p = 0.049). Previous stent grafting or esophageal foreign body with Aortoesophageal fistula (AEF), and systemic vasculitis, as etiologies, resulted in encouraging immediate outcome but worse midterm prognosis.ConclusionTAP lesions at zone 2/3 and emergent TEVAR predict worse midterm outcomes compared to zone 4 lesions and elective TEVAR. The outcomes are also mainly restricted by the etiology of the TAP.

Experience of IVIG Treatment in an Overweight 14-year-old Child With Kawasaki Disease: A Case Report

Kawasaki disease, or mucocutaneous lymph node syndrome, is an acute systemic vasculitis involving small and medium-sized vessels. It can be complicated by varying degrees of cardiac damage, especially coronary artery disease. The disease mainly occurs in children aged < 5 years, with rarer cases in older children and adults. Intravenous immunoglobulin combined with aspirin is the widely accepted treatment regimen in the acute phase, but the dosage recommended by the American Heart Association guidelines is not suitable for heavier children. This article reports the successful management of an overweight 14-year-old child with Kawasaki disease.