Type Of Vasculitis Research Articles

Decreased Absolute Number of Circulating Regulatory T Cells in Patients With Takayasu's Arteritis.

BackgroundTakayasu’s arteritis (TA) is a type of primary large vessel vasculitis. Th1, Th17, and Tfh cells have been reported to be associated with TA relapse. However, the relationship between regulatory T cells (Tregs) and TA remains unclear.ObjectiveTo analyze the levels of circulating lymphocytes, especially Treg cells (CD4+CD25+FOXP3+ T cells) and serum cytokines in TA patients and explore their relationship with their changes and TA disease activity.MethodsA total of 57 TA patients and 43 sex- and age-matched healthy controls (HCs) were enrolled. According to NIH standards, 36 patients had active disease status. Flow cytometry combined with counting was used to detect the absolute numbers and ratios of Th1, Th2, Th17, and Treg cells in the peripheral blood of all the subjects. Magnetic bead-based multiplex immunoassay was used to detect cytokines.ResultsCompared to HCs, the absolute number and proportion of peripheral Treg cells in TA patients was significantly decreased, while Th17 cells were significantly increased. Furthermore, compared to the inactive group, the TA active group had significantly increased levels of interleukin (IL)-6, IL-10, and tumor necrosis factor (TNF)-α, but lower IL-10 levels. The absolute number of Th2 cells was negatively associated with platelet (PLT) and NIS scores in TA patients. The proportion of Th2 cells was negatively associated with the erythrocyte sedimentation rate in TA patients. After treatment, Treg cells were markedly increased.ConclusionThere was a Th17-Treg cell imbalance with a significant reduction in peripheral Treg cells and an increase in Th17 cells in TA patients compared to the HCs. The levels of IL-6, IL-10, IL-17, and TNF-α appeared to be related to disease activity.

Doppler signs of extravaginal testicular torsion mimicked by small vessel intra-testicular vasculitis in henoch-schoenlein purpura: A case report

Henoch-Schoenlein Purpura (HSP) is one of the most common types of multi-systemic vasculitis seen in childhood with “classic triad” of purpura, arthritis, and abdominal pain. Scrotal disease including scrotal swelling, epididymitis, and orchitis are very rare complications of HSP that can mimic the symptoms and signs of testicular torsion, which is one of the major surgical emergencies in children. Radiological modalities are commonly used to diagnose possible causes of acute scrotum such as testicular torsion. Here we report an 8-year-old boy, complaining of acute pain and swelling in his left testicle. Although colour Doppler ultrasonography of his testes showed increased-size parenchymal echo in the left testicle and its epididymis, with no detectable blood flow on the same side, immediate surgical exploration indicated normal pulse in both testicles’ arteries, with no signs of torsion. Finally, the pathological specimen results were consistent with the diagnosis of HSP-related testicular involvement. In conclusion, the occurrence of small vessel intra-testicular vasculitis in Henoch-Schoenlein Purpura can mimic clinical and Doppler ultrasonic signs of extravaginal testicular torsion.

Pattern of prescription drugs used for patients diagnosed with systemic vasculitis

IntroductionVasculitis is a heterogeneous group of diseases characterized by inflammation of the blood vessel wall, which can cause thrombosis, stenosis, or occlusion. Its pharmacological management involves corticosteroids and conventional and biological disease-modifying antirheumatic drugs. ObjectivesThe objective was to determine the different types of systemic vasculitis and their pharmacological treatment in a group of patients from Colombia, 2019–2020. Materials and methodsThis was a cross-sectional study that identified different types of systemic vasculitis and their pharmacological management in a group of patients from a drug-dispensing database of approximately 8.5 million people. Sociodemographic, comorbidity and pharmacological variables were considered. A descriptive analysis was performed. ResultsA total of 621 patients with a diagnosis of systemic vasculitis were identified. The patients had a median age of 55.0 years, and 74.4% were women. The most common vasculitis types were those limited to the skin (30.1%), granulomatosis with polyangiitis (17.6%), and necrotizing vasculopathy (10.8%). A total of 81.0% of cases received corticosteroid prescriptions, 44.0% received azathioprine, and 24.0% received methotrexate; only 1.6% were prescribed biological antirheumatic drugs. Cardiovascular diseases were the most common comorbidity. ConclusionsThe pattern of prescription medications used for patients diagnosed with systemic vasculitis is heterogeneous. Much of this population has associated comorbidities, which increases the use of medications according to the current management guidelines.

Cardiac phenotypes in the acute-phase of microscopic polyangiitis involves dilatation of the left atrium caused by LV diastolic dysfunction

Abstract Background Microscopic polyangiitis (MPA) is a type of primary systemic vasculitis that affects various organs, especially the lungs and kidneys. However, few reports regarding cardiac features of MPA patients were found. Purpose We aim to investigate the echocardiographic parameters of acute-phase MPA. Methods and results This single-center retrospective study included 15 patients with MPA (Mean age at 72.2±7.1 years, women 73.3%) who underwent echocardiography within two weeks of commencing steroid therapy for induction or reinduction. The echocardiography parameters of the patients were compared with those of 30 age and sex-matched controls. In the MPA group, the commonly affected organs were kidneys (93.3%) and lungs (46.7%); 5 patients (33.3%) had a history of hypertension, which had a similar frequency as the control group. No significant difference in left ventricular (LV) diameter, LV ejection fraction, e', or inferior vena cava diameter was observed between the two groups. However, the MPA group showed significantly higher left atrial (LA) diameter (p=0.033) and LA volume index (p=0.001), as well as higher early diastolic filling velocity (E-wave, p=0.015; E/A, p=0.043; E/e', p=0.041), diastolic pulmonary venous flow velocity (p=0.013), trans-tricuspid pressure gradient (p=0.019), and shorter deceleration time (p=0.038), associated with mildly thicker ventricular walls of left ventricle (LV) than the control group. Moreover, serum levels of C-reactive protein showed significant correlation between E wave (r=0.58, p=0.023), E/A (r=0.67, p=0.006), and deceleration time (r=−0.69, p=0.005) in the MPA group. These results may indicate that in MPA, increased LV stiffness, rather than impairment of LV relaxation was contributed to LV diastolic function, resulting in LA enlargement. Conclusion Patients with acute-phase MPA had LA dilatation associated with LV diastolic dysfunction. This finding indicates the importance of cardiac assessment in patients with MPA. Funding Acknowledgement Type of funding sources: None.

Гастроинтестинальные проявления системных васкулитов: спектр клинических симптомов, диагностика и лечение

Системные васкулиты представляют собой гетерогенную группу заболеваний, которые характеризуются воспалением стенок кровеносных сосудов и классифицируются в зависимости от типа и размера вовлеченных в патологический процесс сосудов. Гастроинтестинальная система может быть вовлечена в патологический процесс при первичных и вторичных васкулитах. Поражение желудочно-кишечного тракта (ЖКТ) чаще всего встречается при IgA-васкулите, АНЦА-ассоциированных васкулитах, узелковом полиартериите и синдроме Бехчета. Исходами воспалительного поражения сосудов являются аневризмы или окклюзии в брыжеечных, печеночных или селезеночных сосудах с развитием воспаления, эрозий и/или язв, которые могут привести к перфорации, кровотечению и непроходимости желудка и кишечника и быть важной причиной заболеваемости и смертности у пациентов с васкулитами. Клинические признаки поражения ЖКТ при системных васкулитах обычно неспецифичны, к ним относятся лихорадка, боль в животе, тошнота, рвота, диарея и кровотечение. Диагностика базируется главным образом на визуализации при помощи катетерной ангиографии, компьютерной томографической и/или магнитно-резонансной ангиографии, эндоскопии с проведением гистопатологического исследования. Лечение поражения ЖКТ зависит от типа васкулита и обычно включает применение высоких доз глюкокортикоидов, иммунодепрессантов, таких как циклофосфамид, азатиоприн или микофенолата мофетил, и биологических препаратов. В данном обзоре литературы представлены данные о патофизиологии, клинических данных, диагностике, лечении и исходе поражения ЖКТ при различных типах системных васкулитов.

The "Ant-farm"-like Appearance of Restricted Lower Limb Vasculitis on Fluorodeoxyglucose-positron Emission Tomography.

Restricted lower limb vasculitis is a type of localized muscle vasculitis limited to the lower limbs. The usefulness of fluorodeoxyglucose-positron emission tomography (FDG-PET) for the diagnosis of this entity has not yet been reported. We herein report three patients with a fever and persistent lower limb pain. FDG-PET revealed linear and patchy FDG uptakes in their lower limbs. Combined with magnetic resonance imaging and histological findings, they were diagnosed with lower limb vasculitis. Linear and patchy FDG uptakes are considered to reflect the presence of muscle vasculitis. The characteristic “ant-farm”-like FDG-PET images can be a diagnostic clue for the currently overlooked vasculitis.

The Joint Vasculitis Registry in German-speaking countries (GeVas) \u2013 a prospective, multicenter registry for the follow-up of long-term outcomes in vasculitis

BackgroundVasculitides comprise a group of rare diseases which affect less than 5 in 10.000 individuals. Most types of vasculitis can become organ- and life-threatening and are characterized by chronicity, high morbidity and relapses, altogether resulting in significant morbidity and mortality. Previous studies have been either monocentric or mainly retrospective – studies with a prospective design mostly consisted of rather small cohorts of 100 to 200 patients.The aim of the Joint Vasculitis Registry in German-speaking countries (GeVas) is to record all patients who have been recently diagnosed with vasculitis or who have changed their treatment due to a relapse (inception cohort). In GeVas, data are collected prospectively in a multicenter design in Germany, Austria and Switzerland. By this approach, courses of vasculitis and their outcomes can be monitored over an extended period.MethodsGeVas is a prospective, web-based, multicenter, clinician-driven registry for the documentation of organ manifestations, damage, long-term progress and other outcomes of various types of vasculitis. The registry started recruiting in June 2019. As of October 2020, 14 centers have been initiated and started recruiting patients in Germany. Involvement of sites in Austria and the German-speaking counties of Switzerland is scheduled in the near future.DiscussionIn June 2019, we successfully established a prospective multicenter vasculitis registry being the first of its kind in German-speaking countries. The participating centers are currently recruiting, and systematic analysis of long-term vasculitis outcomes is expected in the ensuing period.Trial registrationGerman Clinical Trials Register (Deutsches Register Klinischer Studien): DRKS00011866. Registered 10 May 2019.

Pulmonary artery thrombosis as the first presentation of Beh\xe7et\u2019s syndrome: a\xa0case report and review of the\xa0literature

BackgroundBehçet’s syndrome is a type of systemic chronic vasculitis of unknown etiology, frequently characterized by recurrent oral and genital ulcers and uveitis. It is less commonly characterized by arthritis and skin, vascular, and gastrointestinal involvements. Behçet’s syndrome affects various sizes of vessels by perivascular infiltration and vasculitis. Unlike other classic types of vasculitis, Behçet’s syndrome patients can present with both arterial and venous involvement. Although vascular Behçet’s syndrome is found in only around 15% of Behçet’s syndrome patients, it is the major cause of morbidity and mortality among them. Furthermore, although deep venous thrombosis has high incidence in Behçet’s syndrome patients, pulmonary artery thrombosis is an uncommon complication. Combining the findings of this and previous case reports of pulmonary artery thrombosis in Behçet’s syndrome patients, we sought to determine the best treatment options for pulmonary artery thrombosis in Behçet’s syndrome patients.Case presentationWe present the case of a 22-year-old Arabian male who was admitted to an emergency department with acute chest pain, dyspnea, and hemoptysis for 2 weeks. He gave a long history of recurrent oral and genital ulcers for the last 4 months but without seeking medical advice. Spiral computed tomography showed arterial filling defects with a pulmonary nodule for which the presence of a pulmonary artery aneurysm ruled out. The lung perfusion scan showed multiple pulmonary perfusion defects. After excluding common infectious diseases such as tuberculosis and brucellosis, a diagnosis of Behçet’s syndrome with pulmonary artery thrombosis was made. Steroids with enoxaparin were initiated. The patient was discharged later on prednisolone (tapering dose) with adalimumab and apixaban. He was on regular follow-up for the next 9 months.ConclusionsVascular involvement in Behçet’s syndrome is a major contributor to morbidity and mortality of Behçet’s syndrome patients. Consequently, early detection of vascular involvement has a major impact on the prognosis of patients with Behçet’s syndrome.

Surgical Repair of a Ruptured Giant Abdominal Aortic Aneurysm in a 16-Year-Old with Takayasu’s Arteritis: Case Report and Etiological Review

Takayasu’s arteritis is a type of primary systemic vasculitis that affects medium and large arteries, including the aorta and its main branches, as well as the pulmonary and coronary arteries. Although rare in children, it is the third most common vasculitis in the pediatric population, often with delayed diagnosis due to the nonspecific presentation of clinical symptoms in its initial phase. This is a case of a 16-year-old girl with a giant ruptured abdominal aortic aneurysm, who needed surgery [...]

A rare case of Polyarteritis Nodosa associated with autoimmune hepatitis: a case report

BackgroundPolyarteritis nodosa is a type of vasculitis affecting medium- and small-sized arteries that has been associated with hepatitis B but does not have an established relationship with autoimmune hepatitis. Here we report the case of an adult patient with autoimmune hepatitis who, shortly after diagnosis, developed life-threatening polyarteritis nodosa.Case presentationA 45-year-old woman was diagnosed with autoimmune hepatitis after initially presenting with a two-month history of fatigue, nausea, and anorexia and a three-week history of scleral icterus. Her liver biopsy showed mild portal fibrosis and her liver chemistries improved with prednisone and azathioprine. Three months later, she presented to the emergency department with fever, bilateral ankle pain, rash, oral ulcers, and poor vision. Physical examination was notable for erythema nodosum, anterior uveitis, retinal vasculitis, and frosted branch angiitis (frosted branch angiitis (a widespread florid translucent perivascular exudate). She subsequently developed repeated episodes of ischemic acute bowel necrosis that required multiple surgeries and extensive small bowel resections. Surgical pathology of the small bowel resection revealed ischemic necrosis, medium and small vessel vasculitis with microvascular thrombi consistent with polyarteritis nodosa. Azathioprine was discontinued and she was treated with pulse steroids followed by a prednisone taper, cyclophosphamide, and intravenous immune globulin with overall improvement in her symptomatology. Since her hospitalization, she has been maintained on low-dose prednisone and mycophenolate mofetil.ConclusionsIn patients with recent diagnosis of autoimmune hepatitis, there should be a modest suspicion for concomitant polyarteritis nodosa if symptoms and signs of multisystem vasculitis develop.

Identification of neutrophil \u03b22-integrin LFA-1 as a potential mechanistic biomarker in ANCA-associated vasculitis via microarray and validation analyses

BackgroundLeukocyte activation by anti-neutrophil cytoplasmic antibody (ANCA) and the subsequent leukocyte–endothelium interaction play a key role in the development of endothelial damage in ANCA-associated vasculitis (AAV). In contrast to that of leukocyte activation, the exact role of the leukocyte–endothelium interaction via integrin remains unclear. Here, we performed microarray and validation analyses to explore association between the expression levels of lymphocyte function-associated antigen-1 (LFA-1) and the clinical characteristics of patients with AAV.MethodsWe performed gene set enrichment analysis (GSEA) to identify the functional gene sets differentially expressed between patients with AAV and other types of vasculitis and the healthy controls (HCs). Flow cytometry was performed to validate the GSEA results. Treatment-naïve patients were monitored until 24 weeks of treatment. To examine the role of LFA-1 in the neutrophil–endothelium interaction, we performed a leukocyte adhesion and transmigration assay using peripheral blood and human umbilical vein endothelial cells (HUVECs).ResultsGSEA revealed that the molecular pathways involving integrin-related genes were significantly upregulated in patients with AAV compared to that in patients with other types of vasculitis and the HCs. Flow cytometry revealed that the percentage of neutrophils expressing LFA-1 was significantly higher in patients with AAV than in those with large-vessel vasculitis or polyarteritis nodosa and the HCs. LFA-1 levels in the neutrophils were higher in patients with MPO-ANCA-positive expression than in those with a positive PR3-ANCA expression and correlated with the peripheral eosinophil count, serum rheumatoid factor titre, serum C-reactive protein levels, and the vasculitis activity score of systemic and chest components. After 24 weeks of treatment, including prednisolone, cyclophosphamide, rituximab, azathioprine, methotrexate, and/or tacrolimus, neutrophil LFA-1 expression remained high in the non-responder patients, but decreased in the responder patients. The in vitro assay showed that leukocyte migration toward HUVECs was dependent on the interaction between LFA-1 and intercellular adhesion molecule-1 (ICAM1); the migration of leukocytes was inhibited by blocking the adhesion of LFA-1 to ICAM1.ConclusionsThe expression of LFA-1 in neutrophils is increased in patients with AAV. Neutrophil LFA-1 levels correlate with the clinical features of AAV. Inhibiting the adhesion of LFA-1 and ICAM1 impedes the neutrophil–endothelium interaction and may have a therapeutic role in AAV.

Mönckeberg sclerosis with giant cells as a masquerade of giant cell arteritis.

Giant cell arteritis (GCA) is the most common type of vasculitis in adults, which is classified as a large/medium vessel vasculitis. It has a predilection for the ophthalmic circulation and extracranial carotid system. Temporal artery biopsy specimens can show the presence of inflammatory multinucleated giant cells. Here, we report just the third case of Mönckeberg sclerosis with multinucleated giant cells affecting the temporal artery and mimicking GCA. This rare finding in the evaluation of a common vasculitis is important for rheumatologists to be aware of and emphasizes close collaboration between clinicians and pathologists.

Vessel wall MR imaging of central nervous system vasculitis: a systematic review.

Beyond vessel wall enhancement, little is understood about vessel wall MR imaging (VW-MRI) features of vasculitis affecting the central nervous system (CNS). We reviewed vessel wall MR imaging patterns of inflammatory versus infectious vasculitis and also compared imaging patterns for intracranial versus extracranial arteries of the head and neck. Studies reporting vasculitis of the CNS/head and neck and included MR imaging descriptions of vessel wall features were identified by searching PubMed, Scopus, Cochrane, Web of Science, and EMBASE up to June 10, 2020. From 6065 publications, 115 met the inclusion criteria. Data on study characteristics, vasculitis type, MR details, and VW-MRI descriptions were extracted. Studies used VW-MRI for inflammatory (64%), infectious (17%), or both inflammatory and infectious vasculitides (19%). Vasculitis affecting intracranial versus extracranial arteries were reported in 58% and 39% of studies, respectively. Commonly reported VW-MRI features were vessel wall enhancement (89%), thickening (72%), edema (10%), and perivascular enhancement (16%). Inflammatory vasculitides affecting the intracranial arteries were less frequently reported to have vessel wall thickening (p = 0.006) and perivascular enhancement (p = 0.001) than extracranial arteries. Varicella zoster/herpes simplex vasculitis (VZV/HSV, 45%) and primary angiitis of the CNS (PACNS, 22%) were the most commonly reported CNS infectious and inflammatory vasculitides, respectively. Patients with VZV/HSV vasculitis more frequently showed decreased or resolution of vessel wall enhancement after therapy compared to PACNS (89% versus 59%). To establish imaging biomarkers of vessel wall inflammation in the CNS, VW-MRI features of vasculitis accounting for disease mechanism and anatomy should be better understood.

Primary Central Nervous System Vasculitis

Primary central nervous system (CNS) vasculitis is an uncommon disorder of unknown etiology that is restricted to the brain and spinal cord. Patients do not present with specific clinical features or a classical clinical course, and no blood or imaging investigations are available for diagnostic confirmation. Cerebral biopsy and angiography are the gold standards for diagnosis. No randomized clinical trials have described a therapeutic regimen effective for primary CNS vasculitis; therefore, treatment of primary CNS vasculitis is based on therapeutic strategies used for other types of vasculitis. Early diagnosis is important because corticosteroid-based treatment with or without concomitant cyclophosphamide administration can often prevent serious outcomes and may be followed by a favorable response. Several immunosuppressants such as mycophenolate mofetil, tumor necrosis factor-α blockers, and rituximab may be useful options for patients refractory to the aforementioned regimen. We describe some diagnostic and therapeutic approaches for the management of this condition, with a focus on the importance of obtaining tissue and angiographic evaluation (formal contrast-enhanced or magnetic resonance/computed tomography angiography).

Vasculitis

Vasculitis is a group of clinicopathological entities involving inflammation of the blood vessel walls. The different forms of vasculitis may vary in terms of aetiology, pathogenesis, type, and calibre of vessel involved, type of inflammation, preferential organ distribution, clinical manifestations, genetic predisposition, and demographic characteristics. The classification most widely used currently is based on the nominal definitions proposed by the International Chapel Hill Consensus Conference (CHCC) in its 2012 revision. The aetiology of vasculitis is still not fully understood, despite recent advances in the study of genetics and pathogenesis. Due to the variability of vasculitis, there is no single, uniform diagnostic method. Diagnosis of vasculitis is challenging in most cases. However, a detailed clinical history, careful physical examination and appropriate laboratory tests from the information obtained from them are necessary in all types of vasculitis to establish a correct diagnosis, its probable date and manner of onset, the organs or systems affected and the extent of disease.

P091 Can use of high cost drugs for rare rheumatic diseases be reliably identified within routinely collected NHS data? Results from a pilot study of rituximab use in vasculitis using data from the National Disease Registration Service (NDRS) and the Registration of Complex Rare Diseases - Exemplars in Rheumatology (RECORDER) project

Abstract Background/Aims Understanding real-world usage of high cost drugs and subsequent outcomes are crucial to support high quality care, adoption of innovation, and reduce unwarranted variation in treatment. Hospital Episode Statistics (HES) contain diagnoses (coded by ICD-10) and procedures/treatment (coded by OPCS) for admissions in England. However, OPCS codes are not specific for individual drugs, for example X921 (cytokine inhibitors band 1) includes rituximab and 15 other drugs. We aimed to validate the accurate identification of patients treated with rituximab for ANCA-associated vasculitis (AAV) using HES data. Methods We used data available to the National Congenital Anomaly and Rare Disease Registration Service (NCARDRS), part of NDRS at Public Health England and their legal permissions (CAG 10-02(d)/2015). We extracted from HES all patients treated at one hospital who ever had a coded diagnosis of either granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, microscopic polyangiitis, polyarteritis nodosa or 'arteritis unspecified’, which is applied by coders to several clinical terms including “systemic vasculitis” and “ANCA associated vasculitis”. Enabled by data sharing agreements, we reviewed hospital records of those patients who had a Finished Consultant Episode (FCE) during 2018/19, to validate diagnoses and whether X921 reliably identified rituximab. Results Initially using codes only for granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, or microscopic polyangiitis we identified 65 people, 60 of whom had AAV confirmed in their medical notes (Positive predictive value (PPV) 92.3%; 95% CI 83.0-97.5). When we expanded our search to include in addition people with codes for polyarteritis nodosa or ‘arteritis unspecified’ who had rheumatology or renal follow up, we identified an additional 13 people with AAV and 10 who had other types of systemic vasculitis (PPV for AAV 79.5, 95% CI 69.6-87.4; PPV for systemic vasculitis 90.9%, 95% CI 82.9-96.0). Among patients identified only by the specific ICD-10 codes for the 3 subtypes of AAV there were 51 episodes coded as X921, of which 50 correctly identified a rituximab infusion (and 1 was tocilizumab). No additional infusions for these patients were missed by the coding. The PPV was 98.0% (95% CI 89.6-100). Among patients identified by the more inclusive algorithm, there were 61 episodes coded as X921 in that year. Of these, 59 were for rituximab treatment for AAV and an additional 4 rituximab infusions occurred but were not coded. The PPV was 96.7% (95% CI 88.7-99.6), and the sensitivity was 93.7% (95% CI 84.5-98.2). Conclusion This pilot study demonstrates how the use of novel algorithms within the NDRS RECORDER project can enable the identification and registration of people with AAV and their high-cost treatment at whole-population level. Further work is now underway to study this national cohort, and to apply this methodology to other rare autoimmune diseases and high cost drugs. Disclosure M. Chakravorty: None. F. Pearce: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. M. Rutter: None. P. Lanyon: Grants/research support; Recipient of research grant from Vifor Phama. Vifor Pharma had no influence on the design, interpretation or reporting of this work. J. Aston: None. M. Bythell: None. S. Stevens: None.

POS-138 SEVERE INFECTIONS IN ANTINEUTROPHIL CYTOPLASMIC ANTIBODY-ASSOCIATED VASCULITIS: CLINICO-BACTERIOLOGICAL PROFILE AND RISK FACTORS

Infections are the leading cause of morbidity and mortality in patients with Antineutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis (AAV) especially during the first year of follow-up. Indeed, patients with AAV are at high risk of infection because of immunodepression secondary to the vasculitis itself and to immunosuppressive therapies. Few studies have looked at this subject in our country. The aim of our study is to determine the clinical biological and bacteriological profile as well as the factors of gravity of severe infections during AAV. It was a retrospective monocentric study carried out in the department of internal medicine A at Charles Nicolle Hospital including patients followed for AAV between January 1973 and December 2016 who presented at least one severe infection. An infection was considered severe if it required hospitalization or intravenous antibiotic therapy or if it resulted in death. 68 patients were followed for AAV during the study period. 18 patients had at least one severe infection, that was 26.5% of all patients with AAV. The mean age at diagnosis was 50 +/-14 years. The sex ratio was 1. 39% of patients were hypertensive 22% diabetics and 50% smokers. The subtypes of VAA were: nine cases of microscopic polyangiitis, eight cases of granulomatosis with polyangiitis and one case of eosinophilic granulomatosis with polyangiitis. ANCA were positive in all of our patients. Renal involvement was noted in all cases, kidney biopsy was performed in 72% of cases. VAA treatment included corticostreroids cyclophosphamid mycophenolate mofetil and Rituximab. We have identified 55 infections, the median was three infections per patient [2-3,25]. The first infection occurred 41 days [1-443] after diagnostic confirmation and 30.5 days [-9 - 443] after the start of immunosuppressive therapy. Two of our patients died from infection before receiving any immunosuppressive treatment. The other patients received at least one immunosuppressive treatment before the first infection. The most frequent infectious sites were pulmonary (29% of cases) and urinary (20%). 74% of infections were bacterial 20% fungal and 6% viral. Microbiological documentation was possible in 55% of cases. Opportunistic infections accounted for 26.7% of documented infections. Eight patients received cotrimoxazole pneumocystosis prophylaxis. Seven deaths from septic shock resulted in an infection mortality of 10.3%.Age, comorbidities, type of vasculitis, the type of immunosuppressive therapy lymphopenia and the location of infection did not have a statistically significant relationship with death from septic shock. However, lymphopenia and plasma exchange were associated with hospitalization in intensive care. Infections are a common and serious complication during AAV. Preventive measures should be adopted such as anti-pneumocystosis prophylaxis but also the generalization of anti-pneumococcal vaccination and antiviral prophylaxis.

Anuria as a Chief Presentation of Takayasu Arteritis, a Case Report

Takayasu vasculitis is a rare type of large vessel vasculitis that primarily affects the aorta and its main branches; signs and symptoms are usually due to systemic inflammation or ischemia of an organ or limb, depending on the group of blood vessels involved. In addition, Takayasu arteritis is associated with increased platelet and coagulation activity, leading to a hypercoagulable state and thrombus formation. We report a case of a 47-year-old male who presented with a history of complete anuria for 3 days and was found to have progressively worsening kidney function. Renal Doppler ultrasound confirmed the presence of bilateral renal artery thrombosis, while Contrast- Enhanced Computed Tomography (CECT) of the abdomen and pelvis showed extensive abdominal aortic thrombosis with radiological findings consistent with large vessel vacuities. After catheter-directed thrombolytic therapy of the renal arteries, the patient started producing urine and his kidney function significantly improved. Later, Positron Emission Tomography scan (PET) confirmed large vessel Takayasu arteritis. Echocardiography showed no intracardiac thrombus, along with an extensive work up for thrombophilia, as autoimmune and vasculitis serology came back negative. This is an extremely rare presentation of Takayasu arteritis, with an unusual recovery of acute renal failure after delayed anuria due to bilateral renal artery thrombosis.

COVID-19 vasculitis and novel vasculitis mimics.

COVID-19 has been occasionally linked to histologically confirmed cutaneous vasculitis and a Kawasaki-like vasculitis, with these entities generally having minimal or no lung involvement and a good prognosis. Unlike these vasculitis types, patients with severe COVID-19 pneumonia can develop cutaneous vasculitis-like lesions and systemic arterial and venous thromboemboli, including cryptogenic strokes and other vasculopathy features. Proposed underlying mechanisms for these severe manifestations have encompassed immune dysregulation, including an anti-phospholipid syndrome-like state, complement activation, viral dissemination with direct systemic endothelial infection, viral RNAaemia with immunothrombosis, clotting pathway activation mediated by hypoxaemia, and immobility. In this Viewpoint, we highlight how imaging and post-mortem findings from patients with COVID-19 indicate a novel thrombosis in the pulmonary venous territory distal to the alveolar capillary bed, a territory that normally acts as a clot filtration system, which might represent an unappreciated nidus for systemic microembolism. Additionally, we suggest that this mechanism represents a novel vasculitis mimic related to COVID-19 that might lead to cryptogenic strokes across multivessel territories, acute kidney injury with haematuria, a skin vasculitis mimic, intestinal ischaemia, and other organ ischaemic manifestations. This finding is supported by pathological reports of extensive pulmonary venular thrombosis and peripheral organ thrombosis with pauci-immune cellular infiltrates. Therefore, severe COVID-19 pneumonia with extensive pulmonary intravascular coagulopathy might help to explain the numerous systemic complications of COVID-19, in which the demonstration of direct organ infection has not adequately explained the pathology.

Profile of pediatric vasculitides – Prospective hospital-based data from eastern India

Background: The pediatric vasculitides are multisystem autoimmune disorders involving the blood vessels of varying sizes. Wide geographic and ethnic variations are noted in terms of incidence and clinical features.Materials and Methods: This prospective, observational study was carried out over a period of 4 years from 2015 February to 2019 February in a tertiary care referral hospital to delineate the epidemiological pattern, clinical features, and pathological characteristics of pediatric vasculitides. All consecutive patients with vasculitis defined by the American College of Rheumatology and Chappel Hill Consensus Criteria diagnostic criteria were included in the study. They underwent relevant investigations and were treated as per the standard guidelines.Results: Of the total 110 patients with different types of vasculitis, 90% (n = 99) had primary vasculitis and the rest had secondary (10%, n = 11). Immunoglobulin A vasculitis (Henoch–Schonlein Purpura) (HSP) (n = 53, 48.19%) and Kawasaki disease (KD) (n = 35, 31.82%) were the most frequent types of the vasculitis followed by Takayasu arteritis and polyarteritis nodosa. Secondary vasculitides included those associated with systemic lupus erythematosus, juvenile idiopathic arthritis, scrub typhus infection, and drug penicillamine. Various typical and atypical features along with a definite seasonal pattern of their prevalence had been documented.Conclusion: A profile of pediatric vasculitides with a specific trend in their prevalence and seasonal pattern has been documented. HSP and KD constitute the predominant types of pediatric vasculitides. KD, although diagnosed more often than previous, HSP still outnumbers KD in Eastern India.