6,568 results match your criteria vasculitis early

Retinoic acid receptor responder1 promotes development of glomerular diseases via the Nuclear Factor-κB signaling pathway.

Kidney Int 2021 Jun 17. Epub 2021 Jun 17.

KI/AZ Integrated Cardio Metabolic Centre, Division of Pathology, Department of; Laboratory Medicine. Electronic address:

Inflammatory pathways are activated in most glomerular diseases but molecular mechanisms driving them in kidney tissue are poorly known. We identified retinoic acid receptor responder 1 (Rarres1) as a highly podocyte-enriched protein in healthy kidneys. Studies in podocyte-specific knockout animals indicated that Rarres1 was not needed for the normal development or maintenance of the glomerulus filtration barrier, and did not modulate the outcome of kidney disease in a model of glomerulonephritis. Read More

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[Treatment of exudative age-related macular degeneration: Consensus of French experts for first-line treatment selection and the importance of long-term risk/benefit ratio].

J Fr Ophtalmol 2021 Jun 16. Epub 2021 Jun 16.

Service d'ophtalmologie, centre hospitalier intercommunal de Créteil, université Paris Est Créteil, 40, avenue de Verdun, 94000 Créteil, France; Centre ophtalmologique du Panthéon, Paris, France. Electronic address:

Choosing a first-line treatment to optimize long-term outcomes is a major challenge for treating patients with neovascular age-related macular degeneration (AMD). The development of several new molecules makes it critical to identify the relevant factors to consider so as to provide an optimal risk-benefit ratio when initiating a treatment in naïve patients with neovascular AMD. This paper proposes a consensus established with the Delphi method (which includes a gradation in a consensus based on an analysis of the convergence rate of answers) to provide criteria that guide the ophthalmologist's decision for treatment initiation and follow-up in neovascular AMD patients. Read More

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Coronary (peri)-arteritis in patients with IgG4-related disease: A case series from the Central Anatolia Region of Turkey.

Int J Rheum Dis 2021 Jun 17. Epub 2021 Jun 17.

Division of Rheumatology, Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey.

Objective: Immunoglobulin G4-related disease (IgG4-RD) is a newly recognized fibro-inflammatory disease which affects many systems, as well as the cardiovascular system. Identifying the coronary involvement like periaortitis, coronary periarteritis and pericarditis is important, as they often cause unfavorable outcomes.

Methods: Eighty-one patients with IgG4-RD were retrospectively evaluated for symptomatic coronary artery involvement from Hacettepe University Vasculitis Research Center (HUVAC) database. Read More

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Catching Takayasu Early: Diagnosing the "Pulseless" Disease in a Child with Palpable Pulses.

Case Rep Pediatr 2021 27;2021:8885944. Epub 2021 May 27.

Gandhi Medical College and Hospital, Secunderabad 500003, India.

Takayasu arteritis is a systemic vasculitis of large vessels that mainly involves the aorta and its branches. It normally presents in the third decade of life and is rarely seen in children. We report a case of childhood Takayasu arteritis, in a 12-year-old girl, who presented with abdominal pain and hypertension. Read More

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Characterisation of an enhanced preclinical model of experimental MPO-ANCA autoimmune vasculitis.

J Pathol 2021 Jun 14. Epub 2021 Jun 14.

Centre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, Hammersmith Campus, Du Cane Road, London, W12 0NN, UK.

Experimental autoimmune vasculitis (EAV) is a model of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) induced by immunisation of susceptible rat strains with myeloperoxidase (MPO). Animals develop circulating MPO-ANCA, pulmonary haemorrhage and glomerulonephritis, although renal injury is mild and recovers spontaneously without treatment. In this study we aimed to augment the severity of glomerulonephritis. Read More

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Reactive oxygen species: The Yin and Yang in (auto-)immunity.

Autoimmun Rev 2021 Jun 9;20(8):102869. Epub 2021 Jun 9.

Division of Rheumatology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA 02115, USA.

Reactive oxygen species (ROS) are produced by immune cells in response to antigens. They are produced mostly in the mitochondria and their levels are tightly controlled by a series of metabolic processes. ROS are necessary for the development of the immune response but the role of ROS in the development of autoimmune disease needs further clarification. Read More

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Mortality and cause of death in patients with AAV/PAN in Australia-a population-based study.

Rheumatology (Oxford) 2021 Jun 12. Epub 2021 Jun 12.

School of Medicine, University of Western Australia, Perth, Australia.

Objectives: We compared survival and causes of death in Western Australian (WA) anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) and polyarteritis nodosa (PAN) patients with controls and the WA population.

Methods: In this data linkage study, we identified patients with incident AAV/PAN and age, sex and temporally matched controls 1980 - 2014 from the WA Rheumatic Disease Epidemiological Registry. Survival analyses and time-varying analyses were performed. Read More

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Epidemiology of Kawasaki Disease in Europe.

Maryam Piram

Front Pediatr 2021 25;9:673554. Epub 2021 May 25.

Department of Pediatrics, Research Centre of the Sainte Justine University Hospital, Sainte Justine University Hospital, University of Montreal, Montreal, QC, Canada.

To review major epidemiological aspects of Kawasaki disease (KD) in Europe, describing demographic characteristics, revising its incidence along with time trends and geographic variations, and describing migration studies to provide clues about its etiology. The annual incidence of KD in Europe is about 10-15 per 100,000 children under 5 years old and seems to be relatively stable over time and space. Demographic characteristics are in line with those in other countries of the world, with a higher incidence in children from Asia and possibly North African origin. Read More

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Cryopyrin-associated periodic syndrome: a treatable genetic inflammatory condition.

Pract Neurol 2021 Jun 10. Epub 2021 Jun 10.

Department of Neurology, Royal Hobart Hospital, Hobart, Tasmania, Australia

A 20-year-old man presented with recurrent subdural haemorrhages on a background of progressive sensorineural hearing loss, juvenile idiopathic arthritis and intracranial hypertension of unknown cause. His mother had a similar previous history. They both had a persistently mildly elevated serum C reactive protein. Read More

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[Giant Cell Arteritis. Recommendations in Primary Care].

Semergen 2021 May-Jun;47(4):256-266. Epub 2021 Jun 8.

Centro de Salud Comillas, Madrid, España.

Giant cell arteritis is a systemic vasculitis with significant intra and extracranial involvement that, with early diagnosis and treatment in primary care, can improve its prognosis as it is a medical emergency. Our working group on vascular diseases of the Spanish Society of Primary Care Physicians (SEMERGEN) proposes a series of recommendations based on current scientific evidence for a multidisciplinary approach and follow-up in primary care. Read More

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Giant cell arteritis: what is new in the preclinical and early clinical development pipeline?

Expert Opin Investig Drugs 2021 Jun 9. Epub 2021 Jun 9.

Department of Rheumatology, St. James's Hospital, Dublin, Republic of Ireland.

Introduction: Giant Cell Arteritis (GCA) is the most common systemic vasculitis worldwide. For decades, glucocorticoids have represented the mainstay of treatment, at the expense of toxic systemic effects owing to prolonged courses of high dose treatment regimens. The search for effective drugs permitting lower glucocorticoid treatment regimens in GCA has been a frustrating one. Read More

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Very Early-Onset Inflammatory Bowel Disease (VEO-IBD) Presenting with Recurrent Leukocytoclastic Vasculitis Preceded by Streptococcal Pharyngitis.

Case Rep Pediatr 2021 20;2021:1996430. Epub 2021 May 20.

Department of Pediatric Gastroenterology, Hepatology, and Nutrition, Nicklaus Children's Hospital, Miami, FL, USA.

Inflammatory bowel disease (IBD) that presents in children <6 years of age is known as very early-onset IBD (VEO-IBD). Extraintestinal manifestations in IBD, such as erythema nodosum (EN), pyoderma gangrenosum (PG), and, less likely, leukocytoclastic vasculitis (LV), are more commonly present in Crohn's disease. Association between LV and ulcerative colitis (UC) is not commonly seen. Read More

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Clinical, Laboratory Features and Clinical Courses of Patients with Wiskott Aldrich Syndrome and X-linked Thrombocytopenia-A single center study.

Immunol Invest 2021 Jun 7:1-12. Epub 2021 Jun 7.

Institute of Child Health, Division of Immunology, Hacettepe University Medical School, Ankara, Turkey.

: Wiskott Aldrich Syndrome is an X-linked primary immunodeficiency disorder characterized by microthrombocytopenia, severe immunodeficiency, and eczema. To define clinical-laboratory features, genetic defects (known/novel) of 23 patients of Wiskott Aldrich Syndrome/X-linked Thrombocytopenia (WAS/XLT) cohort, establish relationships between molecular defects and clinical features if present, evaluate patients who underwent hematopoietic stem cell transplantation (HSCT) and did not. Qualitative analysis from patients' hospital files and Sanger sequencing for molecular diagnosis was performed. Read More

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Renal Involvement as Rare Acute Tubulointerstitial Nephritis in a Patient with Eosinophilic Disorder Treated with Early Add-on Administration of Mepolizumab.

Intern Med 2021 Jun 5. Epub 2021 Jun 5.

Department of Internal Medicine, Teikyo University School of Medicine, Japan.

A 39-year-old man presented with peripheral eosinophilia, pulmonary eosinophilic infiltrate, and renal failure due to acute tubulointerstitial nephritis (TIN). He had experienced childhood asthma and was negative for anti-neutrophil cytoplasmic antibody (ANCA). He was tentatively diagnosed with ANCA-negative eosinophilic granulomatous polyangiitis (EGPA) or idiopathic hypereosinophilic syndrome (HES). Read More

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A Case Report of SAVI Mimicking Early-Onset ANCA Vasculitis.

J Clin Immunol 2021 Jun 5. Epub 2021 Jun 5.

Pediatric Allergy-Immunology, Ann & Robert H. Lurie Children's Hospital of Chicago, 225 East Chicago Avenue, Box #60, Chicago, IL, 60611, USA.

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Scurvy and Tinea Corporis Simulating Leukocytoclastic Vasculitis.

Am J Dermatopathol 2021 Jun 2. Epub 2021 Jun 2.

Cockerell Dermatopathology, Dallas, TX; Icahn School of Medicine at Mount Sinai, New York, NY; Division of Rheumatic Diseases, UT Southwestern Medical Center, Dallas, TX; Department of Internal Medicine, UT Southwestern Medical Center, Dallas, TX; Department of Pathology, Veterans Affairs North Texas Health Care System, Dallas, TX; Department of Pathology, UT Southwestern Medical Center, Dallas, TX; Dermatology and Skin Surgery Clinic, Alexandria, LA; Department of Dermatology, UT Southwestern Medical Center, Dallas, TX; and Rheumatology Section, Veterans Affairs North Texas Health Care System, Dallas, TX.

Abstract: Leukocytoclastic vasculitis (LCV) is a small vessel inflammatory condition considered to be caused by circulating immune complexes and often occurs after an acute infection or exposure to a new medication, although it may be associated with an underlying systemic disease or be idiopathic in nature. It is important to determine the etiology, identify the extent of the disease for early intervention and appropriate management, and treat and/or eliminate the underlying cause. Here, we report cases of scurvy and tinea corporis that presented with histopathologic features of LCV and had significant clinical improvement with treatment of the underlying etiologies. Read More

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Cognitive development of children with Kawasaki disease and the parenting stress of their caregivers in Taiwan: a case-control study.

BMJ Open 2021 06 3;11(6):e042996. Epub 2021 Jun 3.

Department of Pediatrics and Kawasaki Disease Center, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, Kaohsiung, Taiwan

Objective: Kawasaki disease (KD) is an acute form of febrile vasculitis that occurs in early childhood. The multisystemic vasculitis common in patients with KD may influence blood perfusion in the brain, and thus caregivers of children with KD may feel stress with regard to caring for them. Intravenous immunoglobulin (IVIG) infusion is the standard treatment for acute KD, and the most serious complication of KD is coronary artery aneurysms (coronary artery lesion (CAL)). Read More

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Efficacy and safety of low-dose corticosteroid with azathioprine in the treatment of elderly-onset IgA vasculitis with nephritis.

BMJ Case Rep 2021 Jun 2;14(6). Epub 2021 Jun 2.

Department of Nephrology, Tokyo Metropolitan Geriatric Hospital, Itabashi-ku, Japan.

The diagnosis of elderly-onset IgA vasculitis (IgAV) and its prognosis can be difficult to ascertain because of its rarity and the frequent presence of comorbidities. Furthermore, the treatment of elderly-onset IgAV remains controversial. We report a case of IgAV in an 87-year-old patient. Read More

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Old and New Challenges in Uveitis Associated with Behçet's Disease.

J Clin Med 2021 May 26;10(11). Epub 2021 May 26.

Department of Ophthalmology, IHU FOReSIGHT, Sorbonne-AP-HP, Groupe Hospitalier Pitié-Salpêtrière, F-75013 Paris, France.

Behçet's disease (BD) is a systemic vasculitis disease of unknown origin occurring in young people, which can be venous, arterial or both, classically occlusive. Ocular involvement is particularly frequent and severe; vascular occlusion secondary to retinal vasculitis may lead to rapid and severe loss of vision. Biologics have transformed the management of intraocular inflammation. Read More

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Differential Diagnosis of Chorea-HIV Infection Delays Diagnosis of Huntington's Disease by Years.

Brain Sci 2021 May 27;11(6). Epub 2021 May 27.

Department of Neurology, Huntington Center North Rhine-Westphalia, Ruhr-University Bochum, St. Josef-Hospital Bochum, Gudrunstraße 56, 44791 Bochum, Germany.

Background: There is a broad range of potential differential diagnoses for chorea. Besides rare, inherited neurodegenerative diseases such as Huntington's disease (HD) chorea can accompany basal ganglia disorders due to vasculitis or infections, e.g. Read More

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Behçet Disease: An Update for Dermatologists.

Am J Clin Dermatol 2021 Jul 1;22(4):477-502. Epub 2021 Jun 1.

Department of Dermatology and Venereology, Akdeniz University School of Medicine, 07059, Antalya, Turkey.

Behçet disease (BD) is a chronic, relapsing, systemic vasculitis of unknown etiology with the clinical features of oral and genital ulcers, cutaneous vasculitic lesions, ocular, articular, vascular, gastrointestinal, neurologic, urogenital and cardiac involvement. BD usually appears around the third or fourth decade of life. Gender distribution is roughly equal. Read More

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Recognition and Management of Cutaneous Connective Tissue Diseases.

Med Clin North Am 2021 Jul;105(4):757-782

Autoimmune Skin Disease Program, Department of Dermatology, Brigham and Women's Hospital, Harvard Medical School, 221 Longwood Avenue, Boston, MA 02115, USA. Electronic address:

Connective tissue diseases (CTDs) encompass a broad spectrum of clinical presentations that involve multidisciplinary management. Cutaneous findings are common in CTD and careful examination of these features aids in appropriate diagnosis and subsequent evaluation. Thorough work-up of CTD is crucial to properly identify disease subtypes and systemic involvement. Read More

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Prevasculitic Eosinophilic Granulomatosis With Polyangiitis.

Cureus 2021 Apr 23;13(4):e14649. Epub 2021 Apr 23.

Critical Care Medicine, University of Pittsburgh Medical Center Mercy, Pittsburgh, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is an exceptionally rare systemic necrotizing vasculitis. The disease is clinically characterized by asthma with concomitant blood and tissue eosinophilia, often progressing to eosinophilic vasculitis. From the onset of asthma, there is usually a three to nine year delay of EGPA diagnosis. Read More

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Isolated sixth nerve palsy as an initial presentation of primary angiitis of the central nervous system.

Brain Dev 2021 May 27. Epub 2021 May 27.

Department of Pediatrics, Kyoto University Graduate School of Medicine, Kyoto, Japan.

Background: Primary angiitis of the central nervous system (PACNS) is a newly-emerging disease, and it is known that early diagnosis with treatment is important for the improvement of prognosis.

Case Description: Here, we report the case of a previously healthy 13-year-old girl who presented with right eye abduction failure, attributed to isolated right sixth nerve palsy, as the initial symptom of PACNS. Magnetic resonance angiography (MRA) showed stenosis in the distal portion of the right internal carotid artery, and delay alternating with nutation for tailored excitation (DANTE)-prepared contrast-enhanced magnetic resonance imaging confirmed vasculitis at the same site. Read More

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Adult-onset autoinflammation caused by somatic mutations in UBA1: A Dutch case series of VEXAS patients.

J Allergy Clin Immunol 2021 May 25. Epub 2021 May 25.

Department of Rheumatology and Clinical Immunology, University Medical Center Groningen, University of Groningen, The Netherlands. Electronic address:

Background: A novel autoinflammatory syndrome was described recently in male patients who harboured somatic mutations in the X-chromosomal UBA1 gene. These patients were characterized by adult-onset, treatment-refractory inflammation with fever, cytopenias, dysplastic bone marrow, vacuoles in myeloid and erythroid progenitor cells, cutaneous and pulmonary inflammation, chondritis and vasculitis, abbreviated as VEXAS.

Objective: This study aimed to (retrospectively) diagnose VEXAS in patients that had been previously registered with unclassified autoinflammation. Read More

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Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood.

Clin J Am Soc Nephrol 2021 May 26. Epub 2021 May 26.

G Montini, Department of Clinical Sciences and Community Health, University of Milan, Fondazione IRCCS Ca' Granda - Ospedale Maggiore Policlinico , Milan, Italy.

Background And Objectives: Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare in children. We report the clinico-pathological features, long-term outcomes, and prognostic factors of a large paediatric cohort of patients with ANCA-associated kidney vasculitis.

Design, Setting, Participants, And Measurements: This retrospective study included 85 consecutive patients with kidney biopsy-proven ANCA-associated vasculitis followed at tertiary referral centres in Italy and Canada. Read More

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Early critical cortical infarction by anti-angiotensin II type 1 receptor antibody: A case report and literature review.

Medicine (Baltimore) 2021 May;100(21):e25958

Department of Internal Medicine.

Rationale: Anti-angiotensin II type 1 receptor antibodies (AT1R-Abs) have been demonstrated to increase the risk of antibody-mediated rejection. We report a case of AT1R-Ab mediated rejection which caused early critical cortical infarction.

Patient Concerns: A 52-year-old man with end-stage kidney disease underwent preemptive kidney transplantation (KT) from his wife. Read More

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IgG-related disease: an atypical presentation of steroid-responsive renal mass.

BMJ Case Rep 2021 May 24;14(5). Epub 2021 May 24.

Internal Medicine, Hospital Sultan Ismail, Johor Bharu, Malaysia

IgG-related disease (IgG-RD) is a systemic fibroinflammatory disease characterised by dense lymphoplasmacytic infiltration rich in IgG-positive plasma cells, storiform fibrosis and obliterative phlebitis. Serum IgG levels are typically elevated but half of the patients had normal serum IgG levels. IgG-RD represents a spectrum of diseases that involve various organs such as the pancreas, liver, kidneys, and salivary glands often manifesting as diffuse organ enlargement or a mass-like lesion mimicking cancer. Read More

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Takayasu's Arteritis in a 33-Year-Old Male.

Cureus 2021 Apr 19;13(4):e14557. Epub 2021 Apr 19.

Internal Medicine, Army Medical College, Rawalpindi, PAK.

Takayasu's arteritis (TA), commonly referred to as "pulseless" disease, is a large-vessel inflammatory vasculitis most commonly affecting the aorta and its major branches. Due to its irregular nature, it has the propensity to involve any organ system thus leading to a wide spectrum of clinical features. Most patients affected by TA are females in their second or third decades of lives. Read More

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Takayasu's arteritis presenting as acute myocardial infarction: case series and review of literature.

Cardiol Young 2021 May 21:1-4. Epub 2021 May 21.

Congenital Heart Center, Department of Paediatrics, University of Florida, Gainesville, FL, USA.

This series describes three adolescent females who presented with chest pain and ventricular dysfunction related to acute coronary ischemia secondary to Takayasu's arteritis with varied courses of disease progression leading to a diverse range of therapies including cardiac transplantation. While Takayasu's arteritis is rare in childhood, it should be strongly considered in any adolescent female presenting with systemic inflammation and chest pain consistent with myocardial infarction. A high index of suspicion can lead to early detection and aggressive management of the underlying vasculitis reducing associated morbidity and mortality. Read More

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