3,759 results match your criteria necrotizing vasculitis

Splenic Infarcts and Pulmonary Renal Syndrome in a Young Patient with Double-Positive Anti-GBM and ANCA-Associated Vasculitis.

Saudi J Kidney Dis Transpl 2021 Jan-Feb;32(1):240-244

Department of Medicine, King Abdulaziz Medical City, Ministry of the National Guard- Health Affairs, Riyadh, Saudi Arabia.

Double-positive disease, defined by double-seropositivity for serum anti-glomerular basement membrane (GBM) antibodies and anti-neutrophil cytoplasmic antibodies (ANCA) is a rare cause of pulmonary-renal syndrome. Here, we present an exceptional course of a 20-year-old male with seropositivity for anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies and anti-GBM antibody, who presented first with renal impairment due to focal necrotizing crescentic glomerulonephritis. After receiving treatment, he presented two years later with a relapse manifesting with diffuse alveolar hemorrhage and multiple splenic infarcts. Read More

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ANCA-Negative Pauci-immune Necrotizing Glomerulonephritis: A Case Series and a New Clinical Classification.

Am J Kidney Dis 2021 Jun 10. Epub 2021 Jun 10.

Department of pathology, Center Hospitalier Universitaire de Nantes, Nantes; Centre de Recherche en Transplantation et en Immunologie, UMR 1064, INSERM, Université de Nantes. Electronic address:

Rationale & Objective: Pauci-immune necrotizing glomerulonephritis (PING) is usually associated with the presence of anti-neutrophil cytoplasm antibodies (ANCA). However, a minority (2-3%) of patients with PING do not have detectable ANCA. We aimed to assess the clinical spectrum and outcome of patients with ANCA-negative PING. Read More

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ANCA-associated vasculitis after COVID-19.

Rheumatol Int 2021 Jun 7. Epub 2021 Jun 7.

Division of Nephrology, Department of Internal Medicine, Ondokuz Mayıs University Medical Faculty, Korfez Mahallesi, 55270, Atakum/Samsun, Turkey.

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) are systemic autoimmune diseases that may lead to renal failure due to the infiltration of mononuclear cells and the destruction of small- and medium-sized blood vessels. It has been shown that severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) may trigger the presentation or exacerbation of autoimmune diseases. Crescentic glomerulonephritis (GN) has rarely been reported in patients with Coronavirus disease-2019 (COVID-19). Read More

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Rheumatoid Arthritis Associated Episcleritis and Scleritis: An Update on Treatment Perspectives.

J Clin Med 2021 May 14;10(10). Epub 2021 May 14.

Department of Ophthalmology, Hospital Charles Nicolle, 76000 Rouen, France.

Episcleritis and scleritis are the most common ocular inflammatory manifestation of rheumatoid arthritis. Rheumatoid arthritis (RA) accounts for 8% to 15% of the cases of scleritis, and 2% of patients with RA will develop scleritis. These patients are more likely to present with diffuse or necrotizing forms of scleritis and have an increased risk of ocular complications and refractory scleral inflammation. Read More

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Case of vaso-occlusive retinopathy in Kikuchi-Fujimoto and lupus overlap syndrome.

BMJ Case Rep 2021 May 31;14(5). Epub 2021 May 31.

Department of Ophthalmology, Medical Eye Unit, Guy's and St Thomas' Hospitals NHS Trust, London, UK.

A 35-year-old woman presented with a constellation of systemic symptoms: rashes, weight loss, arthralgia and mouth ulcers. Six months afterwards, she experienced bilateral and sequential reduction in vision, and was found to have bilateral vaso-occlusive retinopathy, with critical macular ischaemia in the left eye. Her serological markers were consistent with a diagnosis of lupus. 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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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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Immune disorders and rheumatologic manifestations of viral hepatitis.

World J Gastroenterol 2021 May;27(18):2073-2089

Department of Internal Medicine, Gastroenterology and Hepatology, Sechenov University, Moscow 119435, Russia.

Infection with hepatotropic viruses is not limited to the liver and can lead to the development of various immunological disorders (the formation of cryoglobulins, rheumatoid factor, antinuclear antibodies, autoantibodies specific for autoimmune hepatitis and primary biliary cholangitis, and others), which can manifest as glomerulonephritis, arthritis, uveitis, vasculitis (cryoglobulinemic vasculitis, polyarteritis nodosa, Henoch-Schonlein purpura, isolated cutaneous necrotizing vasculitis), and other rheumatologic disorders, and be a trigger for the subsequent development of autoimmune hepatitis and primary biliary cholangitis. A further study of the association between autoimmune liver diseases and hepatotropic virus infection would be useful to assess the results of treatment of these associated diseases with antiviral drugs. The relationship of these immune disorders and their manifestations with hepatotropic viruses is best studied for chronic hepatitis B and C. Read More

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Increased Urinary CD163 Levels in Systemic Vasculitis with Renal Involvement.

Biomed Res Int 2021 30;2021:6637235. Epub 2021 Apr 30.

Hypertension Center of People's Hospital of Xinjiang Uygur Autonomous Region, Xinjiang Hypertension Institute, National Health Committee Key Laboratory of Hypertension Clinical Research, Urumqi, Xinjiang 830001, China.

Objectives: Systemic vasculitis includes a group of disorders characterized by inflammation of the vessel wall, involving multiple systems, and can cause malignant hypertension. CD163 is a specific marker of anti-inflammatory macrophages. This study is aimed at evaluating the CD163 levels in relation to systemic vasculitis and renal involvements. Read More

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Hydralazine-Induced Antineutrophil Cytoplasmic Antibody-Associated Vasculitis: Asymptomatic and Renal-Restricted Presentation.

Am J Case Rep 2021 May 16;22:e931263. Epub 2021 May 16.

Department of Rheumatology, Icahn School of Medicine at Mount Sinai, Queens Hospital Center, New York City, NY, USA.

BACKGROUND Hydralazine, a potent vasodilator widely used to treat hypertension, has been implicated in an increasing number of cases of drug-induced autoimmune diseases in recent years. However, antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis secondary to hydralazine use has rarely been described and most reported cases involved multi-organ-related vasculitis, including skin and lung-kidney manifestations. ANCA-associated vasculitis is an immune-inflammatory condition characterized by necrotizing vasculitis with few or no immune deposits, predominantly affecting small vessels. Read More

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Granulomatosis with Polyangiitis with Ocular Manifestations.

Case Rep Ophthalmol 2021 Jan-Apr;12(1):98-104. Epub 2021 Apr 6.

Postgraduate Education Department, Kazakh Eye Research Institute, Almaty, Kazakhstan.

Granulomatosis with polyangiitis (GPA) is a granulomatous-necrotic systemic vasculitis with a lesion of predominantly the upper and lower respiratory tracts at the onset of the disease (vasculitis, accompanied by granulomatous inflammation), and subsequently renal (glomerulonephritis). In addition, GPA may manifest as inflammation of small arteries and veins. Despite many years of study of this disease, the etiology of GPA remains unknown. Read More

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Fatal hemoperitoneum due to rupture of mesenteric artery in remission state of microscopic polyangiitis, concomitant with severe hypertension and posterior reversible encephalopathy syndrome: an autopsy case report.

CEN Case Rep 2021 May 4. Epub 2021 May 4.

Department of Pathology and Oncology, School of Medicine, University of Occupational and Environmental Health, 1-1 Iseigaoka, Yahatanishi-ku, Kitakyushu, Fukuoka, Japan.

Microscopic polyangiitis (MPA) is a type of necrotizing vasculitis associated with high levels of myeloperoxidase-specific antineutrophil cytoplasmic antibody (MPO-ANCA). While generally associated with renal dysfunction, MPA can also cause intraabdominal hemorrhage in rare cases. A 66-year-old man was admitted to our hospital for renal dysfunction, numbness, and weight loss for 3 months. Read More

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Therapeutic options for cutaneous polyarteritis nodosa: a systematic review.

Rheumatology (Oxford) 2021 May 3. Epub 2021 May 3.

Department of Internal Medicine, Division of Rheumatology, University of Cyprus Medical School, Nicosia, Cyprus.

Objective: Cutaneous polyarteritis nodosa (CPAN) is a necrotizing vasculitis of the middle-size vessels, confined to the skin. We conducted a systematic review in order to identify studies evaluating the different treatment modalities used in CPAN.

Methods: This systematic review was conducted according to PRISMA guidelines, registered in PROSPERO: CRD42020222195. Read More

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Anterior ischemic optic neuropathy and cilioretinal artery occlusion secondary to polyarteritis nodosa: A case report.

Photodiagnosis Photodyn Ther 2021 Apr 27;34:102299. Epub 2021 Apr 27.

Erciyes University Faculty of Medicine, Department of Ophthalmology, Kayseri, Turkey.

Introduction: Polyarteritis nodosa is a systemic vasculitis characterized by necrotizing inflammatory lesions affecting the middle and small arteries.

Case Presentation: A 23-year-old male presented to our ophthalmology clinic with a 1-day history of sudden vision loss in his left eye. Posterior segment examination the left eye showed optic disc borders were faint, hyperemic and fluffy with cilioretinal artery occlusion signs present. Read More

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Life-Threatening Subglottic Stenosis of Granulomatosis with Polyangiitis: A Case Report.

Jin An Jae-Won Song

Medicina (Kaunas) 2021 Apr 27;57(5). Epub 2021 Apr 27.

Department of Thoracic and Cardiovascular Surgery, Kyung Hee University Hospital at Gangdong, Kyung Hee University School of Medicine, Seoul 05278, Korea.

Granulomatosis with polyangiitis (GPA) is an autoimmune disease characterized by necrotizing granulomatous inflammation. Subglottic stenosis, which is defined as narrowing of the airway below the vocal cords, has a frequency of 16-23% in GPA. Herein, we present the case of a 39-year-old woman with subglottic stenosis manifesting as life-threatening GPA, which was recurrent under systemic immunosuppressive therapy. Read More

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Proteinuria Indicates Decreased Normal Glomeruli in ANCA-Associated Glomerulonephritis Independent of Systemic Disease Activity.

J Clin Med 2021 Apr 6;10(7). Epub 2021 Apr 6.

Department of Nephrology and Rheumatology, University Medical Center Göttingen, 37075 Göttingen, Germany.

Background: Renal involvement is a common and severe complication of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV), potentially resulting in a pauci-immune necrotizing and crescentic ANCA glomerulonephritis (GN) with acute kidney injury (AKI), end-stage renal disease (ESRD) or death. There is recent evidence that the degree of proteinuria at diagnosis is associated with long-term renal outcome in ANCA GN. Therefore, we here aimed to systematically describe the association between proteinuria and clinicopathological characteristics in 53 renal biopsies with ANCA GN and corresponding urinary samples at admission. Read More

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Pathogenesis and pathology of anti-neutrophil cytoplasmic antibody(ANCA)-associated vasculitis.

J Transl Autoimmun 2021 3;4:100094. Epub 2021 Mar 3.

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

•AAV is characterized by necrotizing small vessel vasculitis with positive serum ANCA.•MPO/PR3-ANCA and neutrophils play central roles in AAV pathogenicity.•Dysregulated complement system primes neutrophils. Read More

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Primary Angiitis of Central Nervous System-Tumor-Like Mass Lesions: Report of Six Biopsy Confirmed Cases.

Neurol India 2021 Mar-Apr;69(2):451-456

Department of Neurology, Radiology and Neurosurgery, CARE Institute of Neurosciences, CARE Hospitals, Banjara Hills, Hyderabad, Telangana, India.

Tumor-like mass lesion is a rare subtype of primary angiitis of the central nervous system (ML-PACNS). This report describes six patients of histologically verified ML-PACNS. The mean age was 44. Read More

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Unique Presentation of Microscopic Polyangiitis: Hearing and Vision Loss, Dysphagia, and Renal Dysfunction.

Cureus 2021 Mar 23;13(3):e14069. Epub 2021 Mar 23.

Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, USA.

Microscopic polyangiitis (MPA) is an autoimmune small-vessel vasculitis often positive for perinuclear anti-neutrophil cytoplasmic antibody (p-ANCA), or anti-myeloperoxidase (MPO), that classically affects the lungs, kidneys, and skin. Several atypical presentations of MPA involving other organs have also been reported in the literature. We report a unique case of a patient who presented with rare presentations of MPA: hearing and vision loss, dysphagia, renal dysfunction. Read More

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Granulomatosis with polyangiitis presenting as high fever with diffuse alveolar hemorrhage and otitis media: A case report.

World J Clin Cases 2021 Apr;9(10):2344-2351

Department of General Practice, The Second Affiliated Hospital of Guangxi Medical University, Nanning 530007, Guangxi Zhuang Autonomous Region, China.

Background: Granulomatosis with polyangiitis is a necrotizing inflammation of small and medium-sized vessels accompanied by formation of granuloma, involvement of primary granulomatous upper and lower respiratory tracts, glomerulonephritis, and vasculitis of small vessels.

Case Summary: Herein, we described a case of a 52-year-old man admitted with pulmonary nodules and high fever. Autoantibody workup revealed that the patient was positive for c-anti-neutrophil cytoplasmic antibodies and proteinase-3 anti-neutrophil cytoplasmic antibodies. Read More

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Type I Interferonopathies in Children: An Overview.

Front Pediatr 2021 31;9:631329. Epub 2021 Mar 31.

Department of Pediatrics, University of Chieti, Chieti, Italy.

Notable advances in gene sequencing methods in recent years have permitted enormous progress in the phenotypic and genotypic characterization of autoinflammatory syndromes. Interferonopathies are a recent group of inherited autoinflammatory diseases, characterized by a dysregulation of the interferon pathway, leading to constitutive upregulation of its activation mechanisms or downregulation of negative regulatory systems. They are clinically heterogeneous, but some peculiar clinical features may lead to suspicion: a familial "idiopathic" juvenile arthritis resistant to conventional treatments, an early necrotizing vasculitis, a non-infectious interstitial lung disease, and a panniculitis associated or not with a lipodystrophy may represent the "interferon alarm bells. Read More

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Risk of Stroke in Systemic Necrotizing Vasculitis: A Nationwide Study Using the National Claims Database.

Front Immunol 2021 31;12:629902. Epub 2021 Mar 31.

Division of Rheumatology, Department of Internal Medicine, Yonsei University College of Medicine, Seoul, South Korea.

Objective: Evidences indicate that the risk of stroke is increased in autoimmune rheumatic diseases. This study aimed to investigate the incidence of stroke in patients with systemic necrotizing vasculitis (SNV) using the national health database.

Methods: Data were obtained from the Korean National Claims database between 2010 and 2018 to identify incident SNV [anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and polyarteritis nodosa (PAN)] cases. Read More

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Anti-neutrophil cytoplasmic antibody associated glomerulonephritis complicating treatment with hydralazine.

Kidney Int 2021 Apr 20. Epub 2021 Apr 20.

Department of Pathology, Columbia University Irving Medical Center, New York, New York, USA.

Hydralazine, a widely used therapy for hypertension and heart failure, can elicit autoimmune disease, including anti-neutrophil cytoplasmic antibody associated glomerulonephritis (ANCA-GN). We identified 80 cases of ANCA-GN complicating treatment with hydralazine, accounting for 4.3% (80/1858 biopsies) of ANCA-GN diagnosed between 2006 and 2019. Read More

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Differentiating Arteriolosclerotic Ulcers of Martorell from Other Types of Leg Ulcers Based on Vascular Histomorphology.

Acta Derm Venereol 2021 May 4;101(5):adv00449. Epub 2021 May 4.

Skin and Endothelium Research Division (SERD), Department of Dermatology, Medical University of Vienna, AT-1090 Vienna, Austria.

Clinical differential diagnosis of arteriolosclerotic ulcers of Martorell is challenging due to the lack of clearly affirmative instrument-based diagnostic criteria. The aim of this study was to develop vascular histomorphological diagnostic criteria differentiating Martorell ulcers from other types of leg ulcers. The histomorphology of patients diagnosed with arteriolosclerotic ulcers of Martorell (n = 67) was compared with that of patients with venous leg ulcers, necrotizing leukocytoclastic vasculitis, pyoderma gangrenosum, and non-ulcerative controls (n = 15 each). Read More

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Clinical features, visual outcome, and poor prognostic factors in occlusive retinal vasculitis.

Can J Ophthalmol 2021 Apr 7. Epub 2021 Apr 7.

Massachusetts Eye Research and Surgery Institution, Waltham, Mass.; Ocular Immunology and Uveitis Foundation, Waltham, Mass.; Harvard Medical School, Boston, Mass.. Electronic address:

Objective: To investigate the clinical features, treatment, and visual outcome of occlusive retinal vasculitis (ORV), with a focal analysis on prognostic factors associated with poor visual outcome.

Methods: We conducted a retrospective cohort study in patients diagnosed with ORV with at least 6 months of follow-up. Demographic data, ocular features, best corrected visual acuity (BCVA), fluorescein angiography, therapy regimens, and outcomes were collected from the Massachusetts Eye Research and Surgery Institution database from 2006 to 2017. Read More

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Necrotic Ulceration of the Hand Case Review: Think Beyond Infection.

J Emerg Nurs 2021 May 30;47(3):379-383. Epub 2021 Mar 30.

Neutrophilic dermatosis of the dorsal hands is a rare neutrophilic dermatosis that can be associated with inflammatory bowel disease, rheumatoid arthritis, and underlying malignancies. The occurrence of trauma as an initiating factor and its early features of pain and inflammation followed by blistering or ulceration mean that it can be mistaken for necrotizing infection. Neutrophilic dermatosis of the dorsal hands should be considered in all patients who present with such features confined to the back of the hands, particularly those with negative microbiological results or lack of response to antibiotic therapy. Read More

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ANCA-associated vasculitis overlaps with systemic sclerosis: a case report and literature review.

Eur J Med Res 2021 Mar 31;26(1):30. Epub 2021 Mar 31.

Department of Rheumatology and Immunology, Sichuan Provincial People's Hospital, University of Electronic Science and Technology of China, No.32 The First Ring Road West 2, Chengdu, 610072, China.

Background: Systemic sclerosis (SSc) and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) both affect the kidney and may cause renal failure. Treatment of AAV is dramatically different from that of SSc renal crisis (SRC). Kidney biopsy is not recommended for diagnosing SRC, but it is the only reliable diagnostic method for AAV. Read More

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Development of a prediction model of treatment response in patients with cutaneous arteritis: Insights from a cohort of 33 patients.

J Dermatol 2021 Mar 25. Epub 2021 Mar 25.

Department of Dermatology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.

Cutaneous arteritis (CA) is necrotizing vasculitis invading the small- to medium-sized arteries of the skin. The majority of patients can be favorably managed by low- to medium-dose systemic corticosteroids (prednisolone, <0.5 mg/kg/day) or other oral medications such as non-steroidal anti-inflammatory drugs, dapsone, and azathioprine. Read More

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Tension Pneumothorax as Initial Manifestation of Granulomatosis with Polyangiitis (GPA).

Eur J Case Rep Intern Med 2021 11;8(3):002304. Epub 2021 Feb 11.

Internal Medicine Department, Centro Hospitalar Barreiro-Montijo, Barreiro, Portugal.

Granulomatosis with polyangiitis (Wegener's granulomatosis) is a systemic vasculitis that primarily affects small and medium vessels. Its manifestations are usually confined to the upper airway, lower airway and kidney. It can also affect other organs and systems, although this is unusual. Read More

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February 2021