7,114 results match your criteria Polyarteritis Nodosa

The role of vascular inflammation markers in deficiency of adenosine deaminase 2.

Semin Arthritis Rheum 2021 Apr 28;51(4):839-844. Epub 2021 Apr 28.

Department of Pediatrics, Division of Rheumatology, Hacettepe University School of Medicine, Ankara, Turkey; Pediatric Rheumatology Unit, Translational Medicine Laboratories, Hacettepe University, Ankara, Turkey. Electronic address:

Objectives: The first objective was to assess the role of vascular inflammatory factors in the pathogenesis of deficiency of adenosine deaminase 2 (DADA2) and to compare these markers among DADA2 patients with different phenotypes. We also aimed to investigate differences between DADA2 patients with vasculitic features and classic polyarteritis nodosa (PAN) for the aforementioned markers.

Methods: The study included eighteen DADA2 patients, ten PAN patients, and eight healthy controls. Read More

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Acute pancreatitis as a presenting feature in a child with systemic polyarteritis nodosa.

Indian J Pathol Microbiol 2021 Jun;64(Supplement):S180-S181

Department of Pediatrics, Bharati Vidyapeeth University Medical College Hospital and Research Centre, Pune, Maharashtra, India.

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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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A case report of a boy suffering from type 1 diabetes mellitus and familial Mediterranean fever.

Ital J Pediatr 2021 Jun 2;47(1):127. Epub 2021 Jun 2.

Department of Woman, Child and General and Specialized Surgery, University of the Study of Campania "Luigi Vanvitelli", Naples, Italy.

Background: Type 1 diabetes mellitus could be associated with other autoimmune diseases, such as autoimmune thyroid disease, celiac disease, but the association with Familial Mediterranean Fever is rare, we describe a case of a boy with type 1 Diabetes Mellitus associated with Familial Mediterranean Fever (FMF).

Case Presentation: A 13 year old boy already suffering from Diabetes Mellitus type 1 since the age of 4 years, came to our attention because of periodic fever associated with abdominal pain, chest pain and arthralgia. The fever appeared every 15-30 days with peaks that reached 40 °C and lasted 24-48 h. Read More

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Somatic Mutation in and ANCA-associated Vasculitis.

J Rheumatol 2021 Jun 1. Epub 2021 Jun 1.

Vasculitis Clinic, Canadian Network for Research on Vasculitides, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal; Division of Immunology and Allergy, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, Montreal, Quebec, Canada. The authors declare no conflicts of interest. Address correspondence to Dr. J.P. Makhzoum, Vasculitis Clinic, Division of Internal Medicine, Department of Medicine, Hôpital du Sacré-Coeur de Montréal, University of Montreal, 5400 Gouin O Blvd., Montreal, QC H4J 1C5, Canada. Email:

VEXAS (vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic) syndrome was recently discovered in 25 men with late-onset severe and refractory inflammatory syndromes and associated hematologic abnormalities. Various diseases have been described, such as relapsing polychondritis, myelodysplastic syndrome, polyarteritis nodosa, and giant cell arteritis. Read More

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Coronary Vasculitis.

Tommaso Gori

Biomedicines 2021 May 31;9(6). Epub 2021 May 31.

Kardiologie I and DZHK Standort Rhein-Main, Universitätsmedizin Mainz, 55131 Mainz, Germany.

The term coronary "artery vasculitis" is used for a diverse group of diseases with a wide spectrum of manifestations and severity. Clinical manifestations may include pericarditis or myocarditis due to involvement of the coronary microvasculature, stenosis, aneurysm, or spontaneous dissection of large coronaries, or vascular thrombosis. As compared to common atherosclerosis, patients with coronary artery vasculitis are younger and often have a more rapid disease progression. Read More

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Risk Factors and Incidence of Acute Ischemic Stroke: A Comparative Study Between Young Adults and Older Adults.

Cureus 2021 Apr 24;13(4):e14670. Epub 2021 Apr 24.

Neurology, Icahn School of Medicine at Mount Sinai, New York, USA.

Introduction Approximately 5-10% of strokes occur in adults of less than 45 years of age. The rising prevalence of stroke risk factors may increase stroke rates in young adults (YA). We aimed to compare risk factors and outcomes of acute ischemic stroke (AIS) among YA. Read More

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

BMC Rheumatol 2021 May 26;5(1):17. Epub 2021 May 26.

Department of Medicine, Northwell Health, 300 Community Drive, Manhasset, NY, 11030, USA.

Background: Polyarteritis 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 Presentation: A 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. 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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Neurological manifestations of polyarteritis nodosa: a tour of the neuroaxis by case series.

BMC Neurol 2021 May 21;21(1):205. Epub 2021 May 21.

Weill Institute for Neurosciences, University of California, San Francisco, California, USA.

Background: Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations.

Case Presentation: Case 1 presented with mononeuritis multiplex and characteristic skin findings. Read More

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Eosinophilic granulomatosis with polyangiitis: Cutaneous clinical and histopathologic differential diagnosis.

J Cutan Pathol 2021 May 21. Epub 2021 May 21.

Pathology Service, Massachusetts General Hospital and Harvard Medical School, Boston, Massachusetts, USA.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare, but severe systemic vasculitis that can affect skin and other organ systems. Diagnostic criteria have evolved, and many attempts have been made to classify the vasculitides based on clinical and/or histopathologic features, with an aim to develop standardized criteria. According to the EGPA Consensus Task Force recommendations, EGPA is a syndrome of asthma, eosinophilia, pulmonary infiltrates, and extrapulmonary vasculitis (such as cutaneous involvement with purpura). Read More

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Hepatitis B virus infection associated with polyarteritis nodosa and microscopic polyangiitis.

BMJ Case Rep 2021 May 19;14(5). Epub 2021 May 19.

Department of Medicine, University of Maryland Medical Center Midtown Campus, Baltimore, Maryland, USA

We reported a unique case with the coexistence of classic and cutaneous polyarteritis nodosa (PAN), and microscopic polyangiitis (MPA) in hepatitis virus-associated vasculitis. A 77-year-old Asian man presented with extremity weakness and weight loss found to have bilateral foot drop and rash on his hands and legs. Labs reveal positive for hepatitis B core antibody and perinuclear-antineutrophil cytoplasmic antibody (p-ANCA), decreased C3 and C4 levels. Read More

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Cutaneous polyarteritis nodosa presenting as a paraneoplastic phenomenon in chronic myelogenous leukemia.

JAAD Case Rep 2021 Jun 1;12:25-28. Epub 2021 Apr 1.

Department of Dermatology, University of Colorado Anschutz Medical Campus, Aurora, Colorado.

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Recurrent brachial artery aneurysm repair in a child managed with Gore-Tex conduit reinforcement.

J Vasc Surg Cases Innov Tech 2021 Jun 9;7(2):295-297. Epub 2021 Feb 9.

Division of Plastic and Reconstructive Surgery, Michael E. DeBakey Department of Surgery, Baylor College of Medicine, Houston, Tex.

Pediatric nonaortic arterial aneurysms are uncommon diagnoses and can be affiliated with underlying conditions, which include neurofibromatosis I, Ehlers-Danlos type IV syndrome, Kawasaki disease, Marfan syndrome, and Loeys-Dietz, polyarteritis nodosa, as well as Klippel-Trenauny syndrome. The standard of care has been early surgical excision and arterial reconstruction when indicated. This report details a case of recurrent brachial artery aneurysm in a 2-year-old boy despite multiple attempts at excision and reconstruction. Read More

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Enlarging Ventriculus Terminalis in a Patient With Polyarteritis Nodosa.

Cureus 2021 Apr 13;13(4):e14460. Epub 2021 Apr 13.

Neurological Surgery, University of Nebraska Medical Center, Omaha, USA.

Ventriculus terminalis (VT) is a cystic embryological remnant within the conus medullaris that normally regresses after birth. In rare cases, it may persist into adulthood and give rise to neurologic symptoms. The pathogenesis remains unclear but is thought to be related to failed embryonic regression with other proposed possible etiologies including vascular disturbances. Read More

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Coronary artery bypass using bilateral internal thoracic artery grafts in polyarteritis nodosa.

J Card Surg 2021 May 11. Epub 2021 May 11.

Department of Cardiovascular Surgery, Tokyo Women's Medical University, Tokyo, Japan.

Polyarteritis nodosa (PAN) affects small- and medium-sized arteries but rarely occurs in coronary artery aneurysms and stenosis. For patients with PAN, coronary artery bypass grafting (CABG) can be challenging, especially with respect to graft selection. We performed CABG using a bilateral internal thoracic artery (ITA) graft for a 21-year-old patient with PAN, with successful postoperative outcomes. Read More

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Identification of neutrophil β2-integrin LFA-1 as a potential mechanistic biomarker in ANCA-associated vasculitis via microarray and validation analyses.

Arthritis Res Ther 2021 05 6;23(1):136. Epub 2021 May 6.

Division of Rheumatology, Department of Internal Medicine, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, Japan.

Background: Leukocyte 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. 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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An Atypical Presentation of Polyarteritis Nodosa: Case Report and Review of the Literature.

Cureus 2021 Mar 30;13(3):e14197. Epub 2021 Mar 30.

Rheumatology, Arthritis Consultants, Reno, USA.

Polyarteritis nodosa (PAN) is a rare multisystem vasculopathy that predominantly affects medium-sized arteries. Involvement of the cardiac and/or pulmonary vasculature may be fatal. We describe a 67-year-old Japanese male who presented with multiple coronary artery aneurysms and subsequently developed lower extremity gangrene. 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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Sudden death complicating a coronary arteritis: polyarteritis nodosa (case report).

Pan Afr Med J 2021 3;38:113. Epub 2021 Feb 3.

Cardiology Department, Military Hospital of Tunis, Tunis, Tunisia.

Coronary artery aneurysms are uncommon, are usually associated with atherosclerosis, and rarely involve all three major coronary arteries. Data on the optimal choice of acute myocardial infarction (AMI)´s revascularization in the context of polyarteritis nodosa (PAN) is limited to case reports and is still an open question. The present report describes a rare case of a young male patient followed for PAN presenting with acute myocardial infarction (AMI). Read More

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Validation of the provisional seven-item criteria for the diagnosis of polyarteritis nodosa.

Rheumatol Int 2021 Apr 27. Epub 2021 Apr 27.

Department of Internal Medicine, Postgraduate Institute of Medical Education and Research, Chandigarh, India.

The 1990 American College of Rheumatology (ACR) criteria for the classification of polyarteritis nodosa (PAN) have many pitfalls and performed poorly when used for diagnostic purposes. Recently, a provisional seven-item diagnostic criteria for PAN was proposed. To validate the provisional seven-item diagnostic criteria for PAN in a cohort of PAN patients from a tertiary care centre in India. Read More

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Systematic review of childhood-onset polyarteritis nodosa and DADA2.

Semin Arthritis Rheum 2021 Jun 19;51(3):559-564. Epub 2021 Apr 19.

Division of Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, and Hacettepe University Vasculitis Research Center, Ankara, 06100, Turkey. Electronic address:

Background: Diagnosis of childhood polyarteritis nodosa (PAN) has become challenging after the definition of deficiency of adenosine deaminase 2 (DADA2). We aimed to define the differential features of pediatric PAN and DADA2 patients in our center and in the literature.

Methods: The charts of pediatric PAN and DADA2 patients followed at the Pediatric Rheumatology Unit of Hacettepe University between 2010-2020 were analyzed. Read More

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Celiac artery aneurysm causing an acute abdomen.

BMJ Case Rep 2021 Apr 21;14(4). Epub 2021 Apr 21.

Rheumatology, Great Western Hospital Foundation NHS Trust, Swindon, UK.

Abdominal pain is a very common presentation in the accident and emergency department. However, vasculitis is not the usual first differential diagnosis. This paper discusses a case of polyarteritis nodosa presenting with acute abdominal pain alone. Read More

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Clinical Characteristics and Outcomes of Mexican Patients With Polyarteritis Nodosa: A Single-Center Study Over 40 Years.

J Clin Rheumatol 2021 Feb 10. Epub 2021 Feb 10.

From the Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

The aim was to describe the clinical characteristics and outcomes of Mexican patients with polyarteritis nodosa (PAN) from a single center.

Methods: We conducted a medical records review study (1975-2018) that included patients with childhood- and adult-onset PAN. Demographics, organ involvement, phenotype, treatment, and outcomes were assessed and compared against previously published cohorts. Read More

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February 2021

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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Clinical presentations and long term prognosis of childhood onset polyarteritis nodosa in single centre of Korea.

Sci Rep 2021 Apr 16;11(1):8393. Epub 2021 Apr 16.

Department of Paediatrics, Seoul National University Children's Hospital, 101, Daehak-ro, Jongno-gu, Seoul, 03080, South Korea.

Childhood-onset polyarteritis nodosa (PAN) is a rare and systemic necrotising vasculitis in children affecting small- to medium-sized arteries. To date, there have been only a few reports because of its rarity. Thus, we aimed to investigate the clinical manifestations, laboratory findings, treatment, and long-term outcomes in patients with childhood-onset PAN and to evaluate the usefulness of the paediatric vasculitis activity score (PVAS). Read More

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CNS involvement in systemic vasculitides.

Neil Scolding

J Neurol Sci 2021 05 27;424:117423. Epub 2021 Mar 27.

Burden Professor of Clinical Neurosciences, University of Bristol Institute of Clinical Neurosciences, UK, Gulu University Faculty of Medicine, Uganda. Electronic address:

Both the CNS and the PNS can be involved in almost all of the vasculitides - including the primary systemic vasculitic disorders, such as microscopic polyangiitis and polyarteritis nodosa, and in non-vasculitic systemic disorders, such as systemic lupus erythematosis and sarcoidosis. The latter diseases also include infections and toxininduced disorders - particularly drugs of abuse such as cocaine and amphetamines. Here we will summarise the spectrum of these disorders as they affect the CNS, concentrating in particular on their distinguishing clinical and investigational features. Read More

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Fatal Coronary Artery Vasculitis With Mixed Features.

Am J Forensic Med Pathol 2021 Apr 7. Epub 2021 Apr 7.

From the University of South Carolina School of Medicine, Columbia, SC Edward Via College of Osteopathic Medicine, Auburn, AL Volusia County Medical Examiner's Office, Daytona Beach, FL Pathology Associates of Greenville, Greenville Office of the Medical Examiner, Greenville County University of South Carolina School of Medicine Greenville, Greenville, SC.

Abstract: Atherosclerotic cardiovascular disease (ASCVD) is often investigated by medical examiners as a cause of sudden death. Because of the variation in presentation of atherosclerotic cardiovascular disease, the examiner must be cautious when assigning a final diagnosis. The presented case depended upon histologic examination of coronary artery lesions to reach an appropriate final diagnosis of vasculitis with mixed features. Read More

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