7,204 results match your criteria Polyarteritis Nodosa


Long-term exposure to fine particulate matter and ozone and the onset of systemic autoimmune rheumatic diseases: an open cohort study in Quebec, Canada.

Arthritis Res Ther 2022 Jun 23;24(1):151. Epub 2022 Jun 23.

Division of Clinical Epidemiology, McGill University Health Centre, Montreal, QC, Canada.

Objectives: To estimate associations between fine particulate matter (PM) and ozone and the onset of systemic autoimmune rheumatic diseases (SARDs).

Methods: An open cohort of over 6 million adults was constructed from provincial physician billing and hospitalization records between 2000 and 2013. We defined incident SARD cases (SLE, Sjogren's syndrome, scleroderma, polymyositis, dermatomyositis, polyarteritis nodosa and related conditions, polymyalgia rheumatic, other necrotizing vasculopathies, and undifferentiated connective tissue disease) based on at least two relevant billing diagnostic codes (within 2 years, with at least 1 billing from a rheumatologist), or at least one relevant hospitalization diagnostic code. Read More

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Cutaneous polyarteritis nodosa diagnosis and treatment: a retrospective case series.

J Am Acad Dermatol 2022 Jun 14. Epub 2022 Jun 14.

University of British Columbia, Department of Medicine, Division of Rheumatology, Vancouver, BC, Canada.

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Polyarteritis nodosa: an evolving primary systemic vasculitis.

Postgrad Med 2022 Jun 22:1-8. Epub 2022 Jun 22.

Department of Medicine, Division of Rheumatology and Immunology, Vanderbilt University Medical Center, Nashville, Tennessee, USA.

Polyarteritis nodosa (PAN) is a primary form of vasculitis characterized by inflammation of primarily medium-sized arteries. Several key events have shaped the current spectrum of the disease including the separation of a subgroup with microscopic polyangiitis, the discovery of the association of hepatitis B, and the discovery of adenosine deaminase 2 deficiency (DADA2). With the discovery of secondary causes of PAN and changing nomenclature, the incidence of PAN has declined over time. Read More

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Use of Biologics to Treat Relapsing and/or Refractory Polyarteritis Nodosa: Data from a European Collaborative Study.

Rheumatology (Oxford) 2022 Jun 10. Epub 2022 Jun 10.

Department of Internal Medicine, National Reference Center for Rare Systemic Autoimmune Diseases, AP-HP, APHP-CUP, Hôpital Cochin, Paris, 75014, France.

Objectives: To describe the effectiveness and safety of biologics for the treatment of relapsing and/or refractory polyarteritis nodosa (PAN).

Methods: A retrospective European collaborative study was conducted in patients with PAN who received biologics for relapsing and/or refractory disease.

Results: Forty-two patients with PAN received a total of 53 biological courses, including TNF-alpha blockers in 15 cases, rituximab (RTX) in 18 cases, tocilizumab (TCZ) in 10 cases, and other biologics in 10 cases. Read More

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Left Main Coronary Artery Fusiform Aneurysm.

Cureus 2022 May 6;14(5):e24790. Epub 2022 May 6.

Cardiology, Marshall University, Huntington, USA.

Aneurysm of the coronary artery is an uncommon condition that is usually found incidentally. The left coronary aneurysm is the least common. We report the case of a young patient with a history of vasculitis who was found to have a left fusiform coronary aneurysm. Read More

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Letter from the editor: Management of cutaneous polyarteritis nodosa Link to JAAD-D-20-04595R5.

Authors:
Dirk M Elston

J Am Acad Dermatol 2022 May 25. Epub 2022 May 25.

Professor and Chairman, Department of Dermatology and Dermatologic Surgery Medical University of SC; MSC 578 135 Rutledge Avenue; 11th Floor Charleston, SC 29425-5780. Electronic address:

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Angiographic characteristics of vasculopathy in patients with idiopathic inflammatory myopathies and systemic sclerosis.

Sci Rep 2022 May 31;12(1):9051. Epub 2022 May 31.

Division of Rheumatology, Department of Internal Medicine, Seoul National University Hospital and College of Medicine, 101 Daehak-ro, Jongno-gu, Seoul, 03080, Republic of Korea.

To describe the peripheral angiographic features of vasculopathy in idiopathic inflammatory myopathies (IIM) and systemic sclerosis (SSc) in comparison to polyarteritis nodosa (PAN). Angiograms of 47 extremities (24 upper and 23 lower) of 11 patients with IIM (n = 5) and SSc (n = 6), and 12 patients with PAN who presented with critical limb ischemia were retrospectively analyzed with regards to the presence of stenosis, occlusion, aneurysms and delayed distal flow, and degree of neovascularization. Diffuse narrowing was more frequent (66. Read More

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Deficiency of Human Adenosine Deaminase Type 2 - A Diagnostic Conundrum for the Hematologist.

Front Immunol 2022 3;13:869570. Epub 2022 May 3.

Pediatric Allergy Immunology Unit, Department of Pediatrics, Advanced Pediatrics Centre, Post Graduate Institute of Medical Education and Research (PGIMER), Chandigarh, India.

Deficiency of adenosine deaminase type 2 (DADA2) was first described in 2014 as a monogenic cause of polyartertitis nodosa (PAN), early onset lacunar stroke and livedo reticularis. The clinical phenotype of DADA2 is, however, very broad and may involve several organ systems. Apart from vasculitis, children may present with i) Hematological manifestations (ii) Lymphoproliferation and iii) Immunodeficiencies. Read More

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Myocardial Infarction in a 7-Year-Old Girl with Polyarteritis Nodosa.

Case Rep Rheumatol 2022 26;2022:2175676. Epub 2022 Apr 26.

King Saud Bin Abdulaziz University for Health Sciences, Riyadh 14611, Saudi Arabia.

Polyarteritis nodosa (PAN) is a rare systemic vasculitis that affects small to medium-sized arteries. It could affect any organ including the heart. However coronary artery involvements are very rare. Read More

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Cutaneous manifestations of acute kidney injury.

Clin Kidney J 2022 May 9;15(5):855-864. Epub 2021 Dec 9.

Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Central Parkway, Newcastle upon Tyne, UK.

Acute kidney injury (AKI) is a common medical problem with a multitude of aetiologies. Prompt diagnosis and management is key in the prevention of complications. Cutaneous signs can often give diagnostic clues of underlying systemic diseases causing AKI. Read More

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Segmental endobronchial valve therapy for a vasculitis-induced emphysema.

Respir Med Case Rep 2022 16;37:101650. Epub 2022 Apr 16.

Department of Medicine II, Division of Pulmonology, Medical University of Vienna, Vienna, Austria.

A 53-year old female patient with history of hypocomplementaemic urticarial vasculitis syndrome (HUVS) and polyarteritis nodosa presented with progressive dyspnoea on exertion due to emphysema. Lung function revealed a severe obstructive ventilator disorder with a forced expiratory volume in 1 second of 22% of predicted, and a significant hyperinflation with a residual volume of 321% of predicted. Multi-detector computed tomography (MDCT) scan and quantitative CT analysis (StratX software) confirmed a lower lobe predominant emphysema. Read More

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Case Report: Idiopathic Subcutaneous Thrombotic Vasculopathy.

Front Med (Lausanne) 2022 15;9:843793. Epub 2022 Apr 15.

Department of Plastic Surgery, University of Kansas Medical Center, Kansas City, KS, United States.

Lower extremity ulcers have significant morbidity, with treatment determined by the underlying disorder. Reported is a 32-year-old female presenting with small skin nodules and bruises across her legs 4 weeks following her second COVID vaccination. These lesions progressed into large, necrotic ulcers over several months. Read More

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Different Course of SARS-CoV-2 Infection in Two Adolescents With Other Immunosuppressive Factors.

Cureus 2022 Feb 28;14(2):e22710. Epub 2022 Feb 28.

Pediatrics, Nutrition and Metabolic Disorders, Children's Memorial Health Institute, Warsaw, POL.

Even after two years of the Coronavirus Disease 2019 (COVID-19) pandemic, despite known risk factors, we are still unable to predict the severity of the infection in specific patients. Due to the contradictory data, the protective role of immunosuppression in preventing the severe course of the infection remains uncertain. Therefore, we want to discuss the influence of several immunosuppressive factors on the COVID-19 pattern in children, based on two case reports regarding 17-year-old boys with other immunosuppressive factors and a completely different course of the disease. Read More

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February 2022

The Association of Serum Anti-Lysosomal-Associated Membrane Protein-2 Antibody with Vasculitis Combined with Hypertension.

Int J Hypertens 2022 18;2022:9656560. Epub 2022 Mar 18.

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

The aim of this study was to explore the association of serum anti-lysosomal-associated membrane protein-2 (anti-LAMP-2) antibody with vasculitis combined with hypertension (VAS-HTN). A total of 51 VAS-HTN patients, 46 essential hypertension (EH) patients, and 46 healthy controls (HC) were included in the study. Serum anti-LAMP-2 antibody levels are increased in VAS-HTN patients as compared with EH and HC (all < 0. Read More

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Regulatory T Cells in Autoimmune Vasculitis.

Front Immunol 2022 28;13:844300. Epub 2022 Feb 28.

Department of Medicine, Mayo College of Medicine and Science, Rochester, MN, United States.

Blood vessels are indispensable for host survival and are protected from inappropriate inflammation by immune privilege. This protection is lost in patients with autoimmune vasculitides, a heterogeneous group of diseases causing damage to arteries, arterioles, and capillaries. Vasculitis leads to vascular wall destruction and/or luminal occlusion, resulting in hemorrhage and tissue ischemia. Read More

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Vitamin D levels in patients with small and medium vessel vasculitis.

Reumatol Clin (Engl Ed) 2022 Mar;18(3):141-146

Marmara University, School of Medicine, Department of Internal Medicine, Division of Rheumatology, Istanbul, Turkey.

Objectives: To determine the prevalence of vitamin D deficiency in patients with small and medium vessel systemic vasculitis.

Methods: In this cross-sectional study, 25-hydroxy (OH) vitamin D3 levels were measured in adult patients with systemic small and medium vessel vasculitis including antineutrophil cytoplasmic antibody-associated vasculitis (AAV), cryoglobulinaemic vasculitis (CryV), IgA vasculitis (IgAV) and polyarteritis nodosa (PAN), and age- and sex-matched healthy subjects (HS) and patients with rheumatoid arthritis (RA) as control groups. 25OH vitamin D3 levels<30ng/ml and <20ng/ml were regarded as insufficiency and deficiency, respectively. Read More

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A Rare Presentation of Polyarteritis Nodosa.

Cureus 2022 Feb 5;14(2):e21925. Epub 2022 Feb 5.

Rheumatology, Henry Ford Health System, Detroit, USA.

Polyarteritis nodosa (PAN) is a rare form of necrotizing medium-vessel vasculitis. PAN has the potential for widespread organ involvement, but the skin, renal, neurologic, and musculoskeletal systems are most commonly involved. A definitive diagnosis can be made with a biopsy of an easily accessible organ such as the skin or an involved nerve or muscle. Read More

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February 2022

Successful treatment of polyarteritis nodosa with intestinal necrosis in a Chinese boy: a case report.

Transl Pediatr 2022 Jan;11(1):157-162

Department of General Surgery, Qilu Children's Hospital of Shandong University, Jinan, China.

Polyarteritis nodosa (PAN) is a systemic necrotizing inflammatory disease of the medium and small arteries which has variable clinical manifestations, course, and organ involvement. Intestinal necrosis resulting from PAN is rare, and successful treatment of such cases is even more uncommon. Here, we report the first successful treatment of PAN with intestinal necrosis in a young Chinese child. Read More

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January 2022

A man in his sixties with chondritis and bone marrow failure.

Tidsskr Nor Laegeforen 2022 03 28;142(4). Epub 2022 Feb 28.

Background: VEXAS syndrome (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic syndrome) first described in 2020, is caused by a limited repertoire of somatic mutations in UBA1, a gene involved in the initiation of ubiquitination. Ubiquitination, adding an ubiquitin protein to a substrate protein, can have various effects on the substrate. Disruption of UBA1 function results in diverse clinical manifestations, mimicking a variety of disorders. Read More

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Colonic Perforation in Polyarteritis Nodosa With Concurrent Perforated Sigmoid Diverticulitis.

Cureus 2022 Jan 23;14(1):e21509. Epub 2022 Jan 23.

School of Medicine, Western Sydney University, Sydney, AUS.

Polyarteritis nodosa (PAN) is a medium-sized vasculitis with neuropathy, cutaneous manifestation, and gastrointestinal tract symptoms. An acute surgical abdomen is a severe but rare development of gastrointestinal involvement of PAN, with large bowel involvement and subsequent perforation being sporadic. Here we present a rare case of PAN who had large bowel involvement with perforation due to newly diagnosed PAN, who also had concurrent and separately perforated sigmoid diverticulitis, confusing the clinical picture. Read More

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January 2022

and relapsing necrotizing vasculitis after COVID-19 vaccination.

Clin Kidney J 2022 Mar 20;15(3):560-563. Epub 2021 Dec 20.

Service de néphrologie-hypertension artérielle, dialyses, transplantation rénale, CHU Bretonneau, Tours, France.

We describe five cases of severe necrotizing vasculitis following the RNA-based vaccine for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), including four relapsing anti neutrophil cytoplasmic antibodies (ANCA) vasculitis, 27 days (1-60) after vaccination and one patient with quiescent chronic hepatitis B and polyarteritis nodosa (PAN) 21 days after vaccination. Ten other cases were reported to the French national pharmacovigilance database: six patients with ANCA-associated vasculitis and four patients with PAN (first symptoms 19 days on average after vaccination). Five of these 10 patients developed kidney dysfunction. Read More

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Difficulties in Differentiating Hypertensive Disorders of Pregnancy With Polyarteritis Nodosa.

J Med Cases 2022 Jan 17;13(1):5-10. Epub 2022 Jan 17.

Department of Obstetrics and Gynecology, Institution of University of Occupational and Environmental Health, Kitakyushu, Fukuoka, Japan.

Polyarteritis nodosa (PAN) is characterized by medium- or small-sized artery vasculitis with vessel wall inflammation and necrosis of muscular arteries, commonly presenting with fatigue, fever, weight loss, and joint pain. PAN in pregnancy is rare and is associated with worsening of vasculitis after delivery, resulting in myocardial infarction and heart failure which frequently lead to maternal death. We report a case of hypertensive disorders of pregnancy (HDP), which is difficult to differentiate from PAN. Read More

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January 2022

An Unusual Case of Vasculitis.

Isr Med Assoc J 2022 Feb;24(2):123

Department of Internal Medicine, Shaare Zedek Medical Center, Jerusalem Israel.

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February 2022

Henoch-Schönlein Purpura /IgA Vasculitis Complicated by Coronary Artery Aneurysm: A Case Report and Literature Review.

Front Pediatr 2021 3;9:781106. Epub 2022 Feb 3.

Department of Nephrology, Rheumatology and Immunology, Hunan Children's Hospital, Changsha, China.

Henoch-Schönlein purpura (HSP)/ IgA vasculitis (IgAV) is the most common form of systemic vasculitis in children and often involves the skin, gastrointestinal tract, joints, and kidneys, though cardiac involvement rarely occurs. We report on a 6-year-old male child with HSP/IgAV who had renal and cardiac involvement at the initial stage of the disease and in whom we found an extremely rare coronary artery aneurysm. After administration of glucocorticoid combined with mycophenolate mofetil, the renal involvement improved, but the coronary artery aneurysm remained. Read More

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February 2022

Mechanisms of vascular inflammation in deficiency of adenosine deaminase 2 (DADA2).

Semin Immunopathol 2022 05 17;44(3):269-280. Epub 2022 Feb 17.

The MOE Key Laboratory of Biosystems Homeostasis and Protection, Life Sciences Institute, Zhejiang University, Hangzhou, China.

Deficiency of adenosine deaminase 2 (DADA2) was first described as a monogenic form of systemic vasculitis that closely resembles polyarteritis nodosa (PAN). The phenotypic spectrum of DADA2 has vastly expanded in recent years and now includes pure red cell aplasia, bone marrow failure syndrome, lymphoproliferative disease, and humoral immunodeficiency. Vasculitis remains the most common presentation of DADA2, and treatment with tumor necrosis factor inhibitors (TNFi) has shown remarkable efficacy in preventing stroke and ameliorating features of systemic inflammation. Read More

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Bilateral renal subcapsular hematoma caused by polyarteritis nodosa: a case report.

CEN Case Rep 2022 Feb 16. Epub 2022 Feb 16.

Department of Medicine, Division of Nephrology, Showa University School of Medicine, Tokyo, Japan.

Polyarteritis nodosa, which is a systemic vasculitis of small- and medium-sized arteries, can cause arterial aneurysms in various organs, sometimes resulting in aneurysm rupture and hemorrhage. A kidney is one of the major targets of polyarteritis nodosa. Here, we report a 73-year-old woman who presented with sudden-onset high fever, diarrhea, and renal injury with bilateral renal subcapsular hematoma shown on contrast-enhanced computed tomography scan. Read More

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February 2022

Diagnostic and Therapeutic Challenges of Vasculitis.

Can J Cardiol 2022 May 11;38(5):623-633. Epub 2022 Feb 11.

Vanderbilt University Medical Center, Nashville, Tennessee, USA. Electronic address:

Systemic vasculitis can be a challenge to differentiate from other forms of vasculopathy. Because treatment for systemic vasculitis is disparate from that for other forms of vasculopathy, clinicians should strive for high diagnostic certainty. This review article aims to highlight the clinical, radiographic, and histologic clues to distinguish systemic vasculitis from mimics. Read More

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