6,874 results match your criteria Polyarteritis Nodosa


Papillophlebitis as manifestation of polyarteritis nodosa.

Arch Soc Esp Oftalmol 2019 Feb 4. Epub 2019 Feb 4.

Servicio de Cardiología, Complejo Hospitalario Universitario Insular Materno Infantil, Las Palmas de Gran Canaria, Las Palmas, España.

A 35 year-old-man diagnosed with polyarteritis nodosa developed papillophlebitis on his left eye. Throughout the evolution of the disease, he had a macular oedema treated with intravitreal injections of aflibercept, with adequate recovery of visual acuity. He also had episcleritis on the other eye. Read More

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http://dx.doi.org/10.1016/j.oftal.2018.12.007DOI Listing
February 2019

Association of serum high mobility group box 1 levels with disease activity and renal involvement in patients with systemic vasculitis.

Medicine (Baltimore) 2019 Feb;98(6):e14493

Center for Hypertension of People's Hospital of Xinjiang Uygur Autonomous Region, Hypertension Institute of Xinjiang, Urumqi, Xinjiang, China.

High mobility group box 1 (HMGB1) is a kind of proinflammatory mediator that acts as an alarmin when released by dying, injured or activated cells. Previous studies have reported that HMGB1 are closely linked to antineutrophil cytoplasmic antibody-associated vasculitis (AAV). The present study aimed to evaluate whether serum HMGB1 levels were associated with systemic vasculitis (VAs). Read More

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http://dx.doi.org/10.1097/MD.0000000000014493DOI Listing
February 2019
3 Reads

Polyarteritis nodosa presenting atypically as appendicitis and pyelonephritis in a single patient.

BMJ Case Rep 2019 Feb 1;12(2). Epub 2019 Feb 1.

Griffith University School of Medicine, Gold Coast, Queensland, Australia.

A 17-year-old man presented to the emergency department 10 days postlaparoscopic appendicectomy with ongoing lower abdominal pain. The pain was associated with fevers, chills, sweating and constipation. There were no other associated symptoms. Read More

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http://dx.doi.org/10.1136/bcr-2018-228072DOI Listing
February 2019
6 Reads

Imaging in small and medium vessel vasculitis.

Int J Rheum Dis 2019 Jan;22 Suppl 1:78-85

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

Vasculitis includes a group of disorders characterized by inflammation of the vessel wall and classified based on the diameter of the predominantly involved vessels. Granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis and Henoch-Schonlein purpura are the important entities in the small vessel vasculitis group, while polyarteritis nodosa and Kawasaki disease represent the medium vessel vasculitis group. The clinical manifestations may be non-specific and there may be considerable overlap with the other disorders. Read More

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http://dx.doi.org/10.1111/1756-185X.13390DOI Listing
January 2019
1 Read

[Muscular polyarteritis nodosa-a case-based review].

Z Rheumatol 2019 Jan 29. Epub 2019 Jan 29.

Rheumatologie, Klinische Immunologie, Nephrologie, Asklepios Klinik Altona, Paul-Ehrlich-Str. 1, 22763, Hamburg, Deutschland.

Background: Myalgia is a common but unspecific set of symptoms that may be caused by orthopedic, neurological and internal medical conditions, often resulting in a diagnostic challenge. Muscular polyarteritis nodosa (PAN) is a rare differential diagnosis of myalgia with elevated serological inflammatory markers.

Objective: Based on three clinical cases and the literature this review describes the essential clinical and diagnostic features of muscular PAN. Read More

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http://dx.doi.org/10.1007/s00393-019-0595-xDOI Listing
January 2019
1 Read

Androgen deprivation therapy for prostate cancer and the risk of autoimmune diseases.

Prostate Cancer Prostatic Dis 2019 Jan 28. Epub 2019 Jan 28.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei, Taiwan.

Background: Androgen deprivation therapy (ADT) has been a mainstay of treatment for advanced prostate cancer (PCa), but limited studies have been performed to investigate the association between ADT and autoimmune diseases.

Methods: We conducted a population-based nationwide cohort study of 17,168 patients newly diagnosed with PCa between 1996 and 2013 using the National Health Insurance Research Database (NHIRD) of Taiwan. Cox proportional hazards models with 1:1 propensity score-matched analysis were used to investigate the association between ADT use and the risk of autoimmune diseases. Read More

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http://www.nature.com/articles/s41391-019-0130-9
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http://dx.doi.org/10.1038/s41391-019-0130-9DOI Listing
January 2019
6 Reads

Four distinct clinical phenotypes of vasculitis affecting medium-sized arteries.

Scand J Rheumatol 2019 Jan 23:1-7. Epub 2019 Jan 23.

a Department of Hematology and Rheumatology , Tohoku University Graduate School of Medicine , Sendai , Japan.

Objective: Within the spectrum of polyarteritis nodosa (PAN), cutaneous PAN (cPAN) is further classified into mild cPAN and severe cPAN which presents with ulcers, necrosis, or neuritis. As distinguishing between severe cPAN and systemic PAN can be difficult, this study evaluated the clinical characteristics of patients with necrotizing arteritis of medium-sized arteries.

Methods: Forty-one patients diagnosed with necrotizing arteritis of medium-sized arteries between 2008 and 2017 at our institution were enrolled in this study. Read More

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http://dx.doi.org/10.1080/03009742.2018.1551965DOI Listing
January 2019
1 Read

Hepatitis C Virus-associated Membranoproliferative Glomerulonephritis Treated with Directly Acting Antiviral Therapy.

Indian J Nephrol 2018 Nov-Dec;28(6):462-464

Department of Nephrology, Institute of Liver and Biliary Sciences, New Delhi, India.

Hepatitis C virus (HCV) infection has been shown to affect kidneys with various histopathological pattern on the kidney biopsy. These commonly include a membranoproliferative glomerulonephritis (MPGN) pattern with mixed cryoglobulinemia (CG), thrombotic microangiopathy, membranous nephropathy, and vasculitis affecting medium and small vessels of the kidneys causing polyarteritis nodosa. It has been rarely associated with MPGN without detectable CG. Read More

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http://dx.doi.org/10.4103/ijn.IJN_235_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6309397PMC
January 2019
1 Read

A 9.5-year-old boy with recurrent neurological manifestations and severe hypertension, treated initially for polyarteritis nodosa, was subsequently diagnosed with adenosine deaminase type 2 deficiency (DADA2) which responded to anti-TNF-α.

Paediatr Int Child Health 2019 Jan 15:1-4. Epub 2019 Jan 15.

a Department of Paediatric Rheumatology, Cerrahpasa Medical School , Istanbul University , Istanbul , Turkey.

A 9.5-year-old boy was referred with a 2-year history of recurrent fever, myalgia, abdominal pain and various neurological manifestations associated with increased acute phase reactants and IgG level. During the recent episode, severe hypertension and right-sided hemiparesis developed and angiography demonstrated irregularities and stenosis in renal and mesenteric artery branches. Read More

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http://dx.doi.org/10.1080/20469047.2018.1559495DOI Listing
January 2019
2 Reads

MPO-ANCA-positive cutaneous polyarteritis nodosa with livedo racemosa arising from immune-deposit-positive necrotizing vasculitis.

G Ital Dermatol Venereol 2019 Jan 9. Epub 2019 Jan 9.

Departments of Rheumatology, Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology, Tokyo, Japan.

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https://www.minervamedica.it/index2.php?show=R23Y9999N00A190
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http://dx.doi.org/10.23736/S0392-0488.18.05956-4DOI Listing
January 2019
4 Reads

Serum lysosomal-associated membrane protein-2 levels are increased in small and medium vessel vasculitis, especially in polyarteritis nodosa.

Clin Exp Rheumatol 2019 Jan 4. Epub 2019 Jan 4.

Centre for Hypertension of People's Hospital of Xinjiang Uygur Autonomous Region; Hypertension Institute of Xinjiang, Urumqi, Xinjiang, China.

Objectives: Lysosomal-associated membrane protein-2 (LAMP-2) is a highly glycosylated type I glycoprotein ex- pressed on the membranes of neutrophils, endothelial cells and other cells, which are closely linked to subsets of systematic vasculitis. The aim of this study was to investigate whether serum LAMP-2 can be used as a biomarker in small and medium vessel vasculitis (SMVV).

Methods: Serum samples from 39 patients with SMVV (including ANCA-associated vasculitis (AAV) and polyarteritis nodosa (PAN)) confirmed by angiography and/or biopsy and 78 healthy controls (HC) were collected. Read More

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January 2019
9 Reads

PET-CT and polyarteritis nodosa-associated artery aneurysms.

QJM 2019 Jan 3. Epub 2019 Jan 3.

Department of General Internal Medicine, Asahi General Hospital, Asahi, Chiba, Japan.

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http://dx.doi.org/10.1093/qjmed/hcy308DOI Listing
January 2019
2 Reads

Human adenosine deaminase 2 deficiency: A multi-faceted inborn error of immunity.

Immunol Rev 2019 Jan;287(1):62-72

Department of Microbiology and Immunology, Laboratory for Childhood Immunology, KU Leuven, Leuven, Belgium.

Human adenosine deaminase 1 deficiency was described in the 1970s to cause severe combined immunodeficiency. The residual adenosine deaminase activity in these patients was attributed to adenosine deaminase 2. Human adenosine deaminase type 2 deficiency (DADA2), due to biallelic deleterious mutations in the ADA2 gene, is the first described monogenic type of small- and medium-size vessel vasculitis. Read More

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http://dx.doi.org/10.1111/imr.12722DOI Listing
January 2019
6 Reads

Vasculitis in Systemic Autoinflammatory Diseases.

Front Pediatr 2018 3;6:377. Epub 2018 Dec 3.

Division of Pediatric Rheumatology, Department of Pediatrics, Hacettepe University Faculty of Medicine, Ankara, Turkey.

Autoinflammatory diseases (AID) are diseases of the innate immune system, characterized by recurrent episodes of localized or systemic inflammation. Vasculitis may accompany AID. The causes of the association of vasculitis with monogenic AID are still debated. Read More

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http://dx.doi.org/10.3389/fped.2018.00377DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6287042PMC
December 2018
15 Reads

What's new in autoinflammation?

Authors:
Seza Ozen

Pediatr Nephrol 2018 Dec 14. Epub 2018 Dec 14.

Department of Pediatrics, Hacettepe University, Ankara, Turkey.

The pathogenesis of autoinflammatory diseases has shed light on the concept of inflammation in general and on our understanding of the role of the innate immune system. The autoinflammatory diseases have a large spectrum with varying features of inflammation. The most common autoinflammatory diseases are those associated with periodic fevers. Read More

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http://dx.doi.org/10.1007/s00467-018-4155-4DOI Listing
December 2018
4 Reads

"Emergency" Cochlear Implantation in Labyrinthitis Ossificans Secondary to Polyarteritis Nodosa: How to Face a Rare Entity.

J Int Adv Otol 2018 Dec 12. Epub 2018 Dec 12.

Department of Otolaryngology, University of Pavia, IRCCS Policlinico "S. Matteo" Foundation, Pavia, Italy.

Polyarteritis nodosa (PAN) is a systemic vasculitis affecting the small- and medium-sized arteries that may present with hearing impairment. In rare cases, PAN may be associated with progressive labyrinthitis ossificans (LO), an otologic emergency requiring early cochlear implantation (CI) to restore hearing before the complete, irreversible cochlear ossification. We report the first case in the literature of a patient affected by PAN with bilateral sudden sensorineural hearing loss and rapid LO who underwent "emergency" bilateral simultaneous CI. Read More

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http://dx.doi.org/10.5152/iao.2018.5463DOI Listing
December 2018
1 Read

Case Report: Unexpected Benefit of Echocardiography in Childhood Polyarteritis Nodosa.

Klin Padiatr 2018 Dec 11. Epub 2018 Dec 11.

General University Hospital, Paediatric Rheumatology and Autoinflammatory Diseases Unit, Praha, Czech Republic.

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http://www.thieme-connect.de/DOI/DOI?10.1055/a-0802-8950
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http://dx.doi.org/10.1055/a-0802-8950DOI Listing
December 2018
11 Reads

Polyarteritis nodosa in case of familial Mediterranean fever.

Turk J Pediatr 2018 ;60(3):326-330

Division of Pediatric Nephrology, Department of Pediatrics, Marmara University Faculty of Medicine, İstanbul, Turkey.

Gökçe İ, Altuntaş Ü, Filinte D, Alpay H. Polyarteritis nodosa in case of familial Mediterranean fever. Turk J Pediatr 2018; 60: 326-330. Read More

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http://dx.doi.org/10.24953/turkjped.2018.03.016DOI Listing
January 2018
2 Reads

Pulmonary manifestations of large, medium, and variable vessel vasculitis.

Respir Med 2018 Dec 10;145:182-191. Epub 2018 Nov 10.

University of Texas Southwestern Medical Center, Department of Pulmonary and Critical Care Medicine, United States.

The hallmark of vasculitis is autoimmune inflammation of blood vessels and surrounding tissues, resulting in an array of constitutional symptoms and organ damage. The lung is commonly targeted in the more familiar ANCA-associated small vessel vasculitidies, but large and medium vessel vasculitides, including Takayasu arteritis, giant cell arteritis, polyarteritis nodosa, Behcet's disease, and necrotizing sarcoid granulomatosis, may also feature prominent pulmonary involvement. Pulmonary manifestations of these conditions include pulmonary arterial aneurysms, pulmonary hypertension, diffuse alveolar hemorrhage, pulmonary nodules, and parenchymal infiltrates. Read More

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http://dx.doi.org/10.1016/j.rmed.2018.11.003DOI Listing
December 2018
3 Reads

Conditions and Factors Associated With Spontaneous Coronary Artery Dissection (from a National Population-Based Cohort Study).

Am J Cardiol 2019 Jan 30;123(2):249-253. Epub 2018 Oct 30.

Brigham and Women's Hospital Heart & Vascular Center, Harvard Medical School, Boston, Massachusetts. Electronic address:

The pathophysiology of spontaneous coronary artery dissection (SCAD) is heterogeneous, associated with systemic arteriopathies and inflammatory diseases, and often compounded by environmental precipitants, genetics, or stressors. However, the frequency of these associated conditions with SCAD on a population level remains unknown. Therefore, the objective of this analysis was to evaluate heterogeneous phenotypes of SCAD in the United States using data from the Nationwide Inpatient Sample collected from January 1, 2004, to September 31, 2015. Read More

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http://dx.doi.org/10.1016/j.amjcard.2018.10.012DOI Listing
January 2019
8 Reads
3.280 Impact Factor

Tocilizumab treatment in refractory polyarteritis nodosa: a case report and review of the literature.

Rheumatol Int 2019 Feb 21;39(2):337-344. Epub 2018 Nov 21.

Asklepios Klinik Altona, Rheumatologie, Klinische Immunologie, Nephrologie, Paul-Ehrlich-Straße 1, 22763, Hamburg, Germany.

Polyarteritis nodosa (PAN) is a rare systemic vasculitis affecting multiple organs. Current standard treatment includes the use of glucocorticoids and cyclophosphamide. Unfortunately, some patients do not respond to this treatment and other therapeutic options are needed. Read More

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http://dx.doi.org/10.1007/s00296-018-4210-2DOI Listing
February 2019
15 Reads

Polyarteritis nodosa: lessons from 25 years of experience.

Clin Exp Rheumatol 2018 Oct 30. Epub 2018 Oct 30.

Department of Paediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, and Hacettepe University Vasculitis Center, Ankara, Turkey.

Objectives: Polyarteritis nodosa is a necrotising vasculitis of predominantly medium size vessels. The present study aimed to summarise the characteristics of PAN patients, and also analyse the trend of decreasing PAN frequency in the last 25 years.

Methods: PAN patients followed up between 1990 and 2015 were included. Read More

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October 2018
13 Reads

Immunoglobulin G4-related disease with recurrent uveitis and kidney tumor mimicking childhood polyarteritis nodosa: a rare case report.

Acta Reumatol Port 2018 Jul-Sep;43(3):226-229

Instituto da Criança, Hospital das Clínicas, Faculdade de Medicina, Universidade de São Paulo, São Paulo, SP, Brasil.

Introduction: Immunoglobulin G4-related disease (IgG4-RD) is an condition rarely reported in children. Additionally, IgG4-RD may rarely mimic vasculitis in adults and may infrequently present with uveitis. In our service, 6,198 patients were followed-up and only one (0. Read More

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November 2018
4 Reads

Diffuse Alveolar Hemorrhage and Pulmonary Vasculitides: Histopathologic Findings.

Semin Respir Crit Care Med 2018 08 7;39(4):425-433. Epub 2018 Nov 7.

Department of Pathology and Laboratory Medicine, David Geffen School of Medicine at UCLA, Los Angeles, California.

Vasculitides are a heterogeneous group of disorders in which inflammation of blood vessel walls is present at least some time during the course of the disease. Vasculitides can affect any caliber or type of vessel in many anatomic sites; however, the disease can alter more than just vasculature. Given the diversity of vasculitides, in 2012, a revised classification system was proposed to categorize vasculitides by the type of vessel involved including size, function, and structural attributes. Read More

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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0038-1669412
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http://dx.doi.org/10.1055/s-0038-1669412DOI Listing
August 2018
12 Reads

An update on the nomenclature for cutaneous vasculitis.

Curr Opin Rheumatol 2019 Jan;31(1):46-52

Section of Dermatology, Department of Surgery and Translational Medicine, University of Florence, Florence, Italy.

Purpose Of Review: Cutaneous vasculitis reflects a spectrum ranging from skin limited to severe systemic forms. To date, there is still no generally acknowledged nomenclature for cutaneous vasculitis. This review aims to summarize the recent advances in the nomenclature of cutaneous vasculitis. Read More

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http://dx.doi.org/10.1097/BOR.0000000000000563DOI Listing
January 2019
10 Reads

Immune-Mediated Systemic Vasculitis as the Proposed Cause of Sudden-Onset Sensorineural Hearing Loss following Lassa Virus Exposure in Cynomolgus Macaques.

MBio 2018 10 30;9(5). Epub 2018 Oct 30.

Office of the Chief Scientists, U.S. Army Medical Research Institute of Infectious Diseases, Fort Detrick, Frederick, Maryland, USA

Lassa virus (LASV) causes a severe, often fatal hemorrhagic disease in regions in Africa where the disease is endemic, and approximately 30% of patients develop sudden-onset sensorineural hearing loss after recovering from acute disease. The causal mechanism of hearing loss in LASV-infected patients remains elusive. Here, we report findings after closely examining the chronic disease experienced by surviving macaques assigned to LASV exposure control groups in two different studies. Read More

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http://dx.doi.org/10.1128/mBio.01896-18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6212830PMC
October 2018
4 Reads

Renal Amyloidosis in Deficiency of Adenosine Deaminase 2: Successful Experience With Canakinumab.

Pediatrics 2018 Nov;142(5)

Departments of Pediatric Rheumatology.

Deficiency of adenosine deaminase 2 (DADA2) is a rare autoinflammatory disease that was firstly described in patients with early-onset strokes, livedo reticularis, and periodic fever resembling polyarteritis nodosa. In reported case series, researchers described highly variable manifestations, including autoimmunity, immunodeficiency, hepatosplenomegaly, pancytopenia, ichthyosiform rash, and arthritis, in patients with DADA2. A thirteen-year-old female patient who was born to consanguineous parents was admitted to our hospital with generalized edema and leg pain. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2018-0948DOI Listing
November 2018
12 Reads

Severe strongyloidiasis and systemic vasculitis: comorbidity, association or both? Case-based review.

Rheumatol Int 2018 Dec 23;38(12):2315-2321. Epub 2018 Oct 23.

Clinic of Allergology and Immunology, Clinical Center of Serbia, Belgrade, Serbia.

A possible association between strongyloidiasis and systemic vasculitis is rarely reported in the literature. We report the case of a patient with severe strongyloidiasis and an angiographic finding consistent with polyarteritis nodosa. Diagnosis of strongyloidiasis was made by finding of larvae and adult parasites in samples of the upper gastrointestinal tract mucosa and stool. Read More

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http://link.springer.com/10.1007/s00296-018-4178-y
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http://dx.doi.org/10.1007/s00296-018-4178-yDOI Listing
December 2018
13 Reads

Orchitis: An unusual presentation of polyarteritis nodosa.

Indian J Pathol Microbiol 2018 Oct-Dec;61(4):600-603

Department of Pathology, IPGME and R, Kolkata, West Bengal, India.

Polyarteritis nodosa (PAN), a systemic necrotizing vasculitis with multiorgan development, is generally restricted to the medium-sized muscular arteries. The varied initial clinical presentations of PAN can lead to a delayed diagnosis. We present the case of a middle-aged male patient who presented with an acute onset right-sided testicular pain as the initial clinical symptom with ischemic changes on ultrasonogram, thereby requiring orchiectomy. Read More

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http://www.ijpmonline.org/text.asp?2018/61/4/600/242994
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http://dx.doi.org/10.4103/IJPM.IJPM_569_17DOI Listing
February 2019
3 Reads

Autoimmune Kidney Diseases Associated with Chronic Viral Infections.

Rheum Dis Clin North Am 2018 11 7;44(4):675-698. Epub 2018 Sep 7.

Department of Medicine, Division of Nephrology, Massachusetts General Hospital, 55 Fruit Street, GRB 7, Boston, MA 02114, USA. Electronic address:

Autoimmune kidney diseases triggered by viruses are an important cause of kidney disease in patients affected by chronic viral infection. Hepatitis B virus (HBV) infection is associated with membranous nephropathy and polyarteritis nodosa. Hepatitis C virus (HCV) infection is a major cause of cryoglobulinemic glomerulonephritis. Read More

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http://dx.doi.org/10.1016/j.rdc.2018.06.006DOI Listing
November 2018
3 Reads

Renal complications of liver diseases.

Expert Rev Gastroenterol Hepatol 2018 Oct 1. Epub 2018 Oct 1.

a Service de Néphrologie, Hémodialyse , Aphérèses et Transplantation rénale, Grenoble-Alpes , France.

Introduction: Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections are the major causes of chronic liver disease. HBV and HCV affect nearly 7% of the world's population. Extra-hepatic complications and particularly renal failure have different mechanisms and manifestations. Read More

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http://dx.doi.org/10.1080/17474124.2018.1530984DOI Listing
October 2018
12 Reads

Nodules developing after radiofrequency ablation of varicosities: A potential clinical and histopathologic mimic of polyarteritis nodosa.

JAAD Case Rep 2018 Sep 14;4(8):814-816. Epub 2018 Sep 14.

Department of Dermatology, Penn State Health Hershey Medical Center, Hershey, Pennsylvania.

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http://dx.doi.org/10.1016/j.jdcr.2018.03.029DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6141695PMC
September 2018
2 Reads

Systemic Necrotizing Vasculitis in Sheep Is Associated With Ovine Herpesvirus 2.

Vet Pathol 2018 Sep 10:300985818795166. Epub 2018 Sep 10.

8 Finn Pathologists, One Eyed Lane, Weybread, Diss, Norfolk, UK.

Ovine herpesvirus 2 (OvHV-2) is one of the gammaherpesviruses in the genus Macavirus that can cause malignant catarrhal fever (MCF) in ungulates. Sheep are the adapted host for OvHV-2 and it is generally assumed that infection is not associated with disease in this species. However, cases of "polyarteritis nodosa" or idiopathic systemic necrotizing vasculitis reported in sheep are similar to vascular lesions in clinically susceptible species with MCF. Read More

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http://dx.doi.org/10.1177/0300985818795166DOI Listing
September 2018
14 Reads

Hospitalizations due to systemic connective tissue diseases: Secular trends and regional disparities in Sweden, 1998-2016.

Int J Rheum Dis 2018 Nov 30;21(11):1900-1906. Epub 2018 Aug 30.

Clinical Epidemiology Unit, Department of Clinical Sciences-Lund, Orthopaedics, Faculty of Medicine, Lund University, Lund, Sweden.

Aim: To investigate secular trends and regional disparities in hospitalizations due to systemic connective tissue diseases (SCTD) in Sweden from 1998 to 2016.

Method: We identified all hospital admissions with a principal diagnosis of SCTD (ICD-10 codes: M30-M36) from the Swedish National Patient Register. Joinpoint regression was used to assess secular trends in age-standardized hospitalization rates (ASHR) and proportions of SCTD from all and musculoskeletal disorders hospitalizations. Read More

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http://doi.wiley.com/10.1111/1756-185X.13341
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http://dx.doi.org/10.1111/1756-185X.13341DOI Listing
November 2018
20 Reads

Vasculitis Pathogenesis: Can We Talk About Precision Medicine?

Front Immunol 2018 14;9:1892. Epub 2018 Aug 14.

Division of Rheumatology, Department of Pediatrics, University of Health Sciences, Ankara Training and Research Hospital, Ankara, Turkey.

Precision medicine is designing the medical care by taking into account the individual variability for each person. We have tried to address whether the existing data may guide precision medicine in primary systemic vasculitides (PSV). We have reviewed genome-wide association studies (GWAS) data, lessons from monogenic mimics of these diseases, and biomarker studies in immunoglobulin A vasculitis/Henoch-Schönlein purpura, Kawasaki disease, anti-neutrophil cytoplasmic antibody-associated vasculitis, polyarteritis nodosa (PAN), Takayasu arteritis, and Behçet's disease (BD). Read More

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http://dx.doi.org/10.3389/fimmu.2018.01892DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6102378PMC
August 2018
4 Reads

Acute Surgical Abdomen Due to Sporadic Polyarteritis Nodosa.

J Clin Rheumatol 2018 Aug 23. Epub 2018 Aug 23.

Department of Internal Medicine University of New Mexico Health Sciences Center, Albuquerque, NM Department of Pathology University of New Mexico Health Sciences Center, Albuquerque, NM Division of Rheumatology, Department of Internal Medicine, University of New Mexico Health Sciences Center, Albuquerque, NM

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http://dx.doi.org/10.1097/RHU.0000000000000891DOI Listing
August 2018
2 Reads

ADA2 Deficiency Mimicking Idiopathic Multicentric Castleman Disease.

Pediatrics 2018 Sep;142(3)

Departments of Microbiology and Immunology and.

Multicentric Castleman disease (MCD) is a rare entity that, unlike unicentric Castleman disease, involves generalized polyclonal lymphoproliferation, systemic inflammation, and multiple-organ system failure resulting from proinflammatory hypercytokinemia, including, in particular, interleukin-6. A subset of MCD is caused by human herpesvirus-8 (HHV-8), although the etiology for HHV-8-negative, idiopathic MCD (iMCD) cases is unknown at present. Recently, a consensus was reached on the diagnostic criteria for iMCD to aid in diagnosis, recognize mimics, and initiate prompt treatment. Read More

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http://pediatrics.aappublications.org/lookup/doi/10.1542/ped
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http://dx.doi.org/10.1542/peds.2017-2266DOI Listing
September 2018
13 Reads

Cutaneous Vasculitis in Cogan's Syndrome: A Report of Two Cases Associated with Chlamydia Infection.

J Nippon Med Sch 2018 ;85(3):172-177

Department of Dermatology, Nippon Medical School.

Cogan's syndrome (CS) is defined by the combination of hearing loss, vertigo, and ocular inflammation of uncertain cause, and can be associated with variable vessel vasculitis. Vasculitic manifestations may include arteritis (affecting large, medium or small arteries), aortitis, and aortic and mitral valvulitis. Cutaneous manifestations including erythema, papules, subcutaneous nodules, and purpura sometimes occur; however, to date, only six cases have been histologically confirmed to have genuine vasculitis. Read More

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http://dx.doi.org/10.1272/jnms.JNMS.2018_85-25DOI Listing
October 2018
14 Reads

Childhood- versus Adult-Onset Polyarteritis Nodosa Results from the French Vasculitis Study Group Registry.

Autoimmun Rev 2018 Oct 14;17(10):984-989. Epub 2018 Aug 14.

National Referral Center for Rare Systemic Autoimmune Diseases, Department of Internal Medicine, Hôpital Cochin, Assistance Publique-Hôpitaux de Paris (APHP), Université Paris Descartes, Paris, France. Electronic address:

Objective: To investigate differences between childhood (cPAN)- and adult-onset polyarteritis nodosa (aPAN) patients.

Methods: cPAN patients' clinical findings at onset and outcomes were compared to those of aPAN patients from the French Vasculitis Study Group registry matched for year of enrollment and initial systemic versus cutaneous disease. Their information on medications, disease activity and damage were collected. Read More

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http://dx.doi.org/10.1016/j.autrev.2018.08.001DOI Listing
October 2018
30 Reads

A retrospective study comparing the phenotype and outcomes of patients with polyarteritis nodosa between UK and Turkish cohorts.

Rheumatol Int 2018 Oct 11;38(10):1833-1840. Epub 2018 Aug 11.

Vasculitis and Lupus Clinic, Addenbrooke's Hospital, University of Cambridge, Cambridge, UK.

There is a need for better definition of polyarteritis nodosa (PAN) subphenotypes and the influence of ethnicity and geography. This study is aimed to study the demographic and clinical features of PAN cohorts from the UK and Turkey (TR) and to compare and contrast disease characteristics. A retrospective survey of databases from two vasculitis centres between 1990 and 2016 for PAN patients fulfilling the EMEA Vasculitis Classification algorithm. Read More

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http://link.springer.com/10.1007/s00296-018-4122-1
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http://dx.doi.org/10.1007/s00296-018-4122-1DOI Listing
October 2018
16 Reads

Polyarteritis nodosa limited to the breasts.

Breast J 2018 Nov 26;24(6):1088. Epub 2018 Jul 26.

Department of Surgery, Komoro Kosei General Hospital, Nagano, Japan.

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http://dx.doi.org/10.1111/tbj.13097DOI Listing
November 2018
3 Reads

Lymphocytic Thrombophilic Arteritis: A Review.

J Clin Rheumatol 2018 Jul 24. Epub 2018 Jul 24.

From the Department of Dermatology, Johns Hopkins University School of Medicine, Baltimore, MD.

Macular lymphocytic arteritis or lymphocytic thrombophilic arteritis (LTA) is a recently described cutaneous arteritis that is characterized by asymptomatic macules and patches of the extremities associated with lymphocytic arteritis at the deep dermal/subcuticular junction for which little information exists on demographics, evaluation, and management. There has been recent debate in the literature whether this disease is a new distinct entity, misdiagnosed cutaneous polyarteritis nodosa (cPAN), or a disease on a spectrum with cPAN. We systematically review the literature on demographic information, medical history, histopathology findings, and treatment management to analyze trends and clarify controversies in characterizations of LTA. Read More

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http://dx.doi.org/10.1097/RHU.0000000000000846DOI Listing
July 2018
2 Reads

Hybrid Surgery to Treat Multiple Visceral Aneurysms Secondary to Polyarteritis Nodosa.

Vasc Specialist Int 2018 Jun 30;34(2):35-38. Epub 2018 Jun 30.

Division of Vascular Surgery, Department of Surgery, Seoul National University Hospital, Seoul, Korea.

A 57-year-old woman presented to vascular surgery clinic with visceral artery aneurysms that were incidentally detected during regular check-up. Imaging studies revealed occlusion of the celiac axis and severe stenosis of the superior mesenteric artery and 3 aneurysms along the posterior and inferior pancreaticoduodenal arteries, as well as the right gastroepiploic artery. Endovascular embolization of all aneurysms was rejected because of the risk of hepatic ischemia. Read More

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http://dx.doi.org/10.5758/vsi.2018.34.2.35DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6027802PMC
June 2018
2 Reads

Polyarteritis Nodosa and Membranous Glomerulonephritis: Two Simultaneous Extrahepatic Manifestations of Hepatitis B.

J Clin Rheumatol 2018 Jun 29. Epub 2018 Jun 29.

Nephrology Division Department of Medicine Universidade Federal de São Paulo-Escola Paulista de Medicina São Paulo, Brazil. Rheumatology Division Department of Medicine Universidade Federal de São Paulo-Escola Paulista de Medicina São Paulo, Nephrology Division Department of Medicine Universidade Federal de São Paulo-Escola Paulista de Medicina São Paulo, Brazil.

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http://dx.doi.org/10.1097/RHU.0000000000000844DOI Listing
June 2018
7 Reads

Deficiency of Adenosine Deaminase 2 in Adult Siblings: Many Years of a Misdiagnosed Disease With Severe Consequences.

Front Immunol 2018 14;9:1361. Epub 2018 Jun 14.

National Human Genome Research Institute, National Institute of Health, Bethesda, MD, United States.

Objective: Describe the clinical characteristics and histopathology findings in a family with two siblings affected with deficiency of adenosine deaminase 2 (DADA2). Both patients presented in childhood with polyarthritis and developed significant neurological and gastrointestinal features of DADA2 in ear, including variable degrees of immunologic and hematologic manifestations.

Methods: Adenosine Deaminase 2 (ADA2; also known as exon sequencing and serum ADA2 levels were performed to confirm the diagnosis of DADA2. Read More

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http://dx.doi.org/10.3389/fimmu.2018.01361DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6010516PMC
June 2018
2 Reads

Clinical and Laboratory Markers Associated With Relapse in Cutaneous Polyarteritis Nodosa.

JAMA Dermatol 2018 Aug;154(8):922-926

Department of Dermatology, Okayama University Graduate School of Medicine, Dentistry, and Pharmaceutical Sciences, Okayama, Japan.

Importance: In cutaneous polyarteritis nodosa (CPAN), less aggressive treatments can be selected, because CPAN is not associated with life-threatening or progressive outcomes. Although patients with a recurrent clinical course may require additional immunosuppressive therapies, no pretreatment factors associated with a worse prognosis in CPAN have been reported.

Objective: To identify clinical or laboratory markers associated with relapse of CPAN. Read More

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http://dx.doi.org/10.1001/jamadermatol.2018.1601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6143022PMC
August 2018
4 Reads

Abdominal pain leading to incidental finding of polyarteritis nodosa.

BMJ Case Rep 2018 Jun 28;2018. Epub 2018 Jun 28.

Internal Medicine, University of Arizona, Tucson, Arizona, USA.

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http://dx.doi.org/10.1136/bcr-2018-225438DOI Listing
June 2018
3 Reads

Deficiency of Adenosine Deaminase 2 (DADA2): Updates on the Phenotype, Genetics, Pathogenesis, and Treatment.

J Clin Immunol 2018 Jul 27;38(5):569-578. Epub 2018 Jun 27.

Inflammatory Disease Section, National Human Genome Research Institute, Bethesda, USA.

Deficiency of ADA2 (DADA2) is the first molecularly described monogenic vasculitis syndrome. DADA2 is caused by biallelic hypomorphic mutations in the ADA2 gene that encodes the adenosine deaminase 2 (ADA2) protein. Over 60 disease-associated mutations have been identified in all domains of ADA2 affecting the catalytic activity, protein dimerization, and secretion. Read More

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http://dx.doi.org/10.1007/s10875-018-0525-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6061100PMC
July 2018
18 Reads