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Polyarteritis nodosa

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https://www.readbyqxmd.com/read/29219653/characteristics-and-outcome-of-intractable-vasculitis-syndrome-in-children-nation-wide-survey-in-japan
#1
Naoko Nakano, Masaaki Mori, Hiroaki Umebayashi, Naomi Iwata, Norimoto Kobayashi, Kenji Masunaga, Tomoyuki Imagawa, Takuji Murata, Noriko Kinjo, Kazushige Nagai, Mari Miyoshi, Syuji Takei, Shumpei Yokota, Eiichi Ishii
OBJECTIVE: Primary systemic vasculitis (PSV) is a rare disorder in children and difficult to distinguish from other diseases. However, appropriate diagnosis and prompt treatment will affect on the morbidity and mortality of intractable PSV. In this study, we conducted a nationwide survey in Japan, to clarify epidemiology and clinical outcome of PSV. METHODS: We had sent survey questionnaires to most of the Japanese institutions that employed pediatricians, requesting the number of patients with refractory PSV who were diagnosed and treated between 2007 and 2011...
December 8, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29209584/sudden-death-from-spontaneous-coronary-artery-dissection-due-to-polyarteritis-nodosa
#2
Cyrus M Munguti, Paul M Ndunda, Tabitha M Muutu
Spontaneous coronary artery dissection (SCAD) is an emerging and rare cause of acute coronary syndrome and sudden cardiac death. While it was previously reported among young females with fibromuscular dysplasia, new literature indicates that this condition could occur in older populations. Polyarteritis nodosa (PAN) causes systemic necrotizing vasculitis which typically affects small to medium-sized muscular arteries and could affect the coronary arteries. A few case reports of PAN causing acute coronary artery disease have been reported in the literature...
October 2, 2017: Curēus
https://www.readbyqxmd.com/read/29162217/atypical-case-of-classical-polyarteritis-nodosa-alveolar-hemorrhage-and-positive-antineutrophil-cytoplasmic-antibody%C3%A2
#3
Nicole A Sitkin, Ethan Bernstein, Mark A Perazella
Classical polyarteritis nodosa (PAN) is a necrotizing arteritis affecting medium-sized vessels. A 73-year-old gentleman who presented with progressive anemia and scant hemoptysis developed acute kidney injury over the course of hospital admission. Kidney biopsy was initially interpreted as consistent with cephalosporin-associated acute interstitial nephritis (AIN) and acute tubular injury (ATI). After oral prednisone (5 days), the patient was discharged with improving serum creatinine. Two days later, he presented with diffuse alveolar hemorrhage...
November 22, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29146054/lymphocytic-thrombophilic-arteritis-a-distinct-inflammatory-type-i-interferon-and-c5b-9-mediated-subcutaneous-endovasculitis
#4
Cynthia M Magro, Jad Saab
BACKGROUND: Lymphocytic thrombophilic arteritis is a recently recognized subcuticular larger vessel vasculitis characterized by striking vascular luminal thrombosis. METHODS: The clinical features, histopathology and phenotypic profile of ten patients with lymphocytic thrombophilic arteritis were explored in an attempt to better define the entity from a clinical and pathophysiologic perspective. RESULTS: The patients were all female (mean age of 43) presenting with generally asymptomatic lower and upper extremity hyperpigmented macules...
December 2017: Annals of Diagnostic Pathology
https://www.readbyqxmd.com/read/29145218/endovascular-coil-treatment-of-a-coronary-artery-aneurysm-related-to-polyarteritis-nodosa
#5
Özgür Bayturan, Serdar Tarhan, Özge Çöpkıran, Fatih Düzgün, Uğur Kemal Tezcan
No abstract text is available yet for this article.
November 2017: Anatolian Journal of Cardiology
https://www.readbyqxmd.com/read/29128969/in-vasculitis-of-small-muscular-arteries-activation-of-vessel-infiltrating-cd8-t-cells-seems-to-be-antigen-independent
#6
Mikiko Kobayashi, Eisaku Ogawa, Ryuhei Okuyama, Hiroyuki Kanno
The etiology of polyarteritis nodosa (PAN) and localized PAN is still unknown, although a T cell-mediated immune mechanism has been considered. CD8 T cells participate not only in the antigen-dependent adaptive immune system, but also in the antigen-independent innate immune system. Non-antigen-activated CD8 T cells express a unique phenotype: granzyme B (GrB) positive /CD25 negative /programmed death-1 (PD-1) negative. The aims of this study were to assess the participation of T cells, especially innate CD8 T cells, in the development of vasculitis...
November 11, 2017: Virchows Archiv: An International Journal of Pathology
https://www.readbyqxmd.com/read/29114186/clinical-utility-of-anti-c1q-antibody-in-primary-and-secondary-vasculitic-conditions
#7
Kabeerdoss Jayakanthan, And Nikhil Gupta, John Mathew, Raheesh Ravindran, Gowri Mahasampth, Debashish Danda
Objective: Anti-C1q antibodies (Anti-C1q Ab) are seen in hypocomplementemic urticarial vasculitis syndrome (HUVS), infection-associated vasculitis such as hepatitis C virus-related vasculitis and in autoimmune diseases such as rheumatoid vasculitis, polyarteritis nodosa, giant cell arteritis, vascular Behcet's disease, and cryoglobulin associated vasculitis. Aim of this study is to evaluate the presence of Anti-C1q Ab in vasculitis and to determine if any difference exists between primary and secondary vasculitis in relation to this antibody...
November 2017: International Journal of Health Sciences
https://www.readbyqxmd.com/read/29109739/a-case-of-polyarteritis-nodosa-presenting-as-rapidly-progressing-intermittent-claudication-of-right-leg
#8
Chathuranga Lakmal Fonseka, Sampath Rukshani Galappaththi, Dayakshi Abeyaratne, Nirmali Tissera, Lalith Wijayaratne
Background: Polyarteritis nodosa (PAN) is a medium vessel vasculitis which causes significant morbidity and mortality. Usually, it presents with constitutional symptoms with angiographic evidence of aneurysms or segmental stenosis of arteries of mesenteric or renal vasculature. It is exceedingly uncommon for PAN to present with symptomatic progressive intermittent claudication. Case Presentation: We describe a 60-year-old male who presented with rapidly progressive intermittent claudication of his right leg...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/29061248/involvement-of-the-peripheral-nervous-system-in-polyarteritis-nodosa-and-antineutrophil-cytoplasmic-antibodies-associated-vasculitis
#9
REVIEW
John B Imboden
Peripheral nerve involvement is common in polyarteritis nodosa and the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. The underlying mechanism is arteritis of the vasa nervorum, leading to ischemic neuropathy. The classic presentation is stepwise involvement of peripheral nerves with ongoing antecedent constitutional symptoms. This article reviews the pathologic findings, clinical syndromes, diagnosis, and treatment of ANCA-associated vasculitides.
November 2017: Rheumatic Diseases Clinics of North America
https://www.readbyqxmd.com/read/29051974/cardiac-disease-in-familial-mediterranean-fever
#10
REVIEW
Eren Erken, Ertugrul Erken
Familial Mediterranean fever (FMF) is an autoinflammatory disease manifested by inflammatory attacks of peritonitis, pleuritis, pericarditis accompanied by fever and arthritis. Mutations of MEFV gene results in pyrin dysfunction, which causes uncontrolled interleukin-1 beta production and triggers the inflammatory attacks. Inflammation persists even during attack-free periods in one-third of the FMF patients. Findings of elevated proinflammatory cytokine patterns during remission as well as inflammatory attacks indicate the continuous subclinical disease activity and inflammation...
October 20, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29040156/accelerated-atheromatosis-and-arteriosclerosis-in-primary-systemic-vasculitides-current-evidence-and-future-perspectives
#11
Ourania D Argyropoulou, Athanase D Protogerou, Petros P Sfikakis
PURPOSE OF REVIEW: Primary systemic vasculitides (PSV) encompass a subset of autoimmune diseases, characterized by inflammation of blood vessels. Atheromatosis and arteriosclerosis may be accelerated in several PSV and account for the increased rate of cardiovascular morbidity that some exhibit. We aimed to summarize recent studies reporting on the acceleration of atheromatosis and/or arteriosclerosis in each type of PSV, using state-of-the-art noninvasive vascular biomarkers with clinical value as end points...
October 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/29038907/muscular-vasculitis-confined-to-lower-limbs-description-of-two-case-reports-and-a-review-of-the-literature
#12
REVIEW
Alessandra Tripoli, Simone Barsotti, Luca Emanuele Pollina, Alessandra Della Rossa, Rossella Neri, Anna d'Ascanio, Chiara Baldini, Marta Mosca
Muscular involvement is common during systemic vasculitides, such as polyarteritis nodosa. However, in rare cases, muscular involvement can be the only clinically evident feature of the disease. The clinical pattern of isolated muscular vasculitis may mimic several other inflammatory muscle disorders, such as idiopathic inflammatory myositis, and may represent a challenge in differential diagnosis. Herewith, we present two clinical cases as examples of peculiar clinical and histopathological characteristics of isolated muscular vasculitis...
December 2017: Rheumatology International
https://www.readbyqxmd.com/read/29021478/rhabdomyolysis-in-a-patient-with-polyarteritis-nodosa
#13
Harunobu Iida, Hironari Hanaoka, Yusa Asari, Kana Ishimori, Tomofumi Kiyokawa, Yukiko Takakuwa, Yoshioki Yamasaki, Hidehiro Yamada, Takahiro Okazaki, Masatomo Doi, Shoichi Ozaki
Polyarteritis nodosa (PAN) is a medium vessel vasculitis affecting systemic organs. Muscle involvement of PAN usually lacks elevation of creatinine kinase (CK). We herein report a case of PAN with rhabdomyolysis. A 71-year-old man was hospitalized because of muscle weakness of the lower limbs that persisted for 1 month. On a physical examination, rapidly progressive lower proximal muscle weakness and bilateral drop foot were observed. His blood test showed an elevation in the C-reactive protein (CRP) (19.5 mg/dL) and CK (13,435 IU/L) levels and negativity for anti-neutrophilic cytoplasmic antibody (ANCA)...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29019754/abnormalities-of-the-coronary-arteries-in-children-looking-beyond-the-origins
#14
Lauren J Saling, Demetrios A Raptis, Keyur Parekh, Toby A Rockefeller, Elizabeth F Sheybani, Sanjeev Bhalla
Coronary arterial abnormalities are uncommon findings in children that have profound clinical implications. Although anomalies of the coronary origins are well described, there are many other disease processes that affect the coronary arteries. Immune system-mediated diseases (eg, Kawasaki disease, polyarteritis nodosa, and other vasculiditides) can result in coronary arterial aneurysms, strictures, and abnormal tapering of the vessels. Because findings at imaging are an important component of diagnosis in these diseases, the radiologist's understanding of them is essential...
October 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/28983940/outline-of-guidelines-for-the-management-of-vasculitis-and-vascular-disorders-in-japan-2016-revised-edition
#15
Takaharu Ikeda, Fukumi Furukawa, Tamihiro Kawakami, Naoko Ishiguro, Miwa Uzuki, Shoichi Ozaki, Kensei Katsuoka, Takeshi Kono, Seiji Kawana, Masanari Kodera, Takashi Sawai, Yasuyuki Sawada, Mariko Seishima, Akiko Tanikawa, Ko-Ron Chen, Minoru Hasegawa
The proposal by the 1994 International Chapel Hill Consensus Conference on the Nomenclature of Systemic Vasculitides (CHCC1994) and by the CHCC2012 markedly influenced the classification and way of considering cutaneous vasculitis. In the proposal by the CHCC1994, hypersensitivity angiitis was defined as an equivalent pathological condition to microscopic polyangiitis or cutaneous leukocytoclastic angiitis (CLA), and it was not adopted as a disease name. However, CLA which was positioned as a type of small-vessel vasculitis is only a pathological name...
October 6, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28983775/deficiency-of-adenosine-deaminase-2-dada2-an-inherited-cause-of-polyarteritis-nodosa-and-a-mimic-of-other-systemic-rheumatologic-disorders
#16
REVIEW
Hasan Hashem, Susan J Kelly, Nancy J Ganson, Michael S Hershfield
PURPOSE OF REVIEW: A new autoinflammatory disease, deficiency of adenosine deaminase 2 (DADA2), caused by mutations in the CECR1 gene, was first reported in 2014. This review aims to update progress in defining, treating, and understanding this multi-faceted disorder. RECENT FINDINGS: DADA2 was first described in patients with systemic inflammation, mild immune deficiency, and vasculopathy manifested as recurrent stroke or polyarteritis nodosa (PAN). More than 125 patients have now been reported, and the phenotype has expanded to include children and adults presenting primarily with pure red cell aplasia (PRCA), or with antibody deficiency...
October 5, 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28980907/reclassification-of-polyarteritis-nodosa-based-on-the-1990-acr-criteria-using-the-2007-ema-algorithm-modified-by-the-2012-chcc-definitions
#17
Eun Seong Park, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
No abstract text is available yet for this article.
September 6, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28979236/optic-neuropathy-secondary-to-polyarteritis-nodosa-case-report-and-diagnostic-challenges
#18
Kristian A Vazquez-Romo, Adrian Rodriguez-Hernandez, Jose A Paczka, Moises A Nuño-Suarez, Alberto D Rocha-Muñoz, Maria G Zavala-Cerna
PURPOSE: To describe a case of optic neuropathy as a primary manifestation of polyarteritis nodosa (PAN) and discuss diagnostic challenges. METHODS: Case report. RESULTS: A 41-year-old Hispanic man presented with a 2-day history of reduced visual acuity in his left eye. Physical examination revealed a complete visual field loss in the affected eye. Best-corrected visual acuity (BCVA) in the left eye was hand motion, and fundus examination revealed a hyperemic optic disk with blurred margins, swelling, retinal folds, dilated veins, and normal size arteries...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28974505/hematopoietic-stem-cell-transplantation-rescues-the-hematological-immunological-and-vascular-phenotype-in-dada2
#19
Hasan Hashem, Ashish R Kumar, Ingo Müller, Florian Babor, Robbert Bredius, Jignesh Dalal, Amy P Hsu, Steven M Holland, Dennis D Hickstein, Stephen Jolles, Robert Krance, Ghadir Sasa, Mervi Taskinen, Minna Koskenvuo, Janna Saarela, Joris van Montfrans, Keith Wilson, Barbara Bosch, Leen Moens, Michael Hershfield, Isabelle Meyts
Deficiency of adenosine deaminase 2 (DADA2) is caused by biallelic deleterious mutations in CECR1 DADA2 results in variable autoinflammation and vasculopathy (recurrent fevers, livedo reticularis, polyarteritis nodosa, lacunar ischemic strokes and intracranial hemorrhages), immunodeficiency and bone marrow failure. TNF-α blockade is the treatment of choice for the autoinflammation and vascular manifestations. Hematopoietic stem cell transplantation (HSCT) represents a potential definitive treatment. We present a cohort of 14 patients from 6 countries who received HSCT for DADA2...
October 3, 2017: Blood
https://www.readbyqxmd.com/read/28968697/polyarteritis-nodosa
#20
Raphael Micheroli, Oliver Distler
No abstract text is available yet for this article.
August 21, 2017: Rheumatology
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