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https://www.readbyqxmd.com/read/28808190/cholesterol-crystal-embolization-following-plaque-rupture-a-systemic-disease-with-unusual-features
#1
Firas Ghanem, Deepthi Vodnala, Jagadeesh K Kalavakunta, Sridevi Durga, Noah Thormeier, Prem Subramaniyam, Scott Abela, George S Abela
Cholesterol crystal embolic (CCE) syndrome is often a clinically challenging condition that has a poor prognostic implication. It is a result of plaque rupture with release of cholesterol crystals into the circulation that embolize into various tissue organs. Plaque rupture seems to be triggered by an expanding necrotic core during cholesterol crystallization forming sharp tipped crystals that perforate and tear the fibrous cap. Embolizing cholesterol crystals then initiate both local and systemic inflammation that eventually lead to vascular fibrosis and obstruction causing symptoms that can mimic other vasculitic conditions...
January 19, 2017: Journal of Biomedical Research
https://www.readbyqxmd.com/read/28780079/antineutrophil-cytoplasmic-antibody-associated-vasculitides-and-igg4-related-disease-a-new-overlap-syndrome
#2
REVIEW
François-Xavier Danlos, Giovanni Maria Rossi, Daniel Blockmans, Giacomo Emmi, Andreas Kronbichler, Stéphane Durupt, Claire Maynard, Luminita Luca, Cyril Garrouste, Bertrand Lioger, Rachel Mourot-Cottet, Robin Dhote, Jean-Benoit Arlet, Thomas Hanslik, Philippe Rouvier, Mikael Ebbo, Xavier Puéchal, Dominique Nochy, Agnès Carlotti, Luc Mouthon, Loïc Guillevin, Augusto Vaglio, Benjamin Terrier
OBJECTIVE: Atypical manifestations have been described in patients with ANCA-associated vasculitides (AAV), such as pachymeningitis, orbital mass or chronic periaortitis. Because these manifestations have been associated to the spectrum of IgG4-related disease (IgG4-RD), we hypothesized that both diseases could overlap. METHODS: We conducted a European retrospective multicenter observational study including patients fulfilling ACR and Chapel Hill criteria for AAV and IgG4-RD Comprehensive Diagnostic Criteria...
August 2, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28732764/vasculitic-peripheral-neuropathy-induced-by-ischemia-reperfusion-in-the-rat-femoral-artery-involves-activation-of-proinflammatory-signaling-pathway-in-the-sciatic-nerve
#3
Chih-Yang Chung, Yi-Wei Chang, Chun-Jen Huang, Po-Kai Wang, Hung-Chieh Wan, Yi-Ying Lin, Ming-Chang Kao
Ischemia-reperfusion (IR) in the rat femoral artery has been proposed as an experimental model of vasculitic peripheral neuropathy (VPN) which presents neuropathic pain and peripheral nerve injury patterns observed clinically. This study investigates the involvement of the proinflammatory signaling pathway underlying the peripheral mechanisms of VPN. Male Sprague-Dawley rats were allocated to receive either a sham operation or IR. IR was induced by occluding the right femoral artery for 4h followed by reperfusion periods from 0 to 72h...
July 19, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28716862/gene-therapy-for-wiskott-aldrich-syndrome-in-a-severely-affected-adult
#4
Emma C Morris, Thomas Fox, Ronjon Chakraverty, Rita Tendeiro, Katie Snell, Christine Rivat, Sarah Grace, Kimberly Gilmour, Sarita Workman, Karen Buckland, Katie Butler, Ronnie Chee, Alan D Salama, Hazem Ibrahim, Havinder Hara, Cecile Duret, Fulvio Mavilio, Frances Male, Frederick D Bushman, Anne Galy, Siobhan O Burns, H Bobby Gaspar, Adrian J Thrasher
Until recently hematopoietic stem cell transplantation was the only curative option for Wiskott-Aldrich syndrome (WAS). The first attempts at gene therapy for WAS using a ϒ-retroviral vector improved immunological parameters substantially but were complicated by acute leukemia as a result of insertional mutagenesis in a high proportion of patients. More recently treatment of children with a state-of-the-art self-inactivating lentiviral vector (LV-w1.6 WASp) has resulted in significant clinical benefit without inducing selection of clones harbouring integrations near oncogenes...
July 17, 2017: Blood
https://www.readbyqxmd.com/read/28705292/a-case-of-ischemia-induced-perianal-ulceration-mimicking-pyoderma-gangrenosum
#5
Michael Joseph Lavery, Nicola Cooke
A 68-year-old woman, with a 75-pack-year smoking history and a history of chronic excess alcohol intake, presented with a 5-week history of worsening perianal pain and ulceration. She recently had an inpatient admission with back pain and urinary tract infection during which she developed diarrhea and fecal incontinence. Initially, the perianal ulceration was diagnosed as severe irritant contact dermatitis and treated with barrier creams and topical clobetasone 17-butyrate 0.05% weight for weight, oxytetracycline 3...
2017: Skinmed
https://www.readbyqxmd.com/read/28697227/postoperative-pyoderma-gangrenosum-following-video-assisted-thoracic-surgery
#6
Stanislav N Tolkachjov, Philip Y Sun, Alina G Bridges
<p>Pyoderma gangrenosum (PG) is a neutrophilic, ulcerative dermatosis that can develop at sites of cutaneous trauma, including surgical incisions, a phenomenon known as pathergy. The characteristic lesion is a painful, rapidly expanding ulceration with a violaceous undermined border.<sup>1</sup> A biopsy taken from the expanding violaceous border shows predominantly neutrophilic dermal inflammation with neutrophilic abscess formation.</p> <p>The etiology of PG appears to be variable among patients, as about a half of the reported cases are associated with systemic disease such as inflammatory bowel disease, rheumatoid arthritis, or myeloproliferative disorders, while the other half seem to be idiopathic...
July 1, 2017: Journal of Drugs in Dermatology: JDD
https://www.readbyqxmd.com/read/28690859/case-report-of-a-lupus-patient-with-a-severe-flare-and-miliary-tuberculosis-need-for-proper-guidelines-for-management
#7
Masooma Hashmat, Ramsa Suhail Rana, Tafazzul-E-Haque Mahmud, Aflak Rasheed, Ata Ur Rehman, Syed Ali Rukh Pirzada, Muhammad Ammad Asghar, Sadia Asif, Amjad Ali
Systemic lupus erythematosus (SLE) is an autoimmune disease that is frequently treated with high doses of corticosteroids and other immunosuppressive drugs. Thus patients with SLE are at increased risk for infections with several pathogens including Mycobacterium tuberculosis. There are no established guidelines available for treatment of tuberculosis in SLE patients with high disease activity due to lack of relevant studies and management based more on physician expertise. We report a case of a young SLE patient with high disease activity index (SLEDAI19) as evidenced by the presence of a vasculitic rash, non-healing ulcer on forearm and proteinuria of >1 g/d along with miliary tuberculosis...
July 2017: Oxford Medical Case Reports
https://www.readbyqxmd.com/read/28612332/cocaine-levamisole-induced-vasculitis-vasculopathy-syndrome
#8
REVIEW
Javier Marquez, Lina Aguirre, Carolina Muñoz, Andres Echeverri, Mauricio Restrepo, Luis F Pinto
PURPOSE OF REVIEW: To understand the clinical spectrum of cocaine-levamisole-induced vasculitis. Worldwide recreational drug consumption is high among the adult population from various social strata. The use of cocaine with levamisole, a frequently added antiparasitic diluent, favors the manifestations of vasculitic lesions, especially in the skin. RECENT FINDINGS: New insights into immunological mechanisms involved in the pathogenesis of the disease. There are still many unknown aspects in the pathogenesis of this disease, such as the immune system interaction with p-ANCAs and the release of inflammatory NETs (neutrophil extracellular traps), which are the origin of auto-antigens and tissue damage, manifesting as vasculitic purpura on the skin...
June 2017: Current Rheumatology Reports
https://www.readbyqxmd.com/read/28566611/vasculitic-neuropathy-following-exposure-to-a-glyphosate-based-herbicide
#9
Yuichi Kawagashira, Haruki Koike, Kazuya Kawabata, Mie Takahashi, Ken Ohyama, Rina Hashimoto, Masahiro Iijima, Masahisa Katsuno, Gen Sobue
We herein report a case of peripheral neuropathy following exposure to large amounts of glyphosate-based herbicide. A 70-year-old man suffered from pain and purpura in the left sole following exposure to glyphosate-based herbicide. Pain and purpura spread to the opposite side and increased in severity. Mild weakness of the lower limbs was also observed. A sural nerve biopsy revealed the infiltration of lymphocytes around small vessels in the epineurium with numerous eosinophils, deposition of hemosiderins and focal axonal degeneration, compatible with findings of vasculitic neuropathy...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28550073/lymphocyte-antigens-targetable-by-monoclonal-antibodies-in-non-systemic-vasculitic-neuropathy
#10
Christian Schneider, Gilbert Wunderlich, Johannes Bleistein, Gereon R Fink, Martina Deckert, Anna Brunn, Helmar Christoph Lehmann
OBJECTIVE: To identify the most relevant antigens for monoclonal antibodies in lymphocytic infiltrates in non-systemic vasculitic neuropathy (NSVN). BACKGROUND: Current immunosuppressive treatment for NSVN is insufficient. Monoclonal antibodies might be a treatment option, but the expression profile for targetable antigens on lymphocytic infiltrates in NSVN is unknown. METHODS: Sural nerve biopsies from a cohort of patients with NSVN were immunohistochemically studied for the expression of potential candidate antigens in perivascular and intramural lymphocytic infiltrates and correlated with neurological and electrophysiological parameters...
May 26, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28536112/necrotic-cutaneous-vasculitic-skin-lesions-a-case-of-atypical-henoch-sch%C3%A3-nlein-purpura-in-a-child-with-heterozygosity-for-factor-v-leiden
#11
Sacha Dhanjal, Anja Saso, Despina Eleftheriou, Sue Laurent
No abstract text is available yet for this article.
May 22, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28515509/anti-nuclear-cytoplasmic-antibody-associated-vasculitis-a-probable-adverse-effect-of-sofosbuvir-treatment-in-chronic-hepatitis-c-patients
#12
Youssef K Ahmad, Salwa Tawfeek, Mohamed Sharaf-Eldin, Hassan E Elbatea, Abdelrahman Kobtan, Ferial El-Kalla, Rehab Badawi, Sherief Abd-Elsalam
Background: Egypt has the largest hepatitis C virus (HCV) epidemic worldwide. Sofosbuvir is an antiviral drug acting by inhibition of the HCV NS5B polymerase. It has shown high efficacy in combination with several other drugs and has a low reported rate of side effects. Objective: The aim of this prospective cohort study was to assess the safety of sofosbuvir-based treatment regimens used to treat chronic hepatitis C infections and to detect any side effects of sofosbuvir not previously reported. Methods: We studied treatment side effects in 3,000 patients with chronic HCV infection treated with sofosbuvir and ribavirin for 24 weeks or treated by pegylated interferon, sofosbuvir, and ribavirin triple therapy for 12 weeks...
April 2017: Hospital Pharmacy
https://www.readbyqxmd.com/read/28490787/the-changing-face-of-polyarteritis-nodosa-and-necrotizing-vasculitis
#13
REVIEW
Seza Ozen
Polyarteritis nodosa (PAN) is a vasculitic disease characterized primarily by necrotizing vasculitis - inflammatory lesions in blood vessels that lead to vessel wall necrosis. Our understanding of PAN and necrotizing vasculitis has evolved over time. In addition to PAN, necrotizing vasculitis is now a recognized feature of a broad range of diseases with different aetiopathogenesis. For example, necrotizing vasculitis associated with hepatitis B virus infection has a different aetiopathogeneis to PAN and is now classified as a separate disease...
June 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28479458/true-ulnar-artery-aneurysm-in-the-proximal-forearm-case-report-and-literature-review
#14
S M McHugh, M A Moloney, E Greco, M Wheatcroft
INTRODUCTION: Ulnar artery aneurysms are rare with less than 150 previously reported. Previously ulnar aneurysms have been most commonly noted as occurring in the distal ulnar artery close to the palmar arch. CASE PRESENTATION: We present the case of a 47-year-old male with a background history of human immunodeficiency virus (HIV) who attended our outpatient clinic with symptoms of distal embolization from a proximal ulnar artery aneurysm. Pre-operatively the aneurysm was thought to arise from the distal brachial artery, and only intra-operatively was the diagnosis of ulnar aneurysm made...
May 4, 2017: Annals of Vascular Surgery
https://www.readbyqxmd.com/read/28447661/the-nonsystemic-vasculitic-neuropathies
#15
REVIEW
Michael P Collins, Robert D Hadden
Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy...
April 27, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28444097/chronic-polyarthritis-as-the-first-manifestation-of-childhood-systemic-polyarteritis-nodosa
#16
Glaucia Vanessa Novak, Koken Hayashi, Kohei Sampa, Yosuke Okumura, Gabriela Ribeiro Viola Ferreira, Clovis Artur Silva
Arthritis has been reported as an acute pattern, generally evanescent with oligoarthritis, mostly affecting knees and ankles in childhood systemic polyarteritis nodosa. However, chronic polyarthritis with morning stiffness mimicking juvenile idiopathic arthritis has not been reported. We describe the case of a 4-year old girl who had additive and chronic polyarthritis with edema, tenderness, pain on motion and morning stiffness for 2 months. After 45 days, she also presented painful subcutaneous nodules and erythematous-violaceous lesions in the extensor region of upper and lower limbs...
January 2017: Einstein
https://www.readbyqxmd.com/read/28442217/vasculopathic-and-vasculitic-dermatoses
#17
REVIEW
Alejandro A Gru, Andrea L Salavaggione
The inflammatory and non-inflammatory disorders affecting the blood vessels are an important cause of death among patients. Additionally, some of the findings could be very subtle, while the consequences of misdiagnosis tragic. There is a limited number of factors by which the cutaneous blood vessels can be affected and the most common examples in the clinical practice will be discussed here: non-inflammatory purpuras; vascular-occlusive disorders; urticarias; vasculitides; and neutrophilic dermatoses.
May 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/28431106/ultrasound-cut-off-values-for-intima-media-thickness-of-temporal-facial-and-axillary-arteries-in-giant-cell-arteritis
#18
Valentin S Schäfer, Aaron Juche, Sofia Ramiro, Andreas Krause, Wolfgang A Schmidt
Objective.: To evaluate the intima-media thickness (IMT) of arteries involved in GCA for determining cut-off values. Methods.: Forty newly diagnosed GCA patients in a fast-track GCA clinic and 40 age- and sex-matched controls were included. IMT measurement was performed at or within 24 h after diagnosis. The common superficial temporal arteries with their frontal and parietal branches and the facial arteries were bilaterally examined with a 10-22 MHz probe and the axillary artery with a 6-18 MHz probe...
April 20, 2017: Rheumatology
https://www.readbyqxmd.com/read/28405208/a-case-of-atypical-kawasaki-disease-with-giant-coronary-artery-aneurysm-containing-thrombus
#19
Eynaud S Micallef, Montalto S Attard, V Grech
INTRODUCTION: Kawasaki disease (KD) is an acute febrile, systemic vasculitic syndrome of unknown etiology, occurring primarily in children younger than 5 years of age. Administration of IVIG within the first 10 days after onset of fever in combination with high dose aspirin reduces the risk of coronary artery damage in KD. Though rare, giant aneurysms of the coronary arteries may develop in untreated cases and prove extremely challenging to manage. CASE PRESENTATION: A 9-month-old Caucasian boy presented to our paediatric emergency department with a 4-week history of intermittent pyrexia and irritability...
July 2016: Images in Paediatric Cardiology
https://www.readbyqxmd.com/read/28362518/role-of-angiography-in-systemic-lupus-erythematosus-induced-choroiditis
#20
Kenneth Rohan Lee, Lai Yin Peng, Tajunisah Begum Iqbal, Visvaraja Subrayan
PURPOSE: To report a case of systemic lupus erythematosus-induced choroidal vasculitis. METHODS: A 34-year-old woman with a long-standing history of systemic lupus erythematosus had a sudden painless loss of vision in the right eye over 12 hours. Ocular examination revealed a visual acuity of counting fingers of 1 foot on the right eye and 20/20 on the left. There was a relative afferent pupillary defect on the right side with a pink, distinct optic disk margin...
March 31, 2017: Ocular Immunology and Inflammation
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