keyword
https://read.qxmd.com/read/38619581/a-case-of-rapid-avacopan-induced-liver-injury-in-pediatric-granulomatosis-with-polyangiitis
#1
JOURNAL ARTICLE
Tomohiko Nishino, Shinya Tomori, Mei Haruyama, Kazuhiro Takahashi, Masakazu Mimaki
Children with anti-neutrophil cytoplasmic antibody-associated vasculitis benefit immensely from avacopan as it reduces the requirement for steroids. However, descriptions of adverse drug reactions in children are lacking, and the dosage and follow-up intervals are unclear. A 10-year-old boy with initial granulomatosis and polyangiitis presented with diffuse pulmonary hemorrhage. Rituximab and 30 mg avacopan were administered twice daily as induction therapy following methylprednisolone pulse therapy. However, sudden liver function test abnormalities were observed on day 31 of avacopan treatment, despite liver enzyme levels being within the normal range 5 days earlier...
April 15, 2024: Pediatric Nephrology
https://read.qxmd.com/read/38618140/anca-associated-vasculitis-with-isolated-splenomegaly-as-the-initial-organ-presentation
#2
Wataru Kitamura, Masatoshi Kuratsune, Akiko Iseki, Shoichi Kuyama
No abstract text is available yet for this article.
2024: Rheumatology Advances in Practice
https://read.qxmd.com/read/38617071/leukocytoclastic-vasculitis-in-a-patient-with-ankylosing-spondylitis-a-case-report
#3
Alireza Khabbazi, Sepideh Tahsini Tekantapeh, Cyrus Asadzadeh, Amir Vahedi
KEY CLINICAL MESSAGE: Although the concurrent occurrence of vasculitis with AS is uncommon, when patients diagnosed with AS exhibit symptoms including skin petechiae, purpura, abdominal discomfort, malaise, elevated ESR, and reduced complement levels, vigilant monitoring for vasculitis is advisable following the exclusion of secondary vasculitis triggers such as malignancies, infections, and pharmaceutical agents. ABSTRACT: The primary characteristic of ankylosing spondylitis (AS) involves inflammation occurring within the sacroiliac joint and the spine, leading to destruction and eventual ankylosis...
April 2024: Clinical Case Reports
https://read.qxmd.com/read/38616666/to-aslo-or-not-to-aslo-utility-of-the-aslo-test-in-dermatology
#4
JOURNAL ARTICLE
Afik Tibi, Ziyad Khamaysi, Emily Avitan-Hersh
BACKGROUND: Group A Streptococcus (GAS) causes a wide spectrum of acute infections and immune-related diseases, most of which include a dermatological presentation. However, dermatological findings have a wide range of other possible etiologies. The diagnosis of GAS-related disease requires an indication of preceding GAS infection by direct culture or by measuring antistreptolysin O (ASLO) titer. OBJECTIVES: To explore the correlation between ASLO positivity and dermatological diseases...
April 2024: Israel Medical Association Journal: IMAJ
https://read.qxmd.com/read/38616268/kawasaki-disease-in-kenya-and-review-of-the-african-literature
#5
JOURNAL ARTICLE
A Migowa, C M Njeru, E Were, T Ngwiri, I Colmegna, C Hitchon, R Scuccimarri
BACKGROUND: Kawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report. METHODS: A retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment...
April 14, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38615739/urticarial-vasculitis-a-rare-clinical-presentation-of-hodgkin-s-lymphoma
#6
JOURNAL ARTICLE
Chase P Rupprecht, Pranay Maniar, Alexandra E Conway, Chih- Yu Fang, Sarah A Taylor-Black
No abstract text is available yet for this article.
April 12, 2024: Annals of Allergy, Asthma & Immunology
https://read.qxmd.com/read/38614886/cost-effectiveness-analysis-of-the-diagnosis-of-temporal-arteritis
#7
JOURNAL ARTICLE
Isabel Del Blanco Alonso, Álvaro Revilla Calavia, Laura Saiz-Viloria, Manuel Diez Martínez, Enrique San Norberto García, Carlos Vaquero Puerta
UNLABELLED: Temporal arteritis (TA) is the most common form of systemic vasculitis. Its diagnosis is based on criteria proposed by the American College of Rheumatology (1990), and its treatment is high-dose corticosteroids. Our objective is to assess the cost of diagnosing TA, and secondarily, cost-effective analysis of different diagnostic strategies (clinical, biopsy, doppler ultrasound) and therapeutic strategies (corticosteroid suspension). MATERIAL AND METHOD: Observational, retrospective study has been carried out on patients with AT (2012-2021)...
April 12, 2024: Reumatología clinica
https://read.qxmd.com/read/38614731/a-case-of-anca-associated-vasculitis-presenting-as-a-chronic-cough-with-night-sweats-in-an-adult-male
#8
JOURNAL ARTICLE
Jacob Corum Williams, Ryan Malcolm Hum, Silke Brix, Pauline Ho
No abstract text is available yet for this article.
November 2023: Clinical Medicine: Journal of the Royal College of Physicians of London
https://read.qxmd.com/read/38614719/a-case-of-hsv2-vasculitis-causing-multi-territory-ischaemic-stroke-in-an-immunocompetent-man
#9
JOURNAL ARTICLE
Okkar Kyaw, Gagira Mudalige
No abstract text is available yet for this article.
November 2023: Clinical Medicine: Journal of the Royal College of Physicians of London
https://read.qxmd.com/read/38614661/sjogren-s-myelitis-presenting-as-hemicord-syndrome
#10
Andrew Lee, Ira Pande, Bruno Gran
We present a case where a 63-year-old right-handed man who presented with a 6-month history of progressive asymmetrical sensorimotor symptoms in lower limbs. This was associated with concomitant rash on the lower limbs, and mild sicca symptoms. MRI spine showed focal T2 hyperintensity in the left hemicord at C3-4 level. Skin biopsy of the rash revealed urticarial vasculitis, and lip biopsy revealed lymphocytic sialadenitis. Initial anti-Ro antibody was negative, but subsequent Ro52 antibody testing returned positive...
July 2023: Clinical Medicine: Journal of the Royal College of Physicians of London
https://read.qxmd.com/read/38614116/vasculitis-the-promising-road-ahead
#11
EDITORIAL
The Lancet Rheumatology
No abstract text is available yet for this article.
April 10, 2024: Lancet Rheumatology
https://read.qxmd.com/read/38613168/immunological-and-hematological-findings-as-major-features-in-a-patient-with-a-new-germline-pathogenic-cbl-variant
#12
Emilia Stellacci, Jennefer N Carter, Luca Pannone, David Stevenson, Dorsa Moslehi, Serenella Venanzi, Jonathan A Bernstein, Marco Tartaglia, Simone Martinelli
Casitas B-lineage lymphoma (CBL) encodes an adaptor protein with E3-ligase activity negatively controlling intracellular signaling downstream of receptor tyrosine kinases. Somatic CBL mutations play a driver role in a variety of cancers, particularly myeloid malignancies, whereas germline defects in the same gene underlie a RASopathy having clinical overlap with Noonan syndrome (NS) and predisposing to juvenile myelomonocytic leukemia and vasculitis. Other features of the disorder include cardiac defects, postnatal growth delay, cryptorchidism, facial dysmorphisms, and predisposition to develop autoimmune disorders...
April 12, 2024: American Journal of Medical Genetics. Part A
https://read.qxmd.com/read/38612603/erythrocyte-glutathione-s-transferase-activity-as-a-sensitive-marker-of-kidney-function-impairment-in-children-with-iga-vasculitis
#13
JOURNAL ARTICLE
Marijan Frkovic, Ana Turcic, Alenka Gagro, Sasa Srsen, Sanda Huljev Frkovic, Dunja Rogic, Marija Jelusic
IgA vasculitis (IgAV) is the most common childhood vasculitis. The main cause of morbidity and mortality in children with IgAV is nephritis (IgAVN), but the risk of its development, severity, and chronicity remain unclear. Erythrocyte glutathione S-transferase (e-GST) activity has been previously detected as a sensitive marker of kidney function impairment in several diseases. We spectrophotometrically assessed and correlated e-GST activity between 55 IgAV patients without nephritis (IgAVwN), 42 IgAVN patients, and 52 healthy controls...
March 28, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38612581/anti-lamp-2-antibody-seropositivity-in-children-with-primary-systemic-vasculitis-affecting-medium-and-large-sized-vessels
#14
JOURNAL ARTICLE
Tayfun Hilmi Akbaba, Kirandeep K Toor, Simranpreet K Mann, Kristen M Gibson, Gabriel Alejandro Alfaro, Banu Balci-Peynircioglu, David A Cabral, Kimberly A Morishita, Kelly L Brown
Chronic primary systemic vasculitis (PSV) comprises a group of heterogeneous diseases that are broadly classified by affected blood vessel size, clinical traits and the presence (or absence) of anti-neutrophil cytoplasmic antibodies (ANCA) against proteinase 3 (PR3) and myeloperoxidase (MPO). In small vessel vasculitis (SVV), ANCA are not present in all patients, and they are rarely detected in patients with vasculitis involving medium (MVV) and large (LVV) blood vessels. Some studies have demonstrated that lysosome-associated membrane protein-2 (LAMP-2/CD107b) is a target of ANCA in SVV, but its presence and prognostic value in childhood MVV and LVV is not known...
March 28, 2024: International Journal of Molecular Sciences
https://read.qxmd.com/read/38610060/deregulation-in-adult-iga-vasculitis-skin-as-the-basis-for-the-discovery-of-novel-serum-biomarkers
#15
JOURNAL ARTICLE
Matija Bajželj, Matjaž Hladnik, Rok Blagus, Vesna Jurčić, Ana Markež, Tanya Deniz Toluay, Snežna Sodin-Šemrl, Alojzija Hočevar, Katja Lakota
INTRODUCTION: Immunoglobulin A vasculitis (IgAV) in adults has a variable disease course, with patients often developing gastrointestinal and renal involvement and thus contributing to higher mortality. Due to understudied molecular mechanisms in IgAV currently used biomarkers for IgAV visceral involvement are largely lacking. Our aim was to search for potential serum biomarkers based on the skin transcriptomic signature. METHODS: RNA sequencing analysis was conducted on skin biopsies collected from 6 treatment-naïve patients (3 skin only and 3 renal involvement) and 3 healthy controls (HC) to get insight into deregulated processes at the transcriptomic level...
April 12, 2024: Arthritis Research & Therapy
https://read.qxmd.com/read/38609161/long-term-performance-of-show-jumping-horses-and-relationship-with-severity-of-ataxia-and-complications-associated-with-myeloencephalopathy-caused-by-equine-herpes-virus-1
#16
JOURNAL ARTICLE
María de la Cuesta-Torrado, Ana Velloso Alvarez, Patricia Neira-Egea, Juan Cuervo-Arango
BACKGROUND: Equine herpesvirus myeloencephalopathy (EHM) has severe impact on the sport horse population. OBJECTIVE: Study the influence of EHM on the likelihood of affected horses to return to their previous performance and investigate the association of clinical variables with prognosis. ANIMALS: Twenty-six horses positive for equine herpesvirus type 1 (EHV-1) were admitted to a veterinary teaching hospital (VTH) during a natural EHM outbreak at an international jumping event...
April 12, 2024: Journal of Veterinary Internal Medicine
https://read.qxmd.com/read/38608995/porto-sinusoidal-vascular-disorder-and-nephrotic-range-proteinuria-due-to-venous-vasculitis-in-beh%C3%A3-et-s-disease
#17
JOURNAL ARTICLE
M E Annink, T B van der Houwen, J J T H Roelofs, R B Takkenberg, J A M van Laar, N C van der Weerd, A E Hak, A J Kwakernaak
Behçet's disease (BD) is an autoinflammatory disease of multifactorial and polygenic etiology, potentially involving arteries and veins of any size resulting in variable vessel vasculitis. We report a case of an Iranian male that presented with porto-sinusoidal vascular disorder due to venous vasculitis as initial manifestation of BD. Despite immunosuppression and anticoagulation, he subsequently developed severe nephrotic-range proteinuria that mimicked a primary renal disease but was completely and immediately ameliorated by stenting of the vena cava...
April 10, 2024: Clinical Immunology: the Official Journal of the Clinical Immunology Society
https://read.qxmd.com/read/38607682/the-joint-vasculitis-registry-in-german-speaking-countries-gevas-subgroup-analysis-of-266-aav-patients
#18
JOURNAL ARTICLE
Sabrina Arnold, Pia Wallmeier, Arlette Tais, Gabriele Ihorst, Marco Janoschke, Fabian Schubach, Peer Aries, Raoul Bergner, Jan Phillip Bremer, Norman Görl, Eva Gutdeutsch, Bernhard Hellmich, Jörg Henes, Bimba Franziska Hoyer, Antje Kangowski, Ina Kötter, Martin Krusche, Tim Magnus, Claudia Metzler, Ulf Müller-Ladner, Jana Petersen, Anke Reichelt de Tenorio, Matthias Schaier, Jan Henrik Schirmer, Ulf Schönermarck, Jens Thiel, Leonore Unger, Nils Venhoff, Julia Weinmann-Menke, Christof Iking-Konert, Peter Lamprecht
OBJECTIVES: Prospective long-term observational data on the disease course of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) were missing in Germany to date. Therefore, the Joint Vasculitis Registry in German-speaking countries (GeVas) has been established to follow the course of patients with AAV. The aim of this study is to present baseline data of patients with newly diagnosed and relapsing AAV enrolled in the GeVas registry. METHODS: GeVas is a prospective, web-based, multicentre, clinician-driven registry for the documentation of organ manifestations, damage, long-term outcomes, and therapy regimens in various types of vasculitis...
April 12, 2024: Clinical and Experimental Rheumatology
https://read.qxmd.com/read/38606757/granulomatosis-with-polyangiitis-or-its-mimic-a-case-report
#19
JOURNAL ARTICLE
Tamás Major, Gábor Nagy, Judit Szabó, Huba Mózes, Gabriella Szűcs, Zoltán Szekanecz, Szilvia Szamosi
Differentiation between granulomatosis with polyangiitis (GPA) limited to the upper airways and cocaine-induced midline destructive lesion (CIMDL) may be particularly difficult because of their common histopathologic features and antineutrophil cytoplasmic antibody (ANCA) profiles. We herein present a case involving a young woman with an initial diagnosis of GPA based on upper and lower airway manifestations and constitutional symptoms, histopathologic evidence of granulomas, a positive cytoplasmic ANCA indirect immunofluorescent test result, and proteinase 3 positivity by enzyme-linked immunosorbent assay (ELISA)...
April 2024: Journal of International Medical Research
https://read.qxmd.com/read/38606534/breast-pain-differential-mondor-s-disease-of-the-breast
#20
JOURNAL ARTICLE
LaRae L Seemann, Tina Ardon, Rebecca A Bowie, Kati C Bullock, Adrianna D M Clapp
Breast pain is a common concern among women in primary care clinics. A rare cause of breast pain is Mondor's disease (MD), which can present as an acute, painful, erythematous, cord-like induration on the breast or anterior chest wall. The disorder is caused by sclerosing superficial thrombophlebitis of the anterolateral thoracoabdominal wall veins. There does not appear to be a racial or ethnic propensity for this condition; however, it is important to understand that it may be more difficult to see in darker skin types (Fitzpatrick skin types IV-VI) and requires close attention on physical exam...
2024: Journal of Investigative Medicine High Impact Case Reports
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