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https://www.readbyqxmd.com/read/29139157/seronegative-granulomatosis-with-polyangiitis-presenting-with-multiple-cranial-nerve-palsies
#1
Eunhye Lee, Jynkyun Park, Seung Hong Choi, Sung-Hye Park
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms...
November 14, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29115055/differences-between-adult-and-pediatric-onset-henoch-schonlein-purpura-from-north-india
#2
Vikas Gupta, Amita Aggarwal, Ranjan Gupta, Abhra Chandra Chowdhury, Vikas Agarwal, Able Lawrence, Ramnath Misra
AIM: Henoch-Schönlein purpura (HSP), a primary vasculitis, characterized by purpura, abdominal pain, arthritis and renal involvement, is predominantly a disease of childhood. However, rarely it can occur in adults in whom it is believed to be a more severe form with poor renal outcomes. We aimed to answer if the age of onset affected the clinical spectrum and renal outcomes of the disease in a north Indian population. Hence, we studied the differences in clinical spectrum and renal outcomes between adult-onset HSP and childhood-onset HSP...
November 8, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29109739/a-case-of-polyarteritis-nodosa-presenting-as-rapidly-progressing-intermittent-claudication-of-right-leg
#3
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/29106654/the-population-level-costs-of-immunosuppression-medications-for-the-treatment-of-glomerulonephritis-are-increasing-over-time-due-to-changing-patterns-of-practice
#4
Sean Barbour, Clifford Lo, Gabriela Espino-Hernandez, Sharareh Sajjadi, John Feehally, Scott Klarenbach, Jagbir Gill
Background: Immunosuppression (IS) is the main treatment for most types of glomerulonephritis (GN). Quantifying the cost of IS is necessary to ensure equitable access to therapies and optimal health outcomes, but the real-world cost of IS treatment for GN is largely unknown. We examined temporal changes in the population-level IS medication costs for GN over a 14-year period in a large Canadian province. Methods: We linked a provincial pathology database (containing all GN cases from 2000 to 2012) with renal and medication administrative databases to capture clinical characteristics and IS medications, with follow-up until 2013...
July 2, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29104431/a-case-of-rare-diffuse-alveolar-hemorrhage-and-review-of-literature
#5
Viviana Scollo, Luca Zanoli, Elisa Russo, Giulio Distefano, Francesco Rapisarda
It is well known that some disorders can cause concomitant kidney dysfunction with lung involvement. These syndromes, characterized by the simultaneous presence of intra-alveolar hemorrhage and acute glomerulonephritis, are caused by numerous and variable disorders. The most frequent are the antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitis and Goodpasture syndrome. A quick discerning of the underlying causes and initiation of adequate treatment is crucial to prevent acute respiratory failure and irreversible loss of renal function...
2017: Clinical Medicine Insights. Case Reports
https://www.readbyqxmd.com/read/29046034/-neurological-manifestations-in-atypical-kawasaki-disease
#6
Edgar Martínez-Guzmán, Luisa Berenise Gámez-González, Francisco Rivas-Larrauri, Giovanni Sorcia-Ramírez, Marco Yamazaki-Nakashimada
BACKGROUND: Kawasaki disease (KD) is a type of systemic vasculitis of unknown etiology. Atypical Kawasaki disease is defined as that where there are signs and symptoms not corresponding to the classical criteria for this nosological entity. Children with atypical Kawasaki disease may present with acute abdominal symptoms, meningeal irritation, pneumonia or renal failure. CLINICAL CASES: We describe 4 children with ages ranging from 2 to 12 years who had atypical Kawasaki disease, with neurological and gastrointestinal symptoms as part of the systemic presentation of the disease...
July 2017: Revista Alergia Mexico: Organo Oficial de la Sociedad Mexicana de Alergia e Inmunología, A.C
https://www.readbyqxmd.com/read/29045980/-anti-neutrophil-cytoplasmic-antibodies-associated-vasculitis-with-lung-hemorrhage-in-the-patient-on-maintenance-haemodialysis-a-case-report
#7
J X Ding, M Wang
Anti-neutrophil cytoplasmic antibodies (ANCA)-associated vasculitis (AAV) is usually a multisystem disorder, and pulmonary renal syndrome is a common presentation. Patients with AAV are less likely to experience relapse when they progress to end-stage renal disease (ESRD). We report a rare case of diffuse alveolar hemorrhage (DAH) in relapsing AAV after eight years of haemodialysis. A 58-year-old woman was admitted to our hospital with the chief complaints of dyspnea and hemoptysis accompanied by anemia, fever, fatigue, and weight loss...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29044584/direct-medical-costs-associated-with-the-extrahepatic-manifestations-of-hepatitis-c-virus-infection-in-france
#8
P Cacoub, M Vautier, A C Desbois, D Saadoun, Z Younossi
BACKGROUND: The economic impact of extrahepatic manifestations of hepatitis C virus (HCV) infection remains unknown for France. AIM: To estimate the prevalence of extrahepatic manifestations of HCV and the direct medical costs associated with them. METHODS: Estimates of 13 extrahepatic manifestations prevalence were obtained from (1) a retrospective data analysis of HCV-infected patients in a specialised centre and the baseline prevalence in the general French population and (2) an international systematic review...
October 18, 2017: Alimentary Pharmacology & Therapeutics
https://www.readbyqxmd.com/read/29043736/renal-involvement-in-paediatric-systemic-vasculitis
#9
Linnea Augestad, Clara Hammarström, Berit Flatø, Anna Bjerre
No abstract text is available yet for this article.
October 16, 2017: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://www.readbyqxmd.com/read/29043145/anca-associated-pauci-immune-glomerulonephritis-in-a-patient-with-bacterial-endocarditis-a-challenging-clinical-dilemma
#10
Andrea Cervi, Dylan Kelly, Iakovina Alexopoulou, Nader Khalidi
PURPOSE: We report the case of a 59-year-old man with chronic hepatitis B and C infection presenting with acute kidney injury and enterococcus faecalis-infective endocarditis (IE). An elevated proteinase-3 (PR3)-ANCA and pauci-immune glomerulonephritis (GN) on renal biopsy were discovered, corresponding to ANCA-mediated GN. We conducted a literature review to assess the role of ANCA in IE and treatment implications. METHODS: On systematic review of the literature, we found five previous cases whereby IE caused by streptococcus and bartonella species were related to ANCA vasculitis-associated GN...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29037908/new-insights-in-the-pathogenesis-of-immunoglobulin-a-vasculitis-henoch-sch%C3%A3-nlein-purpura
#11
REVIEW
Marieke H Heineke, Aranka V Ballering, Agnès Jamin, Sanae Ben Mkaddem, Renato C Monteiro, Marjolein Van Egmond
Immunoglobulin A vasculitis (IgAV), also referred to as Henoch-Schönlein purpura, is the most common form of childhood vasculitis. The pathogenesis of IgAV is still largely unknown. The disease is characterized by IgA1-immune deposits, complement factors and neutrophil infiltration, which is accompanied with vascular inflammation. Incidence of IgAV is twice as high during fall and winter, suggesting an environmental trigger associated to climate. Symptoms can resolve without intervention, but some patients develop glomerulonephritis with features similar to IgA nephropathy that include hematuria, proteinuria and IgA deposition in the glomerulus...
October 14, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29037311/therapeutic-plasma-exchange-in-rheumatic-diseases-a-university-hospital-experience
#12
Juan Pablo Córdoba, Carolina Larrarte, Cristina Estrada, Daniel G Fernández-Ávila
INTRODUCTION: Each day, evidence accumulates related to the use of therapeutic plasma exchange (TPE) in patients with rheumatic diseases. San Ignacio University Hospital has recorded all of the TPE sessions performed by the institution's apheresis group. OBJECTIVE: To describe the TPE experience of patients with rheumatologic diseases in a hospital setting. METHODS: Descriptive, observational, retrospective analysis. This study included analyses of the TPE sessions that were performed in patients with rheumatic diseases from November 2009 to November 2013...
September 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29033424/diffuse-alveolar-hemorrhage-in-iga-vasculitis-with-an-atypical-presentation
#13
Yuhei Ito, Machiko Arita, Shogo Kumagai, Reoto Takei, Maki Noyama, Fumiaki Tokioka, Takumi Nagamoto, Chieko Kawakita, Kenichiro Asano, Chika Okita, Tadashi Ishida
IgA vasculitis (IgAV) commonly occurs in young children, who present with a tetrad of purpura, abdominal pain, arthralgia and nephritis. Diffuse alveolar hemorrhage (DAH) is a rare complication of IgAV. We herein report an adult case of IgAV with a presentation of DAH and nephritis (pulmonary renal syndrome, PRS), but without other typical manifestations, such as purpura, abdominal pain and arthralgia. A 33-year-old man presented with hemoptysis and a low-grade fever and was diagnosed to have IgAV based on the results of a renal biopsy...
October 16, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29027625/serum-c3-complement-levels-in-anca-associated-vasculitis-at-diagnosis-is-a-predictor-of-patient-and-renal-outcome
#14
Matija Crnogorac, Ivica Horvatic, Patricia Kacinari, Danica Galesic Ljubanovic, Kresimir Galesic
AIM: To determinate the prognostic significance of low serum C3 at the time of diagnosis of ANCA-associated vasculitis (AAV). METHODS: Our cohort included 75 consecutive patients with AAV diagnosed from January 2005 to December 2015. C3 levels were measured at the time of diagnosis. Patients were divided into two groups, those with low serum C3 levels (< 0.9 g/l) and those with normal serum C3 levels (0.9-1.8 g/l). We analysed association between serum C3 levels and both combined and singularly patient and renal survival (ESRD)...
October 13, 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/29021484/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-complicated-by-periaortitis-and-cranial-hypertrophic-pachymeningitis-a-report-of-an-autopsy-case
#15
Hajime Kaga, Atsushi Komatsuda, Masaya Saitoh, Mizuho Nara, Ayumi Omokawa, Masaru Togashi, Shin Okuyama, Hideki Wakui, Naoto Takahashi
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a systemic inflammatory disorder categorized as small-vessel vasculitis. We herein report an elderly Japanese man with AAV (granulomatous with polyangiitis affecting the eyes, nose, lungs, and kidneys) who also showed periaortitis at the diagnosis and developed cranial hypertrophic pachymeningitis (HP) during steroid maintenance therapy. His consciousness disturbance caused by HP improved after steroid pulse therapy, but he died of aspiration pneumonia...
October 11, 2017: Internal Medicine
https://www.readbyqxmd.com/read/29021478/rhabdomyolysis-in-a-patient-with-polyarteritis-nodosa
#16
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/29016646/methotrexate-versus-cyclophosphamide-for-remission-maintenance-in-anca-associated-vasculitis-a-randomised-trial
#17
Federica Maritati, Federico Alberici, Elena Oliva, Maria L Urban, Alessandra Palmisano, Francesca Santarsia, Simeone Andrulli, Laura Pavone, Alberto Pesci, Chiara Grasselli, Rosaria Santi, Bruno Tumiati, Lucio Manenti, Carlo Buzio, Augusto Vaglio
OBJECTIVES: The treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is based on remission-induction and remission-maintenance. Methotrexate is a widely used immunosuppressant but only a few studies explored its role for maintenance in AAV. This trial investigated the efficacy and safety of methotrexate as maintenance therapy for AAV. METHODS: In this single-centre, open-label, randomised trial we compared methotrexate and cyclophosphamide for maintenance in AAV...
2017: PloS One
https://www.readbyqxmd.com/read/28984299/medical-renal-diseases-are-frequent-but-often-unrecognized-in-adult-autopsies
#18
Marie E Perrone, Anthony Chang, Kammi J Henriksen
Kidney diseases affect many hospitalized patients and contribute to morbidity and mortality. Therefore, kidney disease should be prevalent, but the frequency and spectrum of medical renal pathology in autopsy specimens has not been well documented. We sought to determine the spectrum of medical renal pathology in adult autopsy specimens and the frequency of overlooked diagnoses. We reviewed the hematoxylin- and eosin-stained kidney sections from 140 adult autopsies performed at a large teaching hospital over a 2-year period...
October 6, 2017: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
https://www.readbyqxmd.com/read/28977502/long-term-azathioprine-maintenance-therapy-in-anca-associated-vasculitis-combined-results-of-long-term-follow-up-data
#19
Anoek A E de Joode, Jan Stephan F Sanders, Xavier Puéchal, Loic P Guillevin, Thomas F Hiemstra, Oliver Flossmann, Nils Rasmussen, Kerstin Westman, David R Jayne, Coen A Stegeman
Objective: We studied whether in ANCA-associated vasculitis patients, duration of AZA maintenance influenced relapse rate during long-term follow-up. Methods: Three hundred and eighty newly diagnosed ANCA-associated vasculitis patients from six European multicentre studies treated with AZA maintenance were included; 58% were male, median age at diagnosis 59.4 years (interquartile range: 48.3-68.2 years); granulomatosis with polyangiitis, n = 236; microscopic polyangiitis, n = 132; or renal limited vasculitis, n = 12...
November 1, 2017: Rheumatology
https://www.readbyqxmd.com/read/28962592/targeted-proteomics-reveals-promising-biomarkers-of-disease-activity-and-organ-involvement-in-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#20
Jun Ishizaki, Ayako Takemori, Koichiro Suemori, Takuya Matsumoto, Yoko Akita, Ken-Ei Sada, Yukio Yuzawa, Koichi Amano, Yoshinari Takasaki, Masayoshi Harigai, Yoshihiro Arimura, Hirofumi Makino, Masaki Yasukawa, Nobuaki Takemori, Hitoshi Hasegawa
BACKGROUND: Targeted proteomics, which involves quantitative analysis of targeted proteins using selected reaction monitoring (SRM) mass spectrometry, has emerged as a new methodology for discovery of clinical biomarkers. In this study, we used targeted serum proteomics to identify circulating biomarkers for prediction of disease activity and organ involvement in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). METHODS: A large-scale SRM assay targeting 135 biomarker candidates was established using a triple-quadrupole mass spectrometer coupled with nanoflow liquid chromatography...
September 29, 2017: Arthritis Research & Therapy
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