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https://www.readbyqxmd.com/read/29260039/intracerebral-hemorrhage-after-iv-tpa-for-stroke-as-early-symptom-of-anca-associated-vasculitis
#1
Neda Zarghami Esfahani, Daniel M Anderson, Connie Pieper, Harold P Adams
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV) are rare diseases characterized by a necrotizing small-vessel vasculitis and circulating ANCA that comprise granulomatosis with polyangiitis, microscopic polyangiitis, and eosinophilic granulomatosis with polyangiitis (EGPA). Acute ischemic stroke (AIS) can be a manifestation of central nervous system (CNS) involvement in these diseases. Furthermore, intracerebral hemorrhage (ICH) is a potential complication of these necrotizing vasculitides...
December 2017: ENeurologicalSci
https://www.readbyqxmd.com/read/29258731/characteristics-and-visual-outcome-of-refractory-retinal-vasculitis-associated-with-antineutrophil-cytoplasm-antibody-associated-vasculitides
#2
Miaoli Lin, Stephen D Anesi, Lina Ma, Aseef Ahmed, Karen Small, C Stephen Foster
PURPOSE: To describe the clinical characteristics, therapies, visual outcomes and prognoses of patients with retinal vasculitis associated with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). DESIGN: Retrospective case series. METHODS: Patients diagnosed with retinal vasculitis associated with AAV and at least 6-months of follow-up were included. Demographic data, systemic and ocular features, best corrected visual acuity at the initial visit and latest visit, fluorescein angiography (FA) and indocyanine green angiography (ICGA) findings, therapy regimen and outcome were collected from the Massachusetts Eye Research and Surgery Institution (MERSI) database from 2006 to 2017...
December 16, 2017: American Journal of Ophthalmology
https://www.readbyqxmd.com/read/29189887/eosinophilic-granulomatosis-with-polyangiitis-without-respiratory-symptoms-or-asthma-in-an-adolescent-case-report-and-literature-review
#3
REVIEW
Gülçin Otar Yener, Zahide Ekici Tekin, Neşe Çallı Demirkan, Selçuk Yüksel
Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a systemic necrotizing vasculitis of the small and medium vessels. It is primarily associated with respiratory conditions such as asthma and sinusitis as well as eosinophilia, neuropathy, pulmonary infiltrates, and vasculitis. EGPA is extremely rare in the pediatric age group, and respiratory system disorders are usually predominant in EGPA patients. A 14-year-old boy presented with rash and severe extremity pain...
November 30, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29185962/renal-outcome-of-kidney-transplantation-in-korean-recipients-with-anca-associated-vasculitis
#4
Eun Seong Park, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We investigated renal outcome of kidney-transplantation in Korean recipients with biopsy-proven renal involvement of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) in a single centre. METHODS: We reviewed the medical records of 144 Korean patients and included 3 female patients with microscopic polyangiitis (MPA) and one male patient with eosinophilic granulomatosis with polyangiitis (EGPA) in this study. We obtained clinical and laboratory data related to kidney-transplantation, analysed renal outcome of kidney-transplantation in 4 recipients with AAV and compared it with those of previous studies...
November 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29185957/the-utility-of-the-acr-eular-2017-provisional-classification-criteria-for-granulomatosis-with-polyangiitis-in-korean-patients-with-antineutrophil-cytoplasmic-antibody-associated-vasculitis
#5
Juyoung Yoo, Ho Jae Kim, Sung Soo Ahn, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
OBJECTIVES: We applied the ACR/EULAR 2017 provisional classification criteria for granulomatosis with polyangiitis (GPA) to 150 Korean patients with previously diagnosed antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) and investigated how many patients with AAV were reclassified as GPA. METHODS: We included patients with 30 GPA, 30 eosinophilic GPA (EGPA) and 90 microscopic polyangiitis (MPA) patients. Patients can be classified as GPA, when the sum of scores is more than 5...
November 27, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29180123/childhood-versus-adult-onset-anca-associated-vasculitides-a-nested-matched-case-control-study-from-the-french-vasculitis-study-group-registry
#6
REVIEW
Michele Iudici, Christian Pagnoux, Pierre Quartier, Matthias Büchler, Ramiro Cevallos, Pascal Cohen, Claire de Moreuil, Philippe Guilpain, Alain Le Quellec, Jacques Serratrice, Benjamin Terrier, Loïc Guillevin, Luc Mouthon, Xavier Puéchal
OBJECTIVE: To investigate differences between childhood-onset ANCA-associated vasculitides (cAAVs) and matched adult-onset controls (aAAVs). METHODS: cAAV clinical pictures at onset and outcomes were compared to a randomly selected sample of aAAV patients from the French Vasculitis Study Group Registry. Cases and controls were matched for AAV (granulomatosis with polyangiitis [GPA], microscopic polyangiitis [MPA] or eosinophilic granulomatosis with polyangiitis [EGPA]), sex and year of enrollment...
November 24, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29127573/eosinophilic-granulomatosis-with-polyangiitis-complicated-by-subarachnoid-hemorrhage-and-coronary-vasculitis-a-case-report-and-review-of-the-literature
#7
REVIEW
Shogo Matsuda, Shuzo Yoshida, Youhei Fujiki, Hidetoshi Satomi, Tohru Takeuchi, Yoshinobu Hirose, Shigeki Makino, Shigeki Arawaka
Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by necrotizing vasculitis of small-sized vessels with extravascular granulomas and eosinophilic infiltration. The case of a 48-year-old Japanese woman with EGPA, who presented concurrently with subarachnoid hemorrhage (SAH) and coronary vasculitis, is reported. She initially presented with bronchial asthma, and then 8 months later she developed various symptoms caused by systemic eosinophilic vasculitis and was admitted to our hospital. Three days after admission, she started oral corticosteroid therapy, and her 2009 Five-Factor Score (FFS) was 0...
November 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29124398/patient-perceptions-of-glucocorticoids-in-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#8
Joanna C Robson, Jill Dawson, Peter F Cronholm, Susan Ashdown, Ebony Easley, Katherine S Kellom, Don Gebhart, Georgia Lanier, Nataliya Milman, Jacqueline Peck, Raashid A Luqmani, Judy A Shea, Gunnar Tomasson, Peter A Merkel
Granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA) are multisystem diseases of small blood vessels, collectively known as the anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). This study explores the patient's perspective on the use of glucocorticoids, which are still a mainstay of treatment in AAV. Patients with AAV from the UK, USA, and Canada were interviewed, using purposive sampling to include a range of disease manifestations and demographics...
November 9, 2017: Rheumatology International
https://www.readbyqxmd.com/read/29119480/rheumatoid-factor-false-positivity-in-patients-with-anca-associated-vasculitis-not-having-medical-conditions-producing-rheumatoid-factor
#9
Jae-Seung Moon, Diane Da-Hyun Lee, Yong-Beom Park, Sang-Won Lee
We investigated the rate of rheumatoid factor (RF) false positivity at diagnosis and its influence on clinical and prognostic features and rheumatoid arthritis (RA) development during the follow-up in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) patients without RA or other medical conditions triggering RF false positivity. We reviewed the medical records of 128 AAV patients. We divided patients with AAV and each variant into two groups according to RF positivity and compared variables between the two groups...
November 8, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29022232/a-case-of-fatal-perimyocarditis-due-to-a-rare-disease
#10
Stefanie Plenzig, Sara Heinbuch, Hannelore Held, Marcel A Verhoff, Constantin Lux
Although myocarditis is caused by viral infections in about 50% of cases in European countries, various other causative agents are known. We report the case of a 51-year-old man who died several months after being diagnosed with asthma by his general practitioner. This diagnosis had been confirmed by a pulmonologist approximately 6 weeks before the man's death. To rule out the possibility of medical malpractice the prosecuting authority ordered a forensic autopsy. At autopsy macroscopic indicators for perimyocarditis and pneumonia were found...
December 2017: Forensic Science, Medicine, and Pathology
https://www.readbyqxmd.com/read/29016646/methotrexate-versus-cyclophosphamide-for-remission-maintenance-in-anca-associated-vasculitis-a-randomised-trial
#11
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/28988280/echocardiographic-features-in-patients-with-anca-associated-vasculitis-within-3%C3%A2-months-before-and-after-diagnosis
#12
Sung Soo Ahn, Eun Seong Park, Seung Min Jung, Jason Jungsik Song, Yong-Beom Park, Sang-Won Lee
We investigated the all-inclusive echocardiographic features in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) within 3 months before and after diagnosis. We reviewed the medical records of 89 AAV patients taking echocardiography and 35 age- and gender-matched controls. We collected clinical and laboratory data and echocardiographic results. We compared the variables between patients with AAV of each variant and controls and among those with microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic GPA (EGPA)...
December 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28983650/-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome
#13
REVIEW
A Jerrentrup, T Müller, J Mariss, S Dylla
This article presents the case of a patient with dyspnea, a history of poorly controlled asthma, sinonasal polyposis, blood eosinophilia and transient pulmonary infiltrates. The autoantibodies antinuclear antibodies, cytoplasmic anti-neutrophil cytoplasmic antibodies (c-ANCA) and perinuclear anti-neutrophil cytoplasmic antibodies (p-ANCA) were negative. These symptoms are typical for eosinophilic granulomatosis with polyangiitis (EGPA, alternatively known as Churg-Strauss syndrome). The delay between onset and diagnosis in this case was several years...
October 5, 2017: Der Internist
https://www.readbyqxmd.com/read/28956128/anti-neutrophil-cytoplasmic-antibody-associated-vasculitis-prevalence-treatment-and-outcomes
#14
REVIEW
Lei Shi
Anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) is a primary small-vessel vasculitis group with three distinct clinical entities, including GPA, MPA, and EGPA. The incidence of AAV has increased since the 1980s and remained stable since the early 2000s. Distinct phenotypes of AAV may also differ in various geographical regions. Elderly people are susceptible to developing AAV, and AAV is used to be a fatal disease before the introduction of glucocorticoids and immunosuppressants. Different treatment protocols should be employed for patients with different disease severity levels...
November 2017: Rheumatology International
https://www.readbyqxmd.com/read/28950436/eosinophilic-granulomatosis-with-polyangiitis-toward-personalized-treatment
#15
Xavier Puéchal, Christian Pagnoux, Loïc Guillevin
Dr. Smitienko and colleagues commented on our trial on the interest of azathioprine (AZA) adjunction to glucocorticoids to induce remission of systemic necrotizing vasculitides (SNVs) without poor-prognosis factors, more specifically on eosinophilic granulomatosis with polyangiitis (EGPA). We fully agree that EGPA is a heterogenous disease. Indeed, we (1) and others (2) demonstrated that antineutrophil cytoplasm antibody (ANCA)-positive and ANCA-negative EGPA differ phenotypically. Among the CHUSPAN2 trial's 95 patients, 51 had new-onset EGPA, 19 (37%) of which were anti-myeloperoxidase-positive...
September 26, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28924115/neurological-complications-in-eosinophilic-granulomatosis-with-polyangiitis-egpa-the-roles-of-history-and-the-results-of-physical-examinations-in-the-diagnosis-of-egpa
#16
Hiroshi Oiwa, Sho Mokuda, Tomoyasu Matsubara, Masamoto Funaki, Ikuko Takeda, Takemori Yamawaki, Kazuhiko Kumagai, Eiji Sugiyama
Objective To investigate the clinical symptoms, the physical and neurological findings, and the clinical course of neurological complications in eosinophilic granulomatosis with polyangiitis (EGPA). Methods A retrospective chart review of EGPA cases managed by two referral hospitals was performed, with a focus on the neurological findings. The study analyzed the symptoms at the onset of EGPA and investigated their chronological relationship. The patient delay (the delay between the onset of symptoms and the initial consultation), and the physician delay (the delay from consultation to the initiation of therapy) were determined and compared...
September 15, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28918161/clinical-and-pathological-significance-of-cutaneous-manifestations-in-anca-associated-vasculitides
#17
REVIEW
Laure Frumholtz, Sara Laurent-Roussel, Olivier Aumaître, François Maurier, Guillaume Le Guenno, Agnes Carlotti, Alexiane Dallot, Jean Louis Kemeny, Laurent Antunes, Nicolas Froment, Sylvie Fraitag, Jonathan London, Alice Berezne, Benoît Terris, Claire Le Jeunne, Luc Mouthon, Selim Aractingi, Loïc Guillevin, Nicolas Dupin, Benjamin Terrier
OBJECTIVES: Cutaneous manifestations (CM) in ANCA-associated vasculitides (AAV) are frequent, but data on clinical significance and clinical-pathological correlations are lacking. METHODS: We conducted a multicenter, retrospective study including 1553 AAV patients. Clinical, biological and pathological features have been analyzed, and tissue samples from 46 biopsies were reviewed in a blind manner. RESULTS: CM were more frequent in EGPA (53...
November 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28916432/rituximab-as-induction-therapy-in-eosinophilic-granulomatosis-with-polyangiitis-refractory-to-conventional-immunosuppressive-treatment-a-36-month-follow-up-analysis
#18
Jens Thiel, Arianna Troilo, Ulrich Salzer, Theresa Schleyer, Kirsten Halmschlag, Marta Rizzi, Natalie Frede, Ana Venhoff, Reinhard E Voll, Nils Venhoff
BACKGROUND: Rituximab (RTX) is approved for induction therapy of granulomatosis with polyangiitis and microscopic polyangiitis. In eosinophilic granulomatosis with polyangiitis (EGPA), organ-threatening manifestations are mainly treated with cyclophosphamide (CYC). RTX as treatment in EGPA has been described in small case series; however long-term data and the efficacy of RTX in EGPA refractory to CYC have not been reported yet. OBJECTIVES: To investigate the efficacy and safety of RTX and conventional immunosuppressive therapy with CYC in EGPA as induction therapy and during long-term follow-up...
September 12, 2017: Journal of Allergy and Clinical Immunology in Practice
https://www.readbyqxmd.com/read/28881714/the-4-plus-2-rituximab-protocol-makes-maintenance-treatment-unneeded-in-patients-with-refractory-anca-associated-vasculitis-a-10-years-observation-study
#19
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: ANCA associated vasculitides (AAV) often present with a chronic relapsing course. Relapse leads to increased immunosuppressive exposure and consequent toxicity. While two randomized controlled trials have shown rituximab (RTX) to be the most effective induction treatment in patients with relapsing disease, the optimal treatment duration and RTX dose remain debated. Whether to administer a maintenance dose to every patient, at a fixed time interval or on the basis of B cell count and ANCA titre or only when disease manifestations do occur is still debated as well...
August 8, 2017: Oncotarget
https://www.readbyqxmd.com/read/28881446/the-epidemiology-of-antineutrophil-cytoplasmic-autoantibody-associated-vasculitis-in-olmsted-county-minnesota-a-twenty-year-us-population-based-study
#20
Alvise Berti, Divi Cornec, Cynthia S Crowson, Ulrich Specks, Eric L Matteson
OBJECTIVE: To estimate the annual incidence, prevalence, and mortality of antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (Wegener's) (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA), in a US-based adult population. METHODS: All medical records of patients with a diagnosis of, or suspicion of having, AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed...
September 7, 2017: Arthritis & Rheumatology
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