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granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/28924343/glaucoma-surgery-in-scleromalacia-using-endoscopic-cyclophotocoagulation-where-conventional-filtration-surgery-or-angle-procedures-are-contraindicated
#1
Ian As Rodrigues, Dan Lindfield, Miles R Stanford, Saurabh Goyal
AIM: To describe the surgical management of glaucoma in a patient with severe scleromalacia, and secondary angle closure. INTRODUCTION: The management of glaucoma with coexisting scleromalacia plus secondary angle closure is challenging as most commonly performed incisional glaucoma surgery as well as minimally invasive glaucoma surgery (MIGS), which targets the drainage angle are all contraindicated. CASE REPORT: Medically refractory glaucoma in a 60-year-old male with a 30-year history of granulomatosis with polyangiitis resulting in extensive severe scleromalacia, cicatricial lower lid retraction with significant conjunctival exposure, and widespread synechial angle closure from chronic anterior uveitis was managed with combined phacoemulsification cataract surgery, and endoscopic cyclophotocoagulation (ECP)...
May 2017: Journal of Current Glaucoma Practice
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
#2
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/28921160/gp2013-a-rituximab-biosimilar
#3
Hannah A Blair
GP2013 is the second biosimilar of the reference monoclonal anti-CD20 antibody rituximab to be approved in the EU. It is approved for use in all indications for which reference rituximab is approved, including follicular lymphoma (FL), diffuse large B-cell non-Hodgkin's lymphoma, chronic lymphocytic leukaemia, rheumatoid arthritis (RA), granulomatosis with polyangiitis and microscopic polyangiitis. GP2013 has similar physicochemical and pharmacodynamic properties to those of reference rituximab, and the pharmacokinetic biosimilarity of the agents has been shown in patients with RA...
September 18, 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28917259/successful-treatment-with-hyperbaric-oxygen-therapy-for-pneumatosis-cystoides-intestinalis-as-a-complication-of-granulomatosis-with-polyangiitis-a-case-report
#4
Kensuke Nakatani, Takaharu Kato, Shinichiro Okada, Risa Matsumoto, Kazuhiro Nishida, Hiroyasu Komuro, Toshiyuki Suganuma
BACKGROUND: Although gastrointestinal involvement in patients with granulomatosis with polyangiitis is uncommon, it is associated with mild to severe life-threatening complications. We present a case of pneumatosis cystoides intestinalis in a patient with granulomatosis with polyangiitis that was treated successfully with hyperbaric oxygen. CASE PRESENTATION: A 70-year-old Japanese man with a 3-year history of granulomatosis with polyangiitis consulted our hospital with a complaint of severe back pain...
September 17, 2017: Journal of Medical Case Reports
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
#5
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/28916114/-scleritis-and-episcleritis
#6
REVIEW
E Héron, T Bourcier
Episcleritis is generally a benign disease with a self-limited course, while scleritis is a severe ocular condition due to a risk of impaired vision in one-fifth of patients and its association with systemic diseases in one third of them. Infectious scleritis, representing 8 % of the etiologies, is mainly of herpetic origin (varicella zoster and herpes simplex viruses). A systemic autoimmune disease is observed in roughly 30 % of scleritis patients: inflammatory rheumatisms (15 %), firstly rheumatoid polyarthritis, systemic vasculitides (8 %), mainly granulomatosis with polyangiitis (Wegener's) and polychondritis, and less often inflammatory bowel disease and systemic lupus erythematosus...
September 12, 2017: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/28914375/central-nervous-system-involvement-in-patients-with-granulomatosis-with-polyangiitis-a-single-center-retrospective-study
#7
George E Fragoulis, Sophia Lionaki, Aliki Venetsanopoulou, Panayiotis G Vlachoyiannopoulos, Haralampos M Moutsopoulos, Athanasios G Tzioufas
The aims of this study were to estimate the frequency of central nervous system (CNS) involvement in Greek patients with granulomatosis with polyangiitis (GPA) and describe the related clinical characteristics and long-term outcomes of these patients. Medical charts of all ANCA-associated vasculitis patients were retrospectively reviewed, and GPA patients with CNS involvement were identified. Demographics, serological, and clinical features throughout the disease course were recorded. Comparisons of disease characteristics and long-term outcomes were performed between GPA patients with and without CNS involvement...
September 15, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28912820/granulomatosis-with-polyangiitis-with-myocarditis-and-ventricular-tachycardia
#8
Ramy Magdy Hanna, Eduardo Lopez, James Wilson
Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis, is a pulmonary-renal syndrome affecting small and medium sized blood vessels. The disease has a prevalence in studies ranging from 3 to 15.7 cases per 100,000, with a noted increasing incidence and prevalence in more recent studies. Pulmonary manifestations include hemorrhage, lung cavitary lesions, and pulmonary fibrosis. Within the kidney, GPA is known to cause rapidly progressive pauci-immune crescentic glomerulonephritis...
2017: Case Reports in Medicine
https://www.readbyqxmd.com/read/28905856/position-paper-revised-2017-international-consensus-on-testing-of-ancas-in-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#9
REVIEW
Xavier Bossuyt, Jan-Willem Cohen Tervaert, Yoshihiro Arimura, Daniel Blockmans, Luis Felipe Flores-Suárez, Loïc Guillevin, Bernhard Hellmich, David Jayne, J Charles Jennette, Cees G M Kallenberg, Sergey Moiseev, Pavel Novikov, Antonella Radice, Judith Anne Savige, Renato Alberto Sinico, Ulrich Specks, Pieter van Paassen, Ming-Hui Zhao, Niels Rasmussen, Jan Damoiseaux, Elena Csernok
Anti-neutrophil cytoplasmic antibodies (ANCAs) are valuable laboratory markers used for the diagnosis of well-defined types of small-vessel vasculitis, including granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). According to the 1999 international consensus on ANCA testing, indirect immunofluorescence (IIF) should be used to screen for ANCAs, and samples containing ANCAs should then be tested by immunoassays for proteinase 3 (PR3)-ANCAs and myeloperoxidase (MPO)-ANCAs. The distinction between PR3-ANCAs and MPO-ANCAs has important clinical and pathogenic implications...
September 14, 2017: Nature Reviews. Rheumatology
https://www.readbyqxmd.com/read/28895041/unmet-needs-in-the-pathogenesis-and-treatment-of-vasculitides
#10
REVIEW
Francesco Muratore, Giulia Pazzola, Alessandra Soriano, Nicolò Pipitone, Stefania Croci, Martina Bonacini, Luigi Boiardi, Carlo Salvarani
Despite the progress in the last years on the field of vasculitides, there are several unmet needs regarding classification, disease activity assessment, predictors of flares and complications, and type of treatment for the different forms. The 1990 American College of Rheumatology (ACR) classification criteria currently used to define giant cell arteritis and Takayasu arteritis were designed to discriminate between different types of vasculitides but not to differentiate vasculitis from other disorders. Recently, efforts have been made to overcome the shortcomings of the ACR criteria...
September 11, 2017: Clinical Reviews in Allergy & Immunology
https://www.readbyqxmd.com/read/28889234/peripheral-arterial-tonometry-in-pulmonary-vasculitis
#11
A Falkowski, K A Wardyn, K Życińska
Vascular wall inflammation in primary vasculitides results in diminished vessel dilation and finally impaired blood flow, causing multiple organs dysfunction and ultimate damage. In granulomatosis with polyangiitis (GPA), the inflammatory process concerns small and medium sized vessels and its pulmonary location is often predominant. The pivotal role in the development of that pathology plays vascular endothelium. Endothelial vasodilatory function strongly depends on the instant production and release of nitrogen oxide (NO), a potent local factor controlling vascular tonus...
September 10, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28884919/granulomatosis-with-polyangiitis-presenting-with-pyoderma-gangrenosum-like-ulceration-and-negative-cytoplasmic-antineutrophilic-cytoplasmic-antibodies-in-a-child
#12
Ashley Kass, Jake D Fagan, Paul Long
Granulomatosis with polyangiitis (GPA), a vasculitis that most commonly affects small to medium-size vessels of the respiratory tract and kidneys, may also present with a wide array of skin findings. We present the case of a 12-year-old boy with pyoderma gangrenosum-like ulcerations on his lower extremity as the initial manifestation of GPA despite negative cytoplasmic antineutrophilic cytoplasmic antibodies (c-ANCAs). Although GPA is strongly associated with c-ANCA, c-ANCA may be negative on presentation. Thus clinical and pathologic clues must be relied upon when serologic confirmation is negative...
September 2017: Pediatric Dermatology
https://www.readbyqxmd.com/read/28883246/dacryoadenitis-with-ptosis-and-diplopia-as-the-initial-presentation-of-granulomatosis-with-polyangiitis
#13
Makoto Hibino, Tetsuri Kondo
A 77-year-old Japanese woman presented to an ophthalmologist with an erythematous swollen upper eyelid and diplopia which was initially diagnosed to be idiopathic dacryoadenitis on the basis of a histological evaluation of an orbital mass that was in remission following a 3.5-month period of systemic corticosteroid therapy. She subsequently developed respiratory symptoms, and was finally diagnosed with systemic granulomatosis with polyangiitis (GPA) based on the clinical and histological features. She was successfully treated with corticosteroids and azathioprine...
September 6, 2017: Internal Medicine
https://www.readbyqxmd.com/read/28882637/successful-retrograde-intubation-after-failed-fiberoptic-intubation-and-percutaneous-cricothyrotomy
#14
Stephanie Brenman, Sachin Gupta, Stephanie Tseeng
BACKGROUND: An obstructive neck lesion presents an airway challenge for any emergency physician. Retrograde intubation is an infrequently used airway alternative that can be employed in the difficult airway algorithm that requires little training and is less invasive than surgical cricothyrotomy. CASE REPORT: We report a case of a 31-year-old male patient who presented with respiratory distress progressing to respiratory failure from upper airway obstruction. The patient had significant tracheal thickening at the level of the thyroid gland based on a computed tomography report from 3 weeks prior to his presentation...
September 4, 2017: Journal of Emergency Medicine
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
#15
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-anca-associated-vasculitis-in-olmsted-county-minnesota-usa-a-20-year-population-based-study
#16
Alvise Berti, Divi Cornec, Cynthia S Crowson, Ulrich Specks, Eric L Matteson
OBJECTIVE: To estimate the incidence, prevalence and mortality of ANCA-associated vasculitis (AAV) and its subsets, granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA) and eosinophilic GPA (EGPA), in a U.S. based adult population. METHODS: All medical records of patients with a diagnosis or suspicion of AAV in Olmsted County, Minnesota from January 1, 1996 to December 31, 2015 were reviewed. Incidence rates were age- and sex-adjusted to the 2010 US white population...
September 7, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28881107/saddle-nose-deformity-and-septal-perforation-in-granulomatosis-with-polyangiitis
#17
Annekatrin Coordes, Sonja Maike Loose, Veit M Hofmann, Grant S Hamilton, Frank Riedel, Dirk Jan Menger, Andreas E Albers
BACKGROUND: Patients who have granulomatosis with polyangiitis (GPA, syn. M. Wegener) often develop an external nose deformity which may have devastating psychological effects. Therefore, reconstruction of nasal deformities by rhinoplasty may become necessary to achieve a normal appearance. OBJECTIVE OF REVIEW: The aim of this systematic review was to investigate the efficacy and safety of surgical reconstruction in external nasal deformities and septal perforation in GPA patients...
September 7, 2017: Clinical Otolaryngology
https://www.readbyqxmd.com/read/28866833/voriconazole-induced-periostitis-deformans-serial-imaging-in-a-patient-with-anca-vasculitis
#18
S Cormican, N Adams, P O'Connell, A McErlean, D de Freitas
OBJECTIVES: A 61-year-old with acute granulomatosis and polyangiitis developed Aspergillus fumigatus pneumonia after admission to the intensive care unit with a small bowel perforation. This occurred after immunosuppression (intravenous methylprednisolone, intravenous cyclophosphamide, and plasmapheresis) for his initial presentation with stage 3 acute kidney injury. MATERIALS AND METHODS: The mycologist recommended long-term treatment with voriconazole after initial recovery...
September 2, 2017: Skeletal Radiology
https://www.readbyqxmd.com/read/28863794/immunoadsorption-in-autoimmune-diseases-affecting-the-kidney
#19
REVIEW
Georg Stummvoll, Martin Aringer, Ammon Handisurya, Kurt Derfler
Autoantibodies play an important role in the pathophysiology of renal involvement in systemic autoimmune diseases, such as systemic lupus erythematosus (SLE), systemic vasculitis, and anti-glomerular basement membrane disease (or Goodpasture syndrome). Direct removal of autoantibodies therefore has been tried in various ways, first by plasma exchange. Today, immunoadsorption is the extracorporeal method that most effectively removes (pathogenic) immune complexes and antibodies. Although past data have shown efficacy and biocompatibility of immunoadsorption in (renal) SLE, it is still an experimental and expensive procedure, and evidence from randomized controlled trials is needed...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28852483/immunoglobulin-levels-and-infection-risk-with-rituximab-induction-for-anti-neutrophil-cytoplasmic-antibody-associated-vasculitis
#20
Shivani Shah, Khushleen Jaggi, Keiko Greenberg, Duvuru Geetha
BACKGROUND: Rituximab (RTX), a B cell-depleting anti-CD20 monoclonal antibody, is approved for treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Low immunoglobulin (Ig) levels have been observed surrounding RTX treatment. The association between the degree of Ig deficiency and infection risk is unclear in AAV patients. METHODS: AAV patients treated with RTX for remission induction at a single center (2005-15) with serum Ig measurements were included...
August 2017: Clinical Kidney Journal
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