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granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/28440998/-extranodal-nk-t-cell-lymphoma-nasal-type-in-granulomatosis-with-polyangiitis-a-case-report
#1
Sergio Alberto Mendoza-Álvarez, Fátima Margarita Rodríguez-Dávila, Leslie Moranchel-García, Virginia Soto, Natalia Quisped
BACKGROUND: Granulomatosis with polyangiitis (GP) is a systemic necrotizing vasculitis with multi-organ involvement that primarily affects the respiratory tract and the kidneys. Fever in these patients is an indicator of activity; however, if it arises in isolation, the physician should exclude other causes. CLINICAL CASE: Male patient admitted due to an unexplained fever and weight loss; it was diagnosed GP by a history of pauci-immune glomerulonephritis, fixed pulmonary nodules and chronic sinusitis of four years of evolution; however, the fever persisted despite treatment and in the absence of infection...
May 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28440995/-the-first-clinical-description-of-granulomatosis-with-polyangiitis-known-before-as-wegener-s-granulomatosis
#2
Ulises Mercado
Before 1950, cases of necrotizing vasculitis were commonly published in journals of pathology. Most of these cases were designated as polyarteritis nodosa. In 1952, the pathologist Pearl Zeek critically reviewed and summarized the literature dealing with polyarteritis nodosa and first grouped the different types of necrotizing vasculitis. But she omitted some types of not well-characterized vasculitis, among them granulomatosis with polyangiitis (Wegener's granulomatosis).
May 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28421235/koebner-phenomenon-in-eosinophilic-granulomatosis-with-polyangiitis-churg-srauss
#3
Maylis Tourte, Coralie Lheure, Julien Gras, Maxime Battistella, Isabelle Simon, Claire Hussenet, Marie Jachiet, Martine Bagot, Abdellatif Tazi, Anne Bergeron, Jean-David Bouaziz
No abstract text is available yet for this article.
April 19, 2017: Acta Dermato-venereologica
https://www.readbyqxmd.com/read/28420847/the-successful-treatment-of-myeloperoxidase-antineutrophil-cytoplasmic-antibody-positive-hypertrophic-pachymeningitis-in-patients-with-the-limited-form-of-granulomatosis-with-polyangiitis-using-methotrexate-two-case-reports
#4
Shinjiro Kaieda, Naomi Yoshida, Midori Minezaki, Shuri Ushijima, Daisuke Wakasugi, Shiroh Miura, Yusuke Uchiyama, Hiroaki Ida, Tomoaki Hoshino
Recent findings have indicated a close relationship between myeloperoxidase antineutrophil cytoplasmic antibody (MPO-ANCA)-positive hypertrophic pachymeningitis and the limited form of granulomatosis with polyangiitis (GPA). In Japan, MPO-ANCA-positive hypertrophic pachymeningitis predominantly occurs in elderly individuals. We herein describe the cases of two patients with MPO-ANCA-positive hypertrophic pachymeningitis associated with the limited form of GPA who were successfully treated with a combination of corticosteroids and methotrexate...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28418953/pulmonary-perfusion-scan-mimicking-hepatobiliary-scintigraphy-in-a-patient-with-an-uncommon-manifestation-of-granulomatosis-with-polyangiitis
#5
Michelle D Hopstone, Evan A Raff, Patrick M Colletti
A 36-year-old man with known granulomatosis with polyangiitis underwent quantitative ventilation-perfusion pulmonary scintigraphy as part of an initial evaluation for lung transplant candidacy. Radiotracer uptake on the perfusion study has the appearance of the hepatic silhouette, drawing initial concern that there may have been a misadministration of Tc with a hepatobiliary agent. Comparison CT reveals extensive cystic bronchiectasis, parenchymal destruction, and volume loss involving the entire left lung and basal portions of the right lung, with the residual portions of functional lung parenchyma conforming to a shape similar to the liver...
April 17, 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28413313/additional-risk-minimisation-measures-for-medicinal-products-in-the-european-union-a-review-of-the-implementation-and-effectiveness-of-measures-in-the-united-kingdom-by-one-marketing-authorisation-holder
#6
Elaine Agyemang, Lorna Bailey, John Talbot
INTRODUCTION: Additional risk minimisation measures (aRMMs) for medicinal products are necessary to address specific important safety issues which may not be practically achieved through routine risk management measures alone. The implementation and determination of effectiveness for aRMMs can be a challenge as it involves multiple stakeholders. It is therefore important to have concise objectives to avoid undue burden on patients, healthcare professionals and the healthcare system. AIM: The aim of this study was to examine how aRMMs are implemented and how effectiveness is assessed in the European Union (EU) using practical examples from Roche Products Limited in the United Kingdom (UK) (referred to as the 'Company')...
2017: Pharmaceutical Medicine
https://www.readbyqxmd.com/read/28413094/-pachymeningitis
#7
O Fain, A Mekinian
Pachymeningitis is a fibrosing and inflammatory process, which involves the dura mater. Some pachymeningitis are cranial and induce headaches and cranial nerve palsies. Others are spinal and responsible for nerve roots or spinal cord compression. MRI shows contrast enhancement thickening of the dura mater. Etiologies are infectious (syphilis, tuberculosis, etc.) or inflammatory (sarcoidosis, granulomatosis with polyangiitis, IgG4-related disease, idiopathic). Corticosteroids are the main treatment. The use of immunosuppressive drugs or rituximab is yet to be determined and probably adapted to each etiology...
April 13, 2017: La Revue de Médecine Interne
https://www.readbyqxmd.com/read/28412705/interferon-%C3%AE-for-induction-and-maintenance-of-remission-in-eosinophilic-granulomatosis-with-polyangiitis-a-single-center-retrospective-observational-cohort-study
#8
Benjamin Seeliger, Martin Förster, Janett Happe, Thomas Forberg, Anne Moeser, Thomas Neumann, Claus Kroegel
OBJECTIVE: Eosinophilic granulomatosis with polyangiitis (EGPA) is characterized by frequent relapses following induction therapy. Interferon-α (IFN-α) can reverse the underlying Th2-driven immune response and has successfully induced remission in previous reports. We undertook this study to investigate its efficacy and safety in patients with EGPA. METHODS: We conducted a retrospective monocentric cohort study including 30 patients (16 women) with active EGPA under IFN-α treatment...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#9
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28405696/-varicella-gastritis-under-immunosuppression-case-report-of-a-woman-after-lung-transplantation-due-to-granulomatosis-with-polyangiitis
#10
S Saman, J-C Henes, D Niepel, H Bosmüller, C R Werner, U M Lauer, N P Malek, T Xenitidis
A 35-year-old woman who had previously undergone a lung transplantation presented with severe abdominal pain and vomiting. The gastroscopy showed diffuse ulcerative gastric lesions. Tests for varicella zoster virus and Epstein-Barr virus via polymerase chain reactions (PCR) on endoscopically obtained gastric biopsies were found to be positive and confirmed varicella gastritis. Intravenous antiviral therapy with acyclovir was administered resulting in a normalization of all clinical symptoms, especially of abdominal pain and inflammation parameters...
April 12, 2017: Der Internist
https://www.readbyqxmd.com/read/28398895/granulomatosis-with-polyangiitis-presenting-as-a-pituitary-lesion
#11
Louise Christou, Ajit Thomas, Shouma Dutta, Simon Shaw, Biju Jose
No abstract text is available yet for this article.
April 2, 2017: British Journal of Hospital Medicine
https://www.readbyqxmd.com/read/28396741/renal-allograft-granulomatous-interstitial-nephritis-observations-of-an-uncommon-injury-pattern-in-22-transplant-recipients
#12
Alton B Farris, Carla L Ellis, Thomas E Rogers, W James Chon, Anthony Chang, Shane M Meehan
Background: Granulomatous interstitial nephritis (GIN) is uncommon in native kidneys, and descriptions in allografts are few. We report clinical and pathologic findings in 22 allograft recipients with GIN identified in renal allograft biopsies and nephrectomies. Methods: Renal allografts with GIN were retrieved from the pathology files of two academic medical centers. Available clinical and pathologic data were compiled retrospectively for a 23-year period. Results: GIN was present in 23 specimens from 22 patients (15 males and 7 females) with allograft dysfunction [serum creatinine averaged 3...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28387944/th17-and-treg-lymphocytes-as-cellular-biomarkers-of-disease-activity-in-granulomatosis-with-polyangiitis
#13
Lorenzo Cosmi
Granulomatosis with Polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is a vasculitis of unknown etiology affecting predominantly small- to medium-sized vessels, usually involving the upper and lower respiratory tract and kidneys. Anti-neutrophil cytoplasmic autoantibodies are probably the initial cause of the inflammatory process that leads to the typical necrotizing lesions. In this issue of the European Journal of Immunology, Szczeklik et al. [Eur. J. Immunol. 2017. 47: 724-733] report some interesting findings on the possible involvement of T-cell subsets in the pathogenesis of the disease...
April 2017: European Journal of Immunology
https://www.readbyqxmd.com/read/28386506/an-unusual-presentation-of-limited-granulomatosis-with-polyangiitis-involving-vagina-and-urethra
#14
Sandra Soro Marín, Enrique Júdez Navarro, Manuela Sianes Fernández, Ginés Sánchez Nievas, Juan Gabriel Lorenzo Romero
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing granulomatosis vasculitis characterized by predilection to affect small- and medium-sized blood vessels and commonly affects the upper and lower respiratory tract and kidneys in most cases. Genital involvement is reported in <1% of cases in large cohorts and nearly all cases have been in the setting of multisystemic disease or during the course of the disease in patients already diagnosed as GPA. A case is presented of uncommon limited urogenital GPA in a 66-year-old woman with an irregular mass occupying urethra and vagina...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/28382598/patients-with-anca-associated-vasculitis-admitted-to-the-intensive-care-unit-with-acute-vasculitis-manifestations-a-retrospective-and-comparative-multicentric-study
#15
Julien Demiselle, Johann Auchabie, François Beloncle, Philippe Gatault, Steven Grangé, Damien Du Cheyron, Jean Dellamonica, Sonia Boyer, Dimitri Titeca Beauport, Lise Piquilloud, Julien Letheulle, Christophe Guitton, Nicolas Chudeau, Guillaume Geri, François Fourrier, René Robert, Emmanuel Guérot, Julie Boisramé-Helms, Pierre Galichon, Pierre-François Dequin, Alexandre Lautrette, Pierre-Edouard Bollaert, Ferhat Meziani, Loïc Guillevin, Nicolas Lerolle, Jean-François Augusto
PURPOSE: Data for ANCA-associated vasculitis (AAV) patients requiring intensive care are scarce. METHODS: We included 97 consecutive patients with acute AAV manifestations (new onset or relapsing disease), admitted to 18 intensive care units (ICUs) over a 10-year period (2002-2012). A group of 95 consecutive AAV patients with new onset or relapsing disease, admitted to two nephrology departments with acute vasculitis manifestations, constituted the control group...
December 2017: Annals of Intensive Care
https://www.readbyqxmd.com/read/28381327/autoimmune-related-nasal-septum-perforation-a-case-report-and-systematic-review
#16
Lohitha Guntupalli, Kunjan Patel, Farhoud Faraji, Joseph D Brunworth
BACKGROUND: Inflammatory injury of nasal respiratory mucosa is a common feature of multisystem autoimmune disease. Certain autoimmune disorders are associated with nasal septum perforation (NSP). We performed a systematic review of the literature to better understand the association of NSP with specific autoimmune disorders. This is a case report of a 29-year-old woman with a history of arthralgia, autoreactive antibody titers, platelet dysfunction, and NSP. The constellation of symptoms and potential familial involvement indicated that the NSP in this patient was an early sign of an autoimmune disorder, an unknown autoimmune disorder, or a known disease with incomplete penetrance...
March 1, 2017: Allergy & Rhinology
https://www.readbyqxmd.com/read/28379475/are-the-1990-american-college-of-rheumatology-vasculitis-classification-criteria-still-valid
#17
Benjamin Seeliger, Jan Sznajd, Joanna C Robson, Andrew Judge, Anthea Craven, Peter C Grayson, Ravi S Suppiah, Richard A Watts, Peter A Merkel, Raashid A Luqmani
Objectives.: Advances in diagnostic techniques have led to better distinction between types of vasculitis, potentially affecting the utility of the 1990 ACR classification criteria for vasculitis. This study tested the performance of these criteria in a contemporary vasculitis cohort. Methods.: The Diagnosis and Classification in Vasculitis Study provided detailed clinical, serological, pathological and radiological data from patients with primary systemic vasculitis and clinical context-specific comparator conditions...
March 31, 2017: Rheumatology
https://www.readbyqxmd.com/read/28377416/spectrum-of-immune-related-conditions-associated-with-risk-of-keratinocyte-cancers-among-elderly-adults-in-the-united-states
#18
Elizabeth L Yanik, Ruth M Pfeiffer, D Michael Freedman, Martin A Weinstock, Elizabeth K Cahoon, Sarah T Arron, Matthew Chaloux, M Kari Connolly, Priyadharsini Nagarajan, Eric A Engels
Elevated keratinocyte carcinoma (KC) risk is present with several immune-related conditions, e.g., solid organ transplantation and non-Hodgkin lymphoma. Because many immune-related conditions are rare, their relationships with KC have not been studied. We used Medicare claims to identify cutaneous squamous cell carcinoma (SCC) and basal cell carcinoma (BCC) cases in 2012, and controls matched on sex and age. All subjects were aged 65-95 years, of white race, and had attended ≥1 dermatologist visit in 2010-2011...
April 4, 2017: Cancer Epidemiology, Biomarkers & Prevention
https://www.readbyqxmd.com/read/28371573/pachymeningitis-and-cerebral-granuloma-in-granulomatosis-with-polyangiitis-is-rituximab-a-promising-treatment-option
#19
Carlos Costa, Tânia Santiago, Joana Espirito-Santo, João Rovisco, Jorge Silva, Armando Malcata
Granulomatosis with polyangiitis (GPA) is a rare autoimmune disease characterized by granulomatous inflammation involving upper and lower respiratory tract, kidneys and peripheral nervous system. However, central nervous system involvement is uncommon and frequently refractory to classical therapy. Rituximab has emerged as promising alternative, but published reports are scarce. We report a case of pachymeningitis and cerebral granuloma in a patient with a history of severe generalized GPA, treated with rituximab...
January 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28365634/lesson-of-the-month-1-beware-the-atypical-presentation-eosinophilic-granulomatosis-with-polyangiitis-presenting-as-acute-coronary-syndrome
#20
Juergen Schiefermueller, Bashir Alaour, Alison Calver, Nick Curzen
We describe the case of a 45-year-old woman presenting with troponin positive cardiac-sounding chest pain. An initial emergency angiogram demonstrated two vessel coronary disease, including a distal right coronary artery occlusion. No percutaneous coronary intervention was performed and the patient was treated medically. At re-presentation with further pain a few days later, coronary angiography demonstrated no significant coronary lesions. After consideration of other multisystem symptoms and raised eosinophil count, the patient was diagnosed with eosinophilic granulomatosis with polyangiitis (formerly known as Churg-Strauss syndrome) presenting with coronary arteritis...
April 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
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