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granulomatosis with polyangiitis

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https://www.readbyqxmd.com/read/29778666/mycophenolate-mofetil-for-induction-and-maintenance-of-remission-in-na%C3%A3-ve-patients-with-granulomatosis-with-polyangiitis-without-renal-involvement
#1
Yasser Emad, Yasser Ragab, Johannes J Rasker
Antineutrophil cytoplasmic antibodies (ANCA) associated vasculitides include granulomatosis with polyangiitis (GPA, previously called Wegener's), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA), previously called Churg-Strauss). In this report we used mycophenolate mofetil (MMF) and steroids to induce and maintain remission in two newly diagnosed cases with c-ANCA associated GPA. The two patients' maintained remission with no disease relapses during one year follow-up...
May 16, 2018: Reumatología Clinica
https://www.readbyqxmd.com/read/29769093/granulomatosis-with-polyangiitis-a-17-year-experience-from-a-tertiary-care-hospital-in-pakistan
#2
Omar Irfan, Haris Khan, Zarrar Khan, Alina Ashraf, Rimsha Ahmed, Javaid Ahmad Khan, Ali Bin Sarwar Zubairi
OBJECTIVE: Granulomatosis with Polyangiitis (GPA) is an autoimmune, multi-system, small and medium vessel vasculitis with granulomatous inflammation. Aim of this study was to assess the clinical and radiological presentations of patients with GPA amongst the Pakistani population. It is a single centre retrospective single observation study. RESULTS: Study was conducted at the Aga Khan University Hospital, Karachi with records were reviewed from January 2000 to December 2017...
May 16, 2018: BMC Research Notes
https://www.readbyqxmd.com/read/29766394/treatment-of-eosinophilic-granulomatosis-with-polyangiitis-a-review
#3
Loïc Raffray, Loïc Guillevin
Eosinophilic granulomatosis with polyangiitis (formerly Churg-Strauss syndrome) is a rare type of anti-neutrophil cytoplasm antibody-associated vasculitis. Nevertheless, eosinophilic granulomatosis with polyangiitis stands apart because it has features of vasculitis and eosinophilic disorders that require targeted therapies somewhat different from those used for other anti-neutrophil cytoplasm antibody-associated vasculitides. Considerable advances have been made in understanding the underlying pathophysiology of eosinophilic granulomatosis with polyangiitis that have highlighted the key role of eosinophils and opened new therapeutic opportunities...
May 15, 2018: Drugs
https://www.readbyqxmd.com/read/29760156/improved-survival-with-renal-transplantation-for-end-stage-renal-disease-due-to-granulomatosis-with-polyangiitis-data-from-the-united-states-renal-data-system
#4
Zachary S Wallace, Rachel Wallwork, Yuqing Zhang, Na Lu, Frank Cortazar, John L Niles, Eliot Heher, John H Stone, Hyon K Choi
BACKGROUND: Renal transplantation is the optimal treatment for selected patients with end-stage renal disease (ESRD). However, the survival benefit of renal transplantation among patients with ESRD attributed to granulomatosis with polyangiitis (GPA) is unknown. METHODS: We identified patients from the United States Renal Data System with ESRD due to GPA (ESRD-GPA) between 1995 and 2014. We restricted our analysis to waitlisted subjects to evaluate the impact of transplantation on mortality...
May 14, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29755460/proteinase-3-interferes-with-c1q-mediated-clearance-of-apoptotic-cells
#5
Pascale Tacnet-Delorme, Julie Gabillet, Simon Chatfield, Nathalie Thieblemont, Philippe Frachet, Véronique Witko-Sarsat
Proteinase 3 (PR3) is the autoantigen in granulomatosis with polyangiitis, an autoimmune necrotizing vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs). Moreover, PR3 is a serine protease whose membrane expression can potentiate inflammatory diseases such as ANCA-associated vasculitis and rheumatoid arthritis. During apoptosis, PR3 is co-externalized with phosphatidylserine (PS) and is known to modulate the clearance of apoptotic cells through a calreticulin (CRT)-dependent mechanism...
2018: Frontiers in Immunology
https://www.readbyqxmd.com/read/29754328/hbsag-negative-and-anti-hbc-positive-in-eosinophilic-granulomatosis-with-polyangiitis-a-retrospective-pilot-study
#6
Sang-Won Lee, Do Young Kim, Sang Hoon Ahn, Yong-Beom Park, Kwang-Hyub Han, Jun Yong Park
We examined whether resolved hepatitis B virus (HBV) infection was associated with antineutrophil cytoplasmic antibody-associated vasculitis (AAV), and affected AAV activity at diagnosis and prognosis during the follow-up. We reviewed the electronic medical records of 153 AAV patients, and included 91 hepatitis B surface antigen (HBsAg)-negative patients having results of both antibody to hepatitis B core antigen (anti-HBc) and surface antigen (anti-HBs). We collected clinical and laboratory data, Birmingham vasculitis activity score (BVAS) and five factor scores (FFS) at diagnosis and relapse rates during the follow-up...
May 12, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29745876/the-presence-of-staphylococcal-superantigens-in-nasal-swabs-and-correlation-with-activity-of-granulomatosis-with-polyangiitis-in-own-material
#7
Justyna Fijołek, Elzbieta Wiatr, Violetta Petroniec, Ewa Augustynowicz-Kopec, Michal Bednarek, Dariusz Gawryluk, Magdalena Maria Martusewicz-Boros, Katarzyna Modrzewska, Elzbieta Radzikowska, Kazimerz Roszkowski-Sliz
OBJECTIVES: Nasal carriage of Staphylococcus aureus and its superantigens (SAg) seem to be a risk factor disease exacerbation in granulomatosis with polyangiitis (GPA). We investigated the association between the presence of SAg in nasal swabs and activity of disease in GPA patients also taking into account correlation with an antimicrobial treatment. METHODS: In a prospective study of a total of 150 GPA patients hospitalised in the period 2009-2016, nasal swabs were examined for the presence of Staphylococcus aureus and SAg...
May 4, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29741122/long-term-risk-and-outcome-of-infection-related-hospitalization-in-granulomatosis-with-polyangiitis-a-nationwide-population-based-cohort-study
#8
M Faurschou, N Obel, B Baslund
OBJECTIVE: To assess the long-term risk and outcome of infection-related hospitalization (IH) among patients with granulomatosis with polyangiitis (GPA). METHOD: We used administrative databases to establish a GPA cohort (n = 398), construct a comparison cohort of population controls (n = 3980), and collect clinical data. Cox regression analyses were used to determine hazard ratios (HRs) as a measure of relative risk. Follow-up began at date of GPA diagnosis and continued for up to 10 years...
May 9, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29735489/rise-and-fall-of-the-eosinophils-in-heart-failure-a-rare-but-important-phenomenon-seen-with-cardiomyopathy
#9
Danish Ali, David Snead, Vijay Anand Dhakshinamurthy, Prithwish Banerjee
A 65-year-old lady and a 69-year-old gentleman, both with a background history of adult-onset asthma, presented with clinical features of heart failure (HF). High-sensitivity cardiac troponin T and eosinophils were significantly raised, along with poor left ventricular (LV) systolic function on cardiac imaging. Endocardial and skin biopsy (in cases 1 and 2, respectively) showed eosinophilic infiltration. This in combination with the clinical features confirmed the diagnosis of eosinophilic myocarditis (EM) secondary to eosinophilic granulomatosis with polyangiitis in case 1...
May 7, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29734937/primary-skull-base-lymphoma-manifestations-and-clinical-outcomes-of-a-great-imitator
#10
John P Marinelli, Mara C Modzeski, John I Lane, Jamie J Van Gompel, Janalee K Stokken, Gita Thanarajasingam, Matthew L Carlson
Objectives Primary skull base lymphoma (PSBL) represents a rare manifestation of extranodal lymphoma. Presenting with nonspecific symptomatology and imaging findings, PSBL often masquerades as more common cranial base pathology and thus can present a diagnostic challenge. The objectives of this study were to characterize the manifestations and clinical outcomes of PSBL. Study Design Case series and chart review. Setting Tertiary referral center. Subjects and Methods Review of 48 patients with PSBL treated between 1994 and 2016...
May 1, 2018: Otolaryngology—Head and Neck Surgery
https://www.readbyqxmd.com/read/29730524/mortality-in-systemic-necrotizing-vasculitides-a-retrospective-analysis-of-the-french-vasculitis-study-group-registry
#11
REVIEW
Sabine Jardel, Xavier Puéchal, Alain Le Quellec, Christian Pagnoux, Mohamed Hamidou, François Maurier, Olivier Aumaitre, Achille Aouba, Thomas Quemeneur, Jean-François Subra, Vincent Cottin, Jean Sibilia, Pascal Godmer, Patrice Cacoub, Anne Laure Fauchais, Eric Hachulla, Delphine Maucort-Boulch, Loïc Guillevin, Jean-Christophe Lega
OBJECTIVE: The aim of the study was to describe the evolution of mortality and cause-specific mortality over time in patients with systemic necrotizing vasculitides (SNV), including polyarteritis nodosa (PAN), granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA). METHODS: Patients with SNV from the French Vasculitis Study Group registry were divided into 5 groups according to the date of diagnosis: <1980, 1980-1989, 1990-1999, 2000-2010, and ≥ 2010...
May 3, 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29724729/long-term-efficacy-of-remission-maintenance-regimens-for-anca-associated-vasculitides
#12
Benjamin Terrier, Christian Pagnoux, Élodie Perrodeau, Adexandre Karras, Chahera Khouatra, Olivier Aumaître, Pascal Cohen, Olivier Decaux, Hélène Desmurs-Clavel, François Maurier, Pierre Gobert, Thomas Quémeneur, Claire Blanchard-Delaunay, Bernard Bonnotte, Pierre-Louis Carron, Eric Daugas, Marize Ducret, Pascal Godmer, Mohamed Hamidou, Olivier Lidove, Nicolas Limal, Xavier Puéchal, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
OBJECTIVE: To compare long-term efficacy of remission-maintenance regimens in patients with newly diagnosed or relapsing antineutrophil cytoplasmic antibodies (ANCA)-associated vasculitides. METHODS: The 28-month Maintenance of Remission using Rituximab in Systemic ANCA-associated Vasculitis trial compared rituximab with azathioprine to maintain remission in patients with newly diagnosed or relapsing granulomatosis with polyangiitis, microscopic polyangiitis or renal-limited ANCA-associated vasculitis...
May 3, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29724306/-clinical-analysis-of-40-patients-with-eosinophilic-lung-diseases-in-peking-union-medical-college-hospital
#13
Xu Dong Zhang, Hai Hong Gong, Jin Ming Gao
Objective To summarize the clinical features of eosinophilic lung diseases(ELD). Methods We retrospectively analyzed the clinical manifestations,laboratory findings,accessory examination results,and pathology of 40 patients who were diagnosed with ELD and hospitalized in Peking Union Medical College Hospital from January 2013 to December 2016.Results There were 19 males and 21 females,and the average age was(48.58±18.25) years.The diagnoses included allergic bronchopulmonary aspergillosis(n=20),eosinophilic granulomatosis with polyangiitis(also known as churg-strauss syndrome)(n=10),chronic eosinophilic pneumonia(n=8),parasitic infection(n=1),and drug-induced eosinophilic pneumonia(n=1)...
April 28, 2018: Zhongguo Yi Xue Ke Xue Yuan Xue Bao. Acta Academiae Medicinae Sinicae
https://www.readbyqxmd.com/read/29722877/rituximab-for-induction-and-maintenance-therapy-of-granulomatosis-with-polyangiitis-a-single-centre-cohort-study-on-114-patients
#14
Xavier Puéchal, Michele Iudici, Ana Luisa Calich, Alexandre Vivot, Benjamin Terrier, Alexis Régent, Pascal Cohen, Claire Le Jeunne, Luc Mouthon, Philippe Ravaud, Loïc Guillevin
Objectives: To assess efficacy and safety of rituximab (RTX) induction and maintenance therapy for granulomatosis with polyangiitis (GPA) in a single-centre cohort study. Methods: All patients with active GPA, not enrolled in trials, who received ⩾1 RTX infusion(s) for induction were included. At remission, protocolized maintenance RTX infusions were given every 6 months for 18 months. Kaplan-Meier curves were used to estimate survival rates. Univariable analyses identified factors associated with remission failure and relapse, and Cox models retained independent predictors of relapse...
May 2, 2018: Rheumatology
https://www.readbyqxmd.com/read/29721696/pyoderma-gangrenosum-like-ulcerations-in-granulomatosis-with-polyangiitis-two-cases-and-literature-review
#15
REVIEW
Giovanni Genovese, Simona Tavecchio, Emilio Berti, Franco Rongioletti, Angelo Valerio Marzano
Granulomatosis with polyangiitis (GPA) is a systemic necrotizing small vessel vasculitis associated with circulating anti-neutrophil cytoplasmic antibodies (ANCAs). Skin manifestations, mostly represented by palpable purpura, papulonodular lesions and livedo reticularis, are present in up to 50% of the cases. Ulcerations with undermined, raised erythematous-violaceous border resembling pyoderma gangrenosum (PG) have rarely been reported as skin involvement in GPA. The presence of circulating ANCAs with a cytoplasmic labelling pattern, the involvement of internal organs, particularly of the lung, and the absence on histology of a mainly neutrophilic infiltrate in early phases of the cutaneous lesions may be regarded as clues to rule out true PG and confirm the diagnosis of GPA skin ulcerations simulating PG...
May 2, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29719830/a-92-year-old-male-with-eosinophilic-asthma-presenting-with-recurrent-palpable-purpuric-plaques
#16
Nicole A Negbenebor, Saami Khalifian, Ruth K Foreman, Daniela Kroshinsky
Churg-Strauss syndrome or eosinophilic granulomatosis with polyangiitis is a systemic vasculitis affecting the small and medium-sized vasculature. It is commonly associated with asthma and eosinophilia. Most patients are diagnosed at around the age of 40. We report a case of biopsy-confirmed Churg-Strauss syndrome in a 92-year-old male with a history of eosinophilic asthma and peripheral eosinophilia who was later diagnosed with Churg-Strauss syndrome.
January 2018: Dermatopathology (Basel, Switzerland)
https://www.readbyqxmd.com/read/29719652/simultaneous-unilateral-presentation-of-three-different-ocular-manifestations-of-granulomatosis-with-polyangiitis
#17
Ali Salehi, Farzan Kianersi, Heshmatollah Ghanbari, Zahra Dastborhan, Ebrahim Gerami, Elnaz Pirmoazzen
Purpose: To report the simultaneous presentation of three ocular manifestations of granulomatosis with polyangiitis in one eye. Case Report: A 42-year-old man with a confirmed diagnosis of granulomatosis with polyangiitis was referred to the emergency room with sudden blurred vision. Eye examination showed hyperemic conjunctiva due to necrotizing scleritis in the superior nasal quadrant of the left eye, a mass in the left superior lid, as well as central retinal artery occlusion in the same eye...
April 2018: Journal of Ophthalmic & Vision Research
https://www.readbyqxmd.com/read/29718465/proteinase-3-and-myeloperoxidase-serotype-in-relation-to-demographic-factors-and-geographic-distribution-in-anti-neutrophil-cytoplasmic-antibody-associated-glomerulonephritis
#18
Maria Weiner, Rune Bjørneklett, Zdenka Hrušková, Bruce Mackinnon, Caroline J Poulton, Leo Sindelar, Aladdin J Mohammad, Per Eriksson, Loreto Gesualdo, Duvuru Geetha, Matija Crnogorac, David Jayne, Susan L Hogan, Colin Geddes, Vladimir Tesar, Knut Aasarød, Mårten Segelmark
Background: In anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis, antigen specificity varies between myeloperoxidase (MPO) and proteinase 3 (PR3). This has been reported to vary in relation to age, gender, geography and extrarenal manifestations. However, studies are difficult to compare as criteria for inclusion vary. The aim of this study was to investigate the relationship between ANCA serotype, latitude, ultraviolet (UV) radiation levels, age, gender and renal function at diagnosis in a large study with uniform inclusion criteria...
April 30, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29716529/severe-localised-granulomatosis-with-polyangiitis-wegener-s-granulomatosis-manifesting-with-extensive-cranial-nerve-palsies-and-cranial-diabetes-insipidus-a-case-report-and-literature-review
#19
James E Peters, Vivek Gupta, Ibtisam T Saeed, Curtis Offiah, Ali S M Jawad
BACKGROUND: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a multisystem vasculitis of small- to medium-sized blood vessels. Cranial involvement can result in cranial nerve palsies and, rarely, pituitary infiltration. CASE PRESENTATION: We describe the case of a 32 year-old woman with limited but severe GPA manifesting as progressive cranial nerve palsies and pituitary dysfunction. Our patient initially presented with localised ENT involvement, but despite treatment with methotrexate, she deteriorated...
May 1, 2018: BMC Neurology
https://www.readbyqxmd.com/read/29709930/a-case-of-dilated-cardiomyopathy-with-eosinophilic-granulomatosis-with-polyangiitis-in-which-active-myocardial-inflammation-was-only-detected-by-endomyocardial-biopsy
#20
Takafumi Nakayama, Shunsuke Murai, Nobuyuki Ohte
Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare type of systemic vasculitis. Cardiac involvement is the main cause of death in patients with this disease. We herein report a case of congestive heart failure in a patient with EGPA. Neither 67 Ga scintigraphy nor cardiac magnetic resonance imaging detected inflammation of the myocardium; however, myocardial biopsy revealed numerous infiltrating inflammatory cells, thereby fulfilling the criteria of inflammatory dilated cardiomyopathy. We improved the LV systolic function by increasing the patient's prednisolone dosage...
April 27, 2018: Internal Medicine
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