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https://www.readbyqxmd.com/read/29786186/-ebv-associated-pneumonia-in-patient-with-granulomatosis-with-polyangiitis-gpa-in-immunosoppressive-therapy-treated-with-aciclovir
#1
Nicola Mongera, Vittorio Di Maso, Elisabetta Ermacora, Michele Carraro, Cristina Bregant, Martina Pian, Umberto Savi, Eric Lorenzon, Giuliano Boscutti
We describe the case of a 74-year-old man admitted to our Nephrology Unit with nephrotic syndrome and mild kidney disease. A complete panel of laboratoristic and instrumental tests did not provide useful information for diagnosis. No specific signs or symptoms suggested the presence of AL amyloidosis. As a matter of fact, diagnosis was reached thanks to the hystopathologic examination of renal tissue and bone marrow, since the associated B-cell lymphoproliferative disorder had not revealed itself through serum and urine electrophoresis and immunofixation...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29780942/successful-reconstruction-of-an-ocular-defect-resulting-from-granulomatosis-with-polyangiitis-following-treatment-with-rituximab
#2
Grace M Kenny, Konstanze Holl-Ulrich, Timothy Fulcher, Elizabeth McElnea, Eoin Kavanagh, Heather Moriarty, Niall Mulligan, Eamonn S Molloy, Geraldine M McCarthy
Purpose: To report a unique case of orbital inflammatory disease which was ultimately diagnosed as granulomatosis with polyangitis (GPA) and thus successfully treated. Observation: A 47 year-old man presented with a rapidly progressive necrotic soft tissue mass within the medial antero-superior aspect of the right eyelid and orbit. He also had transient retinal vasculitis in the left. Serology, histology and imaging were atypical of, but consistent with, GPA. He was thus successfully treated with intravenous rituximab followed by reconstruction of the medial eyelid...
June 2018: American Journal of Ophthalmology Case Reports
https://www.readbyqxmd.com/read/29780909/disseminated-silicone-granulomatosis-in-the-face-and-orbit
#3
Tiffany A Chen, Carmel L Mercado, Katie L Topping, Benjamin P Erickson, Kimberly P Cockerham, Andrea L Kossler
Purpose: To report a case of disseminated silicone granulomatosis presenting with ptosis, proptosis and vision loss. Observations: A 56-year-old female presented with ptosis, proptosis, and vision loss and was noted to have palpable, erythematous masses involving the orbit, face, trunk, and body. She had a history of bilateral silicone breast implants and cosmetic facial filler injections. Orbital biopsy demonstrated non-caseating granulomas with foreign-body giant cells and vacuoles containing material consistent with silicone...
June 2018: American Journal of Ophthalmology Case Reports
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
#4
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/29770266/renal-granulomatosis-post-intravesical-bacillus-calmette-guerin-therapy-for-non-muscle-invasive-bladder-cancer
#5
Karen Tran-Harding, Rashmi T Nair, Halemane Ganesh
Intravesical Bacillus Calmette-Guerin (BCG) immunotherapy is a proven, effective treatment for intermediate- and high-risk non-muscle-invasive bladder cancer. Minor side effects are common and expected but systemic effects can occur in <5% of treated patients. We present a rare case of a 49-year-old male that presented with fever and chills after 3 weeks of intravesical BCG therapy post transurethral resection of bladder tumor. New renal lesions were present on contrast-enhanced computed tomography scan which was histologically proven to be necrotizing renal granulomatosis...
2018: Journal of Clinical Imaging Science
https://www.readbyqxmd.com/read/29769093/granulomatosis-with-polyangiitis-a-17-year-experience-from-a-tertiary-care-hospital-in-pakistan
#6
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/29766816/a-systematic-review-of-the-literature-of-the-three-related-disease-entities-cheilitis-granulomatosa-orofacial-granulomatosis-and-melkersson-rosenthal-syndrome
#7
Goetz Wehl, Markus Rauchenzauner
BACKGROUND AND OBJECTIVE: Melkersson Rosenthal syndrome (MRS) is a rare disorder of unknown etiology and comprises the triad: orofacial edema, recurrent facial paralysis and lingua plicata. In the current literature confusing heterogeneity exists, mixing together the historically grown terms cheilitis granulomatosa or granulomatous cheilitis, Melkersson Rosenthal syndrome and the umbrella term orofacial granulomatosis (OFG). METHODS: We provide a systematic review comprising all three disease entities of orofacial granulomatosis using the computerized database "Pubmed Medline" entering the key words "orofacial granulomatosis" (141 references), "Melkersson-Rosenthal syndrome" (207 references), "granulomatous cheilitis" or "cheilitis granulomatosa" (102 references) back to 1956...
May 14, 2018: Current Pediatric Reviews
https://www.readbyqxmd.com/read/29766394/treatment-of-eosinophilic-granulomatosis-with-polyangiitis-a-review
#8
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
#9
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
#10
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
#11
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
#12
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/29744913/cartilage-hair-hypoplasia-with-cutaneous-lymphomatoid-granulomatosis
#13
D Sathishkumar, J E Gach, M Ogboli, M Desai, T Cole, W Högler, J Motwani, A Norton, B Morland, I Colmenero
Cartilage-hair hypoplasia (CHH) is an autosomal recessive chondrodysplasia characterized by short-stature, sparse hair and impaired cellular immunity. We describe a young girl who was diagnosed with CHH based on the findings of recurrent infections, short stature with metaphyseal chondrodysplasia, and a confirmed bi-allelic RMRP gene mutation. At 13 years, the patient developed an Epstein-Barr virus (EBV)-driven lymphoproliferative disorder involving the lung, which responded partially to chemotherapy. Simultaneously, she developed multiple indurated plaques involving her face, which had histological findings of granulomatous inflammation and EBV-associated low-grade lymphomatoid granulomatosis...
May 10, 2018: Clinical and Experimental Dermatology
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
#14
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/29739366/angiocentric-lymph-proliferative-disorder-lymphomatoid-granulomatosis-in-a-person-with-newly-diagnosed-hiv-infection-a-case-report
#15
Cecilia T Costiniuk, Jason Karamchandani, Ali Bessissow, Jean-Pierre Routy, Jason Szabo, Charles Frenette
BACKGROUND: Angiocentric lymph proliferative disorder (ALPD) is a granulomatous lymphoproliferative condition associated with various primary and secondary immunodeficiency states. ALPD is so rare that its prevalence has not been established. Typically affecting middle-aged adults, this condition is often found in the context of Epstein Bar Virus infection and consists of angiocentric and angioinvasive pulmonary infiltrates. Herein, we present a biopsy-proven case of a patient manifesting with a viral meningoencephalomyelitis-like picture with brain, spinal cord, renal and splenic lesions...
May 8, 2018: BMC Infectious Diseases
https://www.readbyqxmd.com/read/29735489/rise-and-fall-of-the-eosinophils-in-heart-failure-a-rare-but-important-phenomenon-seen-with-cardiomyopathy
#16
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
#17
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
#18
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/29725643/combination-therapy-with-rituximab-and-cyclophosphamide-for-remission-induction-in-anca-vasculitis
#19
Frank B Cortazar, Saif A Muhsin, William F Pendergraft, Zachary S Wallace, Colleen Dunbar, Karen Laliberte, John L Niles
Introduction: Remission induction in antineutrophil cytoplasmic autoantibody (ANCA) vasculitis may be complicated by slow response to treatment and toxicity from glucocorticoids. We describe outcomes with a novel remission induction regimen combining rituximab with a short course of low-dose, oral cyclophosphamide and an accelerated prednisone taper. Methods: Patients were included in this retrospective study if they had newly diagnosed or relapsing ANCA vasculitis with a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS-WG) ≥3 and received a standardized remission induction regimen...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29724729/long-term-efficacy-of-remission-maintenance-regimens-for-anca-associated-vasculitides
#20
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
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