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https://www.readbyqxmd.com/read/28319866/contrast-enhanced-ultrasound-and-computed-tomography-findings-of-granulomatosis-with-polyangiitis-presenting-with-multiple-intrarenal-microaneurysms-a-case-report
#1
Youe Ree Kim, Young Hwan Lee, Jong-Ho Lee, Kwon-Ha Yoon
Granulomatosis with polyangiitis (GPA) is a systemic disorder that affects small- and medium- sized vessels in many organs. Although the kidneys are the second most commonly involved organ in patients with GPA, its manifestation as multiple intrarenal aneurysms is rare. We report an unusual manifestation of GPA with multiple intrarenal microaneurysms, as demonstrated by contrast-enhanced ultrasound and computed tomography.
March 9, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/28318198/-subglottic-stenosis-as-the-initial-manifestation-of-wegener-s-granulomatosis-in-a-teenager-case-report
#2
Giselle Cuestas, Verónica Rodríguez, Flavia Doormann, Alejandra Pringe, Patricio Bellia Munzón, Gastón Bellia Munzón, Carlos Ortega, Rubén Álvarez
Wegener's granulomatosis is a necrotizing granulomatous vasculitis of autoimmune origin that primarily affects the upper and lower airways and kidneys. It is very rare in children and adolescents. When started at a young age it is often associated with subglottic stenosis. Subglottic stenosis is a potentially fatal manifestation of Wegener's granulomatosis. Its diagnosis requires a high index of suspicion since it might develop in the absence of other signs of activity. Occasionally, subglottic stenosis may present as the initial manifestation of the disease...
April 1, 2017: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/28306595/update-on-maintenance-therapy-for-granulomatosis-with-polyangiitis-and-microscopic-polyangiitis
#3
Ora Singer, W Joseph McCune
PURPOSE OF REVIEW: The antineutrophilic cytoplasmic antibody (ANCA)-associated vasculitides are a group of rare systemic diseases. The past several years have seen major therapeutic advances in the treatment of granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). The success rate in induction of remission is high, but reducing the high incidence of relapses remains a therapeutic challenge. RECENT FINDINGS: Studies have shown no improvement in relapse rates in GPA and MPA over the past 2 decades...
March 16, 2017: Current Opinion in Rheumatology
https://www.readbyqxmd.com/read/28306365/ulcerative-granuloma-of-the-eyelid-as-the-initial-manifestation-of-granulomatosis-with-polyangiitis-wegener-s-granulomatosis-a-case-report
#4
Helena Brosa Morros, Olaia Subirà, Montse Gomà Gàllego, Maria José Paúles Villar, Ferran Mascaró Zamora, Maravillas Abia Serrano
A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Radical surgical excision and total tarsorrhaphy were performed. Following treatment, the patient was asymptomatic for 6 weeks until he developed acute renal failure, generalised arthralgia, acute hypertensive anterior uveitis, and dacryoadenitis...
March 17, 2017: Orbit
https://www.readbyqxmd.com/read/28303064/accessory-left-gastric-artery-aneurysms-in-granulomatosis-with-polyangiitis-a-case-report-and-literature-review
#5
Toshihide Tomosugi, Takuji Takahashi, Yoshihisa Kawase, Koichi Yoshida, Shogo Hayashi, Takefumi Sugiyama, Mitsuya Shimizu, Michita Shoka, Kohichi Sawaki, Eiji Onishi, Naomi Hayashi, Hidenobu Matsushita, Osamu Okochi
Aneurysm formation is a potential complication of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. It is a very rare complication, but immediate diagnosis and therapy should be performed because an aneurysm can be life-threatening if it ruptures. An accessory left gastric artery (ALGA) is also a rare variant gastric artery that may obtain its blood supply from the left hepatic artery and left gastric artery. We herein describe a 57-year-old Japanese man who was diagnosed with GPA complicated by aneurysm rupture in an ALGA...
February 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28297701/relationship-between-granulomatosis-and-cancer
#6
Laurent Pierre Nicod
No abstract text is available yet for this article.
March 16, 2017: Respiration; International Review of Thoracic Diseases
https://www.readbyqxmd.com/read/28295528/serum-soluble-interleukin-2-receptor-level-is-more-sensitive-than-angiotensin-converting-enzyme-or-lysozyme-for-diagnosis-of-sarcoidosis-and-may-be-a-marker-of-multiple-organ-involvement
#7
Chuyen Thi Hong Nguyen, Naotomo Kambe, Izumi Kishimoto, Ikuko Ueda-Hayakawa, Hiroyuki Okamoto
Skin lesions in sarcoidosis are often the initial symptoms that enable the dermatologist to be the first to diagnose this granulomatosis. However, diagnosis is sometimes very problematic. In 2015, the diagnostic criteria for sarcoidosis were updated in Japan, with elevated serum soluble interleukin-2 receptor (sIL-2R) replacing negative tuberculin reaction. Therefore, we assessed the clinical utility of sIL-2R compared with two other common markers, angiotensin-converting enzyme (ACE) and lysozyme, in patients who visited the dermatology clinic...
March 11, 2017: Journal of Dermatology
https://www.readbyqxmd.com/read/28295009/-refractory-form-of-orbital-granulomatosis-with-polyangiitis-a-clinical-and-morphological-study
#8
E A Kogan, Ya O Grusha, D S Ismailova, P I Novikov, Yu V Abramova, A D Meshkov, E F Rizopulu
Orbital granulomatosis with polyangiitis (Wegener's granulomatosis, GPA), which is characterized by granulomatous inflammation with small-vessel vasculitis, can develop in local and generalized forms of the disease. The introduction of current immunosuppressive therapy regimens has improved the prognosis of the disease; however, there are immunosuppressive treatment-refractory forms of GPA, the morphology of which has been inadequately investigated. The paper describes a clinical case of refractory GPA involving the orbit, as evidenced by histological and immunohistochemical examinations...
2017: Arkhiv Patologii
https://www.readbyqxmd.com/read/28294494/granulomatosis-with-polyangiitis-an-unusual-intestinal-presentation
#9
Eugenio Morandi, Carlo Corbellini, Marco Castoldi
No abstract text is available yet for this article.
March 15, 2017: ANZ Journal of Surgery
https://www.readbyqxmd.com/read/28293550/dock-8-deficiency-ebv-lymphomatoid-granulomatosis-and-intrafamilial-variation-in-presentation
#10
Victoria R Dimitriades, Vincent Devlin, Stefania Pittaluga, Helen C Su, Steven M Holland, Wyndham Wilson, Kieron Dunleavy, Nirali N Shah, Alexandra F Freeman
Dedicator of cytokinesis 8 (DOCK8) deficiency is an autosomal recessive, combined immunodeficiency within the spectrum of hyper-IgE syndromes. Epstein-Barr virus-positive lymphomatoid granulomatosis (LYG) (EBV + LYG) is a rare diagnosis and a previously unreported presentation of DOCK8 deficiency. A 10-year-old girl was initially evaluated for mild eczema and recurrent sinopulmonary infections. She had normal immunoglobulins with elevated IgE, poor polysaccharide response with low switched memory B cells, low CD4 count, and normal mitogen and antigen responses...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28293459/refractory-optic-perineuritis-due-to-granulomatosis-with-polyangiitis-successfully-treated-with-methotrexate-and-mycophenolate-mofetil-combination-therapy
#11
Yoshitaka Kimura, Kurumi Asako, Hirotoshi Kikuchi, Hajime Kono
Optic perineuritis is an uncommon inflammatory disorder of the optic sheath that causes visual loss or eye pain. There are few case reports of optic perineuritis associated with granulomatosis with polyangiitis. Herein we report the case of a 37-year-old male with granulomatosis with polyangiitis and who presented with headache, blurred vision in the right eye, diplopia, and numbness in the right forehead. Brain magnetic resonance images (MRI) findings revealed hypertrophic pachymeningitis and refractory optic perineuritis...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28284348/chronic-lymphocytic-inflammation-with-pontine-perivascular-enhancement-responsive-to-steroids-clippers-a-lymphocytic-reactive-response-of-the-central-nervous-system-a-case-report
#12
Xiaolin Wang, Dehui Huang, Xusheng Huang, Jiatang Zhang, Ye Ran, Xin Lou, Qiuping Gui, Shengyuan Yu
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroid (CLIPPERS) was first described in 2010. The characteristic clinical picture, radiological distribution and steroid response have been well-described in previous reports. However, the underlying pathogenesis and nosological position of CLIPPERS in the CNS require further investigation for the primary CNS lymphoma have been identified by autopsy subsequently. Here, we report a 51-year-old woman who was diagnosed with CLIPPERS but progressed to primary CNS lymphomatoid granulomatosis, which supports that CLIPPERS is not just an inflammatory CNS disorder...
April 15, 2017: Journal of Neuroimmunology
https://www.readbyqxmd.com/read/28281454/immune-stimulatory-effects-of-neutrophil-extracellular-traps-in-granulomatosis-with-polyangiitis
#13
Christina Lange, Elena Csernok, Frank Moosig, Julia U Holle
OBJECTIVES: The aim of this study was to analyse the role of netting neutrophils in the pathogenesis of granulomatosis with polyangiitis (GPA), especially their interplay with peripheral blood mononuclear cells (PBMCs). METHODS: The amount of cell-free DNA (cfDNA) was determined in sera from GPA patients (pairs active/inactive state of disease, n=18) and from healthy controls (HCs, n=10). Furthermore, we performed in vitro incubation experiments using PBMCs and NETs from patients and HCs for accessing the effect of NETs on PBMC behaviour...
March 8, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28281453/iga-antibodies-to-myeloperoxidase-in-patients-with-eosinophilic-granulomatosis-with-polyangiitis-churg-strauss
#14
Esha Oommen, Amber Hummel, Lisa Allmannsberger, David Cuthbertson, Carette Simon, Christian Pagnoux, Gary S Hoffman, Dieter E Jenne, Nader A Khalidi, Curry L Koening, Carol A Langford, Carol A McAlear, Larry Moreland, Philip Seo, Antoine Sreih, Steven R Ytterberg, Peter A Merkel, Ulrich Specks, Paul A Monach
OBJECTIVES: To determine the prevalence of anti-myeloperoxidase (MPO) antibodies of IgA (IgA anti-MPO) isotype in patients with eosinophilic granulomatosis with polyangiitis (EGPA), and the association of the IgA antibodies with IgG anti-MPO and with disease activity. METHODS: Serum samples from patients with EGPA followed in a multi-center longitudinal cohort were tested by ELISA for the presence of IgA anti-MPO and IgG anti-MPO antibodies. Sera from 87 healthy controls were used to define a positive test...
March 1, 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28277781/coligranuloma-hj%C3%A3-rre-and-wramby-s-disease-reconsidered
#15
W J M Landman, J H H van Eck
Coligranulomatosis (Hjärre and Wramby's disease) is considered to be a disease of chickens, turkeys and partridges that occurs sporadically in individual, adult birds. Therefore, the condition is not of economic importance, but is of interest due to the similarity of its lesions to those of tuberculosis. In a number of cases the disease could be reproduced by inoculation via artificial routes of granuloma homogenate or Escherichia coli bacteria isolated from the lesions. Oral inoculations always failed. Occasionally, also serious outbreaks of granuloma disease have been reported in chickens, turkeys and quails...
February 9, 2017: Avian Pathology: Journal of the W.V.P.A
https://www.readbyqxmd.com/read/28273992/anca-associated-mononeuritis-multiplex-with-overlap-in-vasculitic-syndromes
#16
Ravi Anadure, Coimbatore Narayanan, Govindraj Varadraj, Bevinahalli Nandeesh
Mononeuritis multiplex is a common manifestation of many illnesses which includes Hansen's disease and certain types of systemic vasculitis. The Antineutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis (AAV) is a group of rare diseases which show typical characteristic inflammatory cell infiltration and blood vessel wall necrosis. AAV syndromes include Granulomatosis with Polyangiitis (GPA), Microscopic Polyangiitis (MPA) and Eosinophilic Granulomatosis with Polyangiitis (EGPA). We describe a patient who presented with mononeuritis multiplex and had features of overlap between EGPA and MPA...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28273603/rare-presentation-of-wegener-s-granulomatosis-in-the-pituitary-gland-case-report-and-literature-review
#17
Samantha M Baird, Upasna Pratap, Catriona McLean, Candice P Law, Nicholas Maartens
INTRODUCTION: Wegener's granulomatosis (WG) is a systemic vasculitis that can affect a variety of organs including ear, nose and throat, lungs and kidneys. However WG is unusual in the pituitary and rare in the central nervous system. PRESENTATION OF CASE: A 56-year-old male with likely WG presented with polyuria and polydipsia despite six months of conservative medical management. MRI scanning revealed an enlarging heterogeneously enhancing pituitary gland. Following endoscopic transsphenoidal pituitary biopsy and debulking, final tissue pathology was diagnostic for WG in the pituitary gland...
February 20, 2017: International Journal of Surgery Case Reports
https://www.readbyqxmd.com/read/28272203/sarcoidosis-associated-with-infliximab-therapy-in-ulcerative-colitis-a-case-report
#18
Georgiana-Emmanuela Gîlcă, Smaranda Diaconescu, Gheorghe Gh Bălan, Oana Timofte, Gabriela Ştefănescu
RATIONALE: Although immunomodulatory therapy has been clearly stated as an important landmark in treatment of ulcerative colitis, significantly improving the quality of life for patients with inflammatory bowel disease, there are several aspects to be considered regarding the possible side-effects of anti-TNF alpha agents. In spite of a good safety profile, biologic TNF antagonists may induce paradoxical inflammation, which can manifest as sarcoid-like granulomatosis, consisting of noncaseating granulomas in the affected organs...
March 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28265531/a-cardiac-graft-from-a-donor-with-granulomatosis-with-polyangiitis-a-case-report
#19
Katharina Huenges, Bernd Panholzer, Jochen Cremer, Assad Haneya
Organ shortage unavoidably leads to shifting strategies in modern transplantation medicine. Experiences with specific comorbidities in terms of organ transplantation therefore have to be made. We report a case of a 51-year-old male patient with successful orthotopic heart transplantation from a donor with granulomatosis with polyangiitis. After a good recovery, the patient was discharged to rehabilitation 2 months after transplantation.
January 2017: Thoracic and Cardiovascular Surgeon Reports
https://www.readbyqxmd.com/read/28264210/-granulomatosis-with-polyangiitis-of-the-major-salivary-glands
#20
Mustafa Kurucay, Jan Ole Werner, Hans Bösmüller, Sorin Dumitru Ioanoviciu, Joerg Henes, Marius Horger
No abstract text is available yet for this article.
March 2017: RöFo: Fortschritte Auf Dem Gebiete der Röntgenstrahlen und der Nuklearmedizin
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