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https://www.readbyqxmd.com/read/29780942/successful-reconstruction-of-an-ocular-defect-resulting-from-granulomatosis-with-polyangiitis-following-treatment-with-rituximab
#1
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/29611207/immunological-and-hematological-effects-of-il-5-r%C3%AE-targeted-therapy-an-overview
#2
REVIEW
M Hassani, L Koenderman
IL-5 is an important cytokine for priming and survival of mature eosinophils, and for proliferation and maturation of their progenitors. Hence, IL-5(Rα) targeting will be increasingly used in diseases where eosinophils are the key immune effector cells such as eosinophilic asthma (EA), hyper eosinophilic syndrome (HES), eosinophilic esophagitis (EE) and eosinophilic granulomatosis with polyangitis (EGPA). Therefore, several neutralizing monoclonal antibodies directed against IL-5 (mepolizumab and reslizumab) and its receptor IL-5Rα (benralizumab) have found or will find their way to the clinic...
April 3, 2018: Allergy
https://www.readbyqxmd.com/read/29582832/progressive-outer-retinal-necrosis-after-rituximab-and-cyclophosphamide-therapy
#3
Mohit Dogra, Priya Bajgai, Ashok Kumar, Aman Sharma
We report a case of progressive outer retinal necrosis (PORN) in a patient of microscopic polyangitis (MPA), being treated with immunosuppressive drugs such as cyclophosphamide and rituximab. Her aqueous tap was positive for Varicella Zoster virus and she was treated with oral and intravitreal antivirals, along with discontinuation of one of the immunosuppressive agents, i.e. rituximab, which might have led to reactivation of the virus causing necrotizing retinitis lesions. Rituximab and cyclophosphamide are extremely potent drugs, which are necessary to manage immunological disorders such as MPA...
April 2018: Indian Journal of Ophthalmology
https://www.readbyqxmd.com/read/29392845/recurrence-of-eosinophilic-granulomatosis-with-polyangitis-after-orthotopic-heart-transplant
#4
Ujjwal Rastogi, Ziad Sergie, Sean Pinney, Noah Moss
Eosinophilic granulomatosis with polyangitis (EGPA), previously referred to as Churg-Strauss syndrome, is a necrotizing small vessel vasculitis associated with eosinophilic infiltrates and extravascular granulomas. We report a case of a Caucasian woman successfully bridged to heart transplantation with a continuous flow left ventricular assist device (LVAD) who survived recurrence of EGPA in the allograft.
February 2, 2018: American Journal of Transplantation
https://www.readbyqxmd.com/read/29344320/pyoderma-gangrenosum-associated-granulomatosis-with-polyangitis-a-case-report-and-literature-review
#5
Matthew B Zarraga, Nicole Swenson, Brad Glick
Granulomatosis with polyangitis, formerly known as Wegener's granulomatosis, is a multi-system vasculitis that has a variable clinical presentation. Although uncommon, cutaneous symptoms can be the initial presenting symptom of granulomatosis with polyangitis. We present an unusual case of pyoderma gangrenosum followed by a diagnosis of granulomatosis with polyangitis. We also provide a review of current literature on therapeutic options.
October 2017: Journal of Clinical and Aesthetic Dermatology
https://www.readbyqxmd.com/read/29219653/characteristics-and-outcome-of-intractable-vasculitis-syndrome-in-children-nation-wide-survey-in-japan
#6
Naoko Nakano, Masaaki Mori, Hiroaki Umebayashi, Naomi Iwata, Norimoto Kobayashi, Kenji Masunaga, Tomoyuki Imagawa, Takuji Murata, Noriko Kinjo, Kazushige Nagai, Mari Miyoshi, Syuji Takei, Shumpei Yokota, Eiichi Ishii
OBJECTIVE: Primary systemic vasculitis (PSV) is a rare disorder in children and difficult to distinguish from other diseases. However, appropriate diagnosis and prompt treatment will affect on the morbidity and mortality of intractable PSV. In this study, we conducted a nationwide survey in Japan, to clarify epidemiology and clinical outcome of PSV. METHODS: We had sent survey questionnaires to most of the Japanese institutions that employed pediatricians, requesting the number of patients with refractory PSV who were diagnosed and treated between 2007 and 2011...
December 8, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28619741/saddle-nose-and-bilateral-cauliflower-ear-deformities-with-pyoderma-gangrenosum-like-ulcers-cavitary-pulmonary-lesions-digital-gangrene-and-pulselessness-in-a-young-female
#7
Sweta Subhadarshani, Vishal Gupta, Anurag Chahal, Kaushal K Verma
We report a young female who presented with saddle-nose and bilateral cauliflower ear deformities along with pyoderma gangrenosum-like ulcers, digital gangrene and pulselessness. Subsequently, she was found to have bilateral conductive hearing loss, a corneal opacity, mild aortic regurgitation and radiological evidence of cavitary changes in lungs and aortoarteritis. Our patient had a constellation of symptoms which posed a diagnostic challenge. Finally, a diagnosis of relapsing polychondritis with several unusual features was made...
June 15, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28393729/granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#8
Nazan Şen, Müge Aydın Tufan, Reyhan Yıldız, Emine Duygu Ersözlü Bozkırlı, Eftal Yücel
Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72...
September 2016: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28366156/-granulomatous-polyangitis-wegener-granulomatosis-clinical-findings-and-results-of-long-term-follow-up
#9
Nazan Şen, Müge Aydın Tufan, Reyhan Yıldız, Emine Duygu Ersözlü Bozkırlı, Eftal Yücel
Introduction: To evaluate long-term outcome of patients with granulomatous polyangitis (GPA) followed up in a tertiary university hospital. Patients and Methods: We reviewed medical records of 22 patients with GPA diagnosis confirmed by tissue biopsies between 2004 and 2014. Result: The mean time from the onset of symptoms to diagnosis was 7.8 ± 12.3 months [interquartile range (IR)= 4.0]. The most commonly involved organs were the upper respiratory tract (URT) (72...
September 2016: Tüberküloz Ve Toraks
https://www.readbyqxmd.com/read/28260778/granulomatosis-with-polyangiitis-wegener-granulomatosis-with-unusual-presentation
#10
M A Mannan, K Pasha, S Nahar, K Begum
We came across a 32 years old male admitted in our hospital with prolonged low-grade fever, haemoptysis, leg swelling, weight loss, purpuric rashes and malaena. He received anti-TB treatment at another hospital without any improvement. He was pale with bullous and purpuric lesions over legs and feet. He also had features of consolidation over both lung fields. His CRP was 312mg/L, Urine R/M/E showed 40-50 RBC/HPF, Chest X-ray showed features of bilateral consolidation, c-ANCA-10U/L (positive); Tracheal aspirate for AFB was found to be negative...
January 2017: Mymensingh Medical Journal: MMJ
https://www.readbyqxmd.com/read/27872542/pulmonary-lymphomatoid-granulomatosis-a-case-report-with-review-of-literature
#11
Grover Ankita, Dhawan Shashi
Lymphomatoid granulomatosis is a rare, Epstein Barr Virus (EBV)-associated systemic angiodestructive disorder that may progress to a diffuse large B cell lymphoma. Pulmonary involvement occurs in over 90 % cases followed by kidney, skin and brain. WHO classifies lymphomatoid granulomatosis under the generic heading of B cell proliferations of uncertain malignant potential. Radiologically, pulmonary lymphomatoid granulomatosis (PLG) presents with non specific findings making histopathology the gold standard for diagnosis...
December 2016: Indian Journal of Surgical Oncology
https://www.readbyqxmd.com/read/27728668/venous-sinus-thrombosis-and-penile-granuloma-two-rare-manifestations-in-a-case-of-granulomatosis-with-polyangitis
#12
A Gandhi, R B Kulkarni
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27728634/microscopic-polyangitis-as-pulmonary-renal-synrome
#13
R S Tonk, Shafeeque Rahman, C P Mulaktar, S K Mahto
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/27708542/atypical-arteritis-in-internal-carotid-arteries-a-novel-concept-of-isolated-internal-carotid-arteritis
#14
Kazuki Fukuma, Hisanori Kowa, Hiroyuki Nakayasu, Kenji Nakashima
We presented a 38-year-old woman suffering from acute cerebral infarction due to arteritis limited to bilateral internal carotid arteries without a condition of giant cell arteritis or granulomatosis with polyangitis. Our case is unprecedented and characterized by a young woman with wall enhancement in the internal carotid arteries on contrast-enhanced magnetic resonance imaging (MRI), therapeutic effects of steroids, and positive status for human leucocyte antigen-B39, -B51 and -DR4. These disease characteristics were not in accordance with existing diagnostic criteria of vasculitis, such as Takayasu's arteritis, giant cell arteritis, granulomatosis with polyangiitis, and Behcet's disease...
September 2016: Yonago Acta Medica
https://www.readbyqxmd.com/read/27682495/-solicosis-with-one-micorscopic-polyangitis-case-complicated
#15
L L Song, J H Yu
No abstract text is available yet for this article.
July 20, 2016: Chinese Journal of Industrial Hygiene and Occupational Diseases
https://www.readbyqxmd.com/read/27671887/management-scheme-for-cerebral-wegener-granulomatosis-an-unusual-pseudotumoral-skull-base-pathology
#16
Anne Laure Bernat, Etienne Lefevre, Damien Sène, Philippe Herman, Homa Adle Biassette, Sébastien Froelich
Granulomatosis with polyangiitis (Wegener disease) is a rare antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis that seldom involves brain and meninges. We present a case of a 35-year-old woman with granulomatosis with polyangiitis involving the brain and the meninges. The histologic diagnosis required a complete resection of the lesion. Despite the possibility of postoperative wound infection, surgery was mandatory to ascertain the diagnostic and to treat locally.
December 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27651195/-clinical-characteristics-and-etiology-of-children-with-hypereosinophilia
#17
Chen Xiaohong, X U Yiping, L U Meiping
OBJECTIVE: To analyze the clinical characteristics and etiology of hypereosinophilia in children. METHODS: Clinical data of 88 children with hypereosinophilia admitted in Children's Hospital of Zhejiang University School of Medicine during April 2009 and May 2015 were retrospectively reviewed. The clinical manifestations, etiologies, and the correlation of disease severity with different etiologies were analyzed. RESULTS: The main clinical manifestations were fever, abdominal pain, cough and/or tachypnea, skin rash, hemafecia and diarrhea, which were observed in 19 (21...
May 25, 2016: Zhejiang da Xue Xue Bao. Yi Xue Ban, Journal of Zhejiang University. Medical Sciences
https://www.readbyqxmd.com/read/27606476/chronic-skin-lichenification-as-unusual-presentation-of-eosinophilic-granulomatosis-with-polyangitis-case-report-and-literature-review
#18
REVIEW
F Sbrana, B Loggini, S Galimberti, M Coceani, M Latorre, V Seccia, S L'Abbate, M Mosca, E M Pasanisi, C Baldini
Eosinophilic granulomatosis with polyangitis (EGPA) is an uncommon ANCA-associated systemic small-vessel necrotizing vasculitis. At times, EGPA presenting manifestations can be very different from the usually recognized disease patterns. We report a 52-year-old female patient with 3 years history of itching. During the time occurred a chronic skin lichenification on her legs and gradually developed a full-blown ANCA-MPO positive EGPA in combination with blood hypereosinophilia, eosinophilic vasculitis at skin biopsy, subclinical asthma and chronic rhinosinusitis...
April 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/27591039/anca-negative-eosinophilic-granulomatosis-with-polyangitis-egpa-manifesting-as-a-large-intracardiac-thrombus-and-glomerulonephritis-with-angionecrosis
#19
Yuichi Saito, Sho Okada, Nobusada Funabashi, Yoshio Kobayashi
A 59-year-old woman with a history of bronchial asthma presented with a prolonged fever and eosinophilia. There was transient proteinuria and troponin level was elevated. Antineutrophil cytoplasmic antibody was negative and she did not fulfil criteria for eosinophilic granulomatosis with polyangitis (EGPA). Echocardiography showed a large apical mass in the left ventricle, but there was no systolic dysfunction, local asynergy or ventricular remodelling. On MRI, apical mass was compatible with a thrombus and endocardial region was diffusely damaged...
September 2, 2016: BMJ Case Reports
https://www.readbyqxmd.com/read/27585535/-eosinophilic-granulomatosis-with-polyangitis-churg-strauss-and-severe-pericardial-effusion
#20
Margarida Pujol-López, Sergio Prieto-González, Susanna Prat-González, José Hernández-Rodríguez
No abstract text is available yet for this article.
August 29, 2016: Medicina Clínica
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