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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
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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.
September 23, 2016: World Neurosurgery
https://www.readbyqxmd.com/read/27651195/-clinical-characteristics-and-etiology-of-children-with-hypereosinophilia
#11
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
#12
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
#13
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
#14
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
https://www.readbyqxmd.com/read/27504336/ecmo-rescue-therapy-in-diffuse-alveolar-haemorrhage-a-case-report-with-review-of-literature
#15
Gautam Rawal, Raj Kumar, Sankalp Yadav
Extracorporeal Membrane Oxygenation (ECMO) has evolved as a treatment option for patients having potentially reversible severe respiratory failure who are deteriorating on conventional ventilation. During ECMO, systemic anticoagulation is needed to maintain patency of the circuit. Therefore, ongoing haemorrhage remains a relative contra-indication to ECMO as it can further increase the bleeding. There is only limited evidence available for the use of ECMO in patients with alveolar haemorrhage. Most of these patients did not receive any anticoagulation during ECMO...
June 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/27319977/cutaneous-metaplastic-synovial-cyst-case-report-and-literature-review-from-the-dermatological-point-of-view
#16
Masahiro Fukuyama, Yohei Sato, Jun Hayakawa, Manabu Ohyama
Cutaneous metaplastic synovial cysts (CMSCs) are rare tumors typically comprising a solitary, well-circumscribed cystic mass that is not connected to the joint. Synovial cysts have been reported predominantly by orthopedists or pathologists; however, the presence of CMSC is not generally well recognized by dermatologists. Herein, we report a CMSC in a 68-year-old woman receiving systemic corticosteroid therapy for the treatment of eosinophilic granulomatosis with polyangiitis (EGPA). We attempt to delineate the clinical characteristics of this unusual neoplasm by reviewing the literature, focusing especially on dermatological descriptions...
March 25, 2017: Keio Journal of Medicine
https://www.readbyqxmd.com/read/27274829/familial-vasculitides-granulomatosis-with-polyangitis-and-microscopic-polyangitis-in-two-brothers-with-differing-anti-neutrophil-cytoplasm-antibody-specificity
#17
Maria Prendecki, Tom Cairns, Charles D Pusey
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitis (AAV) is a group of rare autoimmune diseases. Although the aetiology of AAV is uncertain, it is likely that genetic and environmental factors contribute. We report the unusual case of two brothers presenting with AAV with differing clinical pictures and differing ANCA specificity. There is a recently identified difference in genetic risk factors associated with ANCA specificity, making it surprising that first-degree relatives develop AAV with differing clinical and serological features...
June 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27155202/chronic-skin-lichenification-as-unusual-presentation-of-eosinophilic-granulomatosis-with-polyangitis-case-report-and-literature-review
#18
Francesco Sbrana, Barbara Loggini, Sara Galimberti, Michele Coceani, Marta Latorre, Veronica Seccia, Sara L'Abbate, Marta Mosca, Emilio Maria Pasanisi, Chiara 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...
January 12, 2016: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/27075809/a-strange-oculomotor-palsy-with-eosinophilia
#19
Halil Yildiz, Ismaïl Ould-Nana, Julie Lelotte, Thierry Duprez, Bernard Vandercam, Michel Lambert, Chantal Lefebvre
A 75-year-old man was admitted to the Department of internal medicine because of a 2-month history of neurological deterioration. During the previous year, he complained of recurrent sinusitis, asthma, arthralgias, myalgias and asthenia. Later on, an oculomotor palsy, weakness and disturbance of the sensibility of the right upper limb appeared. Blood sample showed 6510 eosinophils per microlitre. The cerebral magnetic resonance demonstrated bilateral frontal and left parietal subcortical lesions from which the most voluminous presented large haemorrhagic areas...
February 7, 2016: Acta Clinica Belgica
https://www.readbyqxmd.com/read/26971201/-pathophysiology-of-eosinophilic-granulomatosis-with-polyangitis-churg-strauss
#20
REVIEW
B Chaigne, J Dion, L Guillevin, L Mouthon, B Terrier
Eosinophilic granulomatosis with polyangitis (EGPA) (formerly Churg-Strauss syndrome) is a rare small-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides. MPO-ANCA is present in only 31 to 38% of patients. In this review, we describe the pathophysiology of EGPA, which is characterized by a genetic predisposition, an environmental association, and a cellular dysfunction of eosinophils, neutrophils, and T and B cells.
May 2016: La Revue de Médecine Interne
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