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Granulomatosis polyangitis

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
A Gandhi, R B Kulkarni
No abstract text is available yet for this article.
January 2016: Journal of the Association of Physicians of India
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
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
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
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
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
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
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
Masahiro Fukuyama, Yohei Sato, Jun Hayakawa, Manabu Ohyama
Cutaneous metaplastic synovial cyst (CMSC) is a rare tumor typically representing a solitary, well-circumscribed and cystic mass, which is not connected to the joint. Synovial cysts have been predominantly reported by orthopedists or pathologists and the presence of CMSC has not been well recognized by dermatologists. Herein, we reported CMSC developing in a 68-year-old woman receiving systemic corticosteroid for the treatment of eosinophilic granulomatosis with polyangitis and attempt to delineate clinical characteristics of this unusual neoplasm by reviewing the literature, especially focusing on dermatological descriptions...
June 17, 2016: Keio Journal of Medicine
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
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
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
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
Anne E Richardson, Kishore Warrier, H Vyas
Pleuropulmonary manifestations of rheumatological diseases are rare in children but pose a significant risk to overall morbidity and mortality. We have reviewed the literature to provide an overview of the respiratory complications of the commonest rheumatological diseases to occur in children (juvenile systemic lupus erythematosus, scleroderma, juvenile dermatomyositis, mixed connective tissue disease, granulomatosis with polyangitis and juvenile idiopathic arthritis). Pulmonary function testing in these patients can be used to refine the differential diagnosis and establish disease severity, but also has a role in ongoing monitoring for respiratory complications...
August 2016: Archives of Disease in Childhood
Sushil Kathar, Zarir F Udwadia
Eosinophilic granulomatosis with polyangitis (EGPA; earlier called Churg-Strauss syndrome) is a small-vessel necrotising vasculitis typically characterised by asthma, lung infiltrates, extra-vascular necrotising granulomas and hyper-eosinophilia. Cardiac disease is a major contributor to disease-related deaths in EGPA. We describe the case of a 39-year-old woman with late onset asthma, allergic rhinosinusitis, and high extra-vascular and peripheral blood eosinophilia, presenting with peripheral neuropathy and pericardial effusion...
April 2015: Indian Journal of Chest Diseases & Allied Sciences
Benjamin Chaigne, Benjamin Terrier, Nathalie Thieblemont, Véronique Witko-Sarsat, Luc Mouthon
Eosinophilic granulomatosis with polyangitis (EGPA) is a rare small- and medium-sized vessel vasculitis belonging to the group of anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV). It is commonly divided into two phenotypes depending on the presence of ANCAs targeting myeloperoxidase (MPO). MPO-ANCAs are present in 31% to 38% of patients and are associated with a vasculitis phenotype of the disease, whereas patients without MPO-ANCA are at risk of cardiac involvement. Despite significant advances in understanding the overall pathogenesis of the disease, the explanation for this dichotomy is still unclear...
February 2016: Autoimmunity Reviews
Felipe Santos, Mehti Salviz, Haris Domond, Joseph B Nadol
OBJECTIVE: To describe the temporal bone histopathology of vasculitis in granulomatosis with polyangitis. BACKGROUND: Granulomatosis with polyangitis is an autoimmune disease that presents as granulomatosis and vasculitis. Otologic findings, including otitis media, hearing loss, vertigo, and facial paralysis are common in this condition. MATERIAL AND METHODS: The temporal bones of four subjects with manifestations of vasculitis attributed to granulomatosis with polyangitis were studied under light microscopy...
December 2015: Otology & Neurotology
D Sène
Eosinophilic fasciitis (EF) is a rare connective tissue disease characterized by symmetrical and painful swelling with a progressive induration and thickening of the skin and soft tissues. The diagnosis of EF is often based on the association of characteristic skin or subcutaneous abnormalities and a thickened fascia with an inflammatory infiltration, mostly composed of lymphocytes and eosinophils. A peripheral eosinophilia is frequently present (60-90%) but is not mandatory for the EF diagnosis. At the onset, the morphological diagnosis might be helped by a muscle magnetic resonance imaging, which typically may evidence an increased signal intensity within the fascia and marked fascia enhancement after gadolinium administration at the acute phase of the disease...
November 2015: La Revue de Médecine Interne
Ziyan Wu, Qingjun Wu, Juanjuan Xu, Si Chen, Fei Sun, Ping Li, Yina Bai, Wenjie Zheng, Hua Chen, Fengchun Zhang, Yongzhe Li
AIM: The vasculitis diseases granulomatosis with polyangiitis (GPA) and microscopic polyangitis (MPA) are the two major forms of anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitides (AAV). A recent genome-wide association study has shown that the genes HLA-DPB1 and HLA-DQ conferred susceptibility to GPA and MPA, respectively. We investigated the linkage between putative AAV-related genes (HLA-DPB1, ARHGAP18, CD226, CTLA-4, MOSPD2 and PRTN3) and AAV in a Han Chinese population...
May 27, 2015: International Journal of Rheumatic Diseases
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