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Wegener Granulomatosis Neuropathy

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Cranial Base Manifestations of Granulomatosis with Polyangiitis.

Patrick T Kiessling, John P Marinelli, Pierce A Peters, David R DeLone, John I Lane, Matthew J Koster, Matthew L Carlson,

OBJECTIVE:Although granulomatosis with polyangiitis (GPA; Wegener's granulomatosis) is classically characterized by systemic disease involving the kidneys and airway, approximately 10% of patients who have it present with isolated central nervous system disease. When involving the skull base, GPA frequently mimics more common pathology, resulting in diagnostic challenges and delay. The ... Read more >>

Otolaryngol Head Neck Surg (Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery)
[2020, :194599820912025]

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Differential clinicopathological features of EGPA-associated neuropathy with and without ANCA.

Ryoji Nishi, Haruki Koike, Ken Ohyama, Yuki Fukami, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Masahisa Katsuno, Gen Sobue,

OBJECTIVE:To investigate the clinicopathologic features of eosinophilic granulomatosis with polyangiitis (EGPA)-associated neuropathy with a focus on the presence or absence of anti-neutrophil cytoplasmic antibodies (ANCAs). METHODS:We examined the clinical features and pathologic findings of sural nerve biopsy specimens from 82 patients with EGPA-associated neuropathy. Of these patients, 32.9% were myeloperoxidase ... Read more >>

Neurology (Neurology)
[2020, :]

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A Rare Case of Central Nervous System Vasculitis in a Patient with Perinuclear Antineutrophil Cytoplasmic Antibodies-associated Interstitial Lung Disease.

Ammar Haikal, Tanawat Attachaipanich, Kyle Myers, Paul Schmidt, Arsany Anis,

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic necrotizing inflammation of the small vessels. Central nervous system (CNS) ANCA-associated vasculitis is a rare manifestation of AAV. Three mechanisms of AAV affecting the CNS have been reported which include contiguous granulomatous invasion from nasal and paranasal sinuses, remote granulomatous lesions, and vasculitis ... Read more >>

Cureus (Cureus)
[2020, 12(2):]

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Microscopic Polyangiitis Initially Presumed to Be Endocarditis.

Takahiro Kaneko, Shunsuke Hino, Yosuke Iijima, Norio Horie,

The antineutrophil cytoplasmic antibody- (ANCA-) associated vasculitides (AAVs), which include fever of unknown origin (FUO), are rare diseases characterized by necrotizing inflammation of small blood vessels and the presence of ANCAs. Microscopic polyangiitis (MPA) is a subtype of the AAVs. Although the prevalence of AAVs has generally increased over the ... Read more >>

(Case Reports in Dentistry)
[2020, 2020:]

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Asthma and hypercapnic respiratory failure.

P K Shamil, Nitesh Gupta, Sumita Agrawal, Pranav Ish, Shibdas Chakrabarti,

Hypercapnia is an uncommon entity in asthma exacerbation. Meticulous evaluation for the underlying aetiology of hypercapnia is warranted in these patients. http://bit.ly/2sTKTk2. ... Read more >>

Breathe (Sheff) (Breathe (Sheffield, England))
[2020, 16(1):190311]

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Eosinophilic granulomatosis with polyangiitis: the multifaceted spectrum of clinical manifestations at different stages of the disease.

Alvise Berti, Sara Boukhlal, Matthieu Groh, Divi Cornec,

Introduction: Eosinophilic granulomatosis with polyangiitis (EGPA) usually occurs in patients with late-onset asthma and sustained peripheral blood eosinophilia and classically presents with a clinical multifaceted spectrum of manifestations, which may vary at the different stages of the natural history of the disease.Areas covered: We reviewed EGPA clinical presentation, focusing on ... Read more >>

Expert Rev Clin Immunol (Expert review of clinical immunology)
[2020, 16(1):51-61]

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A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification.

Farah Zarka, Charles Veillette, Jean-Paul Makhzoum,

Primary systemic vasculitides are rare diseases that may manifest similarly to more commonly encountered conditions. Depending on the size of the vessel affected (large vessel, medium vessel, or small vessel), different vasculitis mimics must be considered. Establishing the right diagnosis of a vasculitis mimic will prevent unnecessary immunosuppressive therapy. ... Read more >>

Int J Rheumatol (International journal of rheumatology)
[2020, 2020:8392542]

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Bilateral corneal perforation and iris prolapse as a complication non-peripheral ulcerative keratitis in a patient with fulminant granulomatosis with polyangiitis: a case report.

Andrés Vargas-Villanueva, Natalia Carvajal-Saiz, Juliana Muñoz-Ortiz, Alejandra de-la-Torre,

J Ophthalmic Inflamm Infect (Journal of ophthalmic inflammation and infection)
[2020, 10(1):2]

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Relapses in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis: a retrospective study.

Roderau Outh, Anne Lemaire, Alexandre Mania, Pauline Berland, Laurent Gerbaud, Olivier Aumaître, Marc André,

OBJECTIVE:To investigate the activity of relapsing events (RE) and their mode of presentation in patients with anti-neutrophil cytoplasmic (ANCA)-associated vasculitis (AAV). METHODS:Patients diagnosed with AAV between 1990 and 2015 experiencing at least one RE were investigated. The different organ involvements were registered during each RE. Presentation at initial onset (IO) ... Read more >>

Clin. Rheumatol. (Clinical rheumatology)
[2020, :]

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Orbital pseudotumor as the presenting symptom of Crohn's disease in a male child.

Rhys Ishihara, Samiksha Fouzdar Jain, Deborah Perry, Adam Reinhardt, Donny Suh, Richard Legge,

Purpose:This report will describe a case of orbital pseudotumor that is associated with underlying Crohn's disease in a pediatric patient. Observations:An 8-year-old male with a past medical history of chronic constipation who presented to the ophthalmologist in July 2017 with a 7-month history double vision, left upper lid ptosis, left ... Read more >>

Am J Ophthalmol Case Rep (American journal of ophthalmology case reports)
[2020, 18:100669]

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Inflammatory optic neuropathy in granulomatosis with polyangiitis can mimick isolated idiopathic optic neuritis.

Manon Clément, Antoine Néel, Frédérique Toulgoat, Michel Weber, Pascal Godmer, Pascal Hutin, Mohamed Hamidou, Pierre Lebranchu,

OBJECTIVE:We describe a clinico-radiological presentation of inflammatory optic neuropathy that mimicked optic neuritis. METHODS:Retrospective single-center case series and literature review of optic neuropathy without orbital pseudotumor. RESULTS:Five local patients fulfilled the inclusion criteria. Clinical presentation revealed rapidly progressive severe unilateral visual loss, retrobulbar pain (n = 4), and paralytic strabismus (simultaneous = 2, protracted = 2) ... Read more >>

Eur J Ophthalmol (European journal of ophthalmology)
[2019, :1120672119889008]

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Microscopic polyangiitis: an incidental finding in a patient with stroke.

Abubakar Tauseef, Muhammad Sohaib Asghar, Mariam Amir, Maryam Zafar, Ayesha Anum, Haris Alvi, Mustafa Dawood, Uzma Rasheed, Mohammed Akram, Nisar Ahmed,

Microscopic polyangiitis (MPA) is a primary systemic vasculitis characterized by inflammation of small-sized vessels associated with the presence of anti-neutrophilic cytoplasmic antibodies. We report a case of a 39-year-old female diagnosed with microscopic polyangiitis as an incidental finding who presented with signs and symptoms of a stroke at a young ... Read more >>

J Community Hosp Intern Med Perspect (Journal of community hospital internal medicine perspectives)
[2020, 10(1):50-54]

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Microscopic Polyangiitis with Pulmonary Fibrosis: An Often-Recognized Manifestation of the Disease.

Liam M Clifford, Jamma Li, Christopher J Renaud, Suran L Fernando,

Background:Microscopic polyangiitis (MPA) can manifest with atypical features such as pulmonary fibrosis and chronic obstructive pulmonary disease (COPD), which are atypical and unusual features of small vessel vasculitis. Case Presentation:This paper presents two patients with microscopic polyangiitis and respiratory symptoms attributable to atypical pulmonary manifestations. Pulmonary fibrosis was present in ... Read more >>

Case Rep Rheumatol (Case reports in rheumatology)
[2019, 2019:2673947]

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Necrotizing Sarcoid Granulomatosis: A Disease Not to be Forgotten.

A I Parejo-Morón, M L Tornero-Divieso, M R Férnandez-Díaz, L Muñoz-Medina, O Preda, N Ortego-Centeno,

Sarcoidosis is a systemic granulomatous disease of unknown aetiology characterised by the appearance of noncaseifying epithelioid granulomas in the affected organs, most commonly the lungs, skin, and eyes (Iannuzzi et al. 2007). Necrotizing Sarcoid Granulomatosis (NGS) is a rare and little-known form of disease, which also presents nodular lung lesions, ... Read more >>

Case Rep Med (Case Reports in Medicine)
[2020, 2020:5730704]

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"Idiopathic Eosinophilic Vasculitis": Another Side of Hypereosinophilic Syndrome? A Comprehensive Analysis of 117 Cases in Asthma-Free Patients.

Guillaume Lefèvre, Amélie Leurs, Jean-Baptiste Gibier, Marie-Christine Copin, Delphine Staumont-Sallé, Frédéric Dezoteux, Cécile Chenivesse, Benjamin Lopez, Louis Terriou, Eric Hachulla, David Launay, Nicolas Etienne, Myriam Labalette, Pascal DeGroote, François Pontana, Thomas Quemeneur, Pierre-Yves Hatron, Nicolas Schleinitz, Jean-François Viallard, Mohamed Hamidou, Thierry Martin, Chafika Morati-Hafsaoui, Matthieu Groh, Marc Lambert, Jean-Emmanuel Kahn, ,

BACKGROUND:The absence of asthma may rule out a diagnosis of eosinophilic granulomatosis with polyangiitis in patients with hypereosinophilic syndrome (HES) and features of vasculitis. OBJECTIVE:To describe eosinophilic vasculitis (EoV) as a possible manifestation of HES in asthma-free patients. METHODS:We screened our hospital database and the literature for patients with HES ... Read more >>

J Allergy Clin Immunol Pract (The journal of allergy and clinical immunology. In practice)
[2019, :]

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Oral corticosteroid-sparing effects of reslizumab in the treatment of eosinophilic granulomatosis with polyangiitis.

Brian D Kent, Grainne d'Ancona, Mariana Fernandes, Linda Green, Cris Roxas, Louise Thomson, Alexandra M Nanzer, Joanne Kavanagh, Sangita Agarwal, David J Jackson,

Blockade of interleukin-5 with reslizumab appears to have significant oral corticosteroid sparing effects in patients with eosinophilic granulomatosis with polyangiitis and severe eosinophilic asthma http://bit.ly/2D2yYSK. ... Read more >>

ERJ Open Res (ERJ open research)
[2020, 6(1):]

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Nail changes in acro-osteolysis: A case report and review of the literature.

Nicola Anne Gray, Christiaan Scott, Reginald Mzudumile Ngwanya, Komala Pillay, Thuraya Isaacs,

JAAD Case Rep (JAAD case reports)
[2019, 5(12):1033-1036]

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Tolosa-Hunt Syndrome: Long-Term Outcome and Role of Steroid-Sparing Agents.

Anupriya Arthur, Ajith Sivadasan, Pavitra Mannam, A T Prabakhar, Sanjith Aaron, Vivek Mathew, M Karthik, Rohith Ninan Benjamin, Shaikh Atif Iqbalahmed, Gideon Lyngsyun Rynjah, Mathew Alexander,

Background:Tolosa-Hunt Syndrome (THS) is one of the causes of cavernous sinus syndrome causing painful ophthalmoplegia. Literature on long-term outcome of this rare condition is scarce. Aims and Objectives:The aim is to study the recurrence and role of steroid-sparing agents in THS. Methodology:All cases of THS treated at a tertiary-level teaching ... Read more >>

Ann Indian Acad Neurol (Annals of Indian Academy of Neurology)
[2020, 23(2):201-205]

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Granulomatosis with Polyangiitis Manifesting as a Symptomatic Sellar Mass in a Young Woman.

Sachin P Gadani, Rohini Nadgir, Annika Windon, Lisa Rooper, Fahid Alghanim,

A pituitary mass is a rare and poorly understood complication of granulomatosis with polyangiitis (GPA). Here we describe the case of a young woman with GPA who presented with signs and symptoms initially suggestive of meningitis but was ultimately found to have hypopituitarism and an enlarging sellar mass. She underwent transsphenoidal ... Read more >>

Cureus (Cureus)
[2019, 11(10):e5823]

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A 60-year-old with heart failure symptoms due to 1st degree AV-Block caused by granulomatosis with polyangiitis (Wegener's granulomatosis).

Davit Saghabalyan, Dirk Schiller, Sven Lerch,

Granulomatosis with polyangiitis is a rare systemic inflammatory disorder mainly affecting the small vessels. Cardiac involvement is rare, conduction delay being the most rare one. This case reports on a middle-aged male patient with heart failure symptoms due to a 1st degree atrioventricular block with a marked PR prolongation of ... Read more >>

J Cardiol Cases (Journal of cardiology cases)
[2020, 21(3):114-118]

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A case of giant cell arteritis presenting with nodular posterior scleritis mimicking a choroidal mass.

Caroline Awh, David A Reichstein, Akshay S Thomas,

Purpose:Herein we present a case of giant cell arteritis presenting with nodular posterior scleritis and exudative retinal detachment mimicking a choroidal mass. Observations:A 67-year-old man presented with sudden onset left-sided periorbital pain, blurry vision, and a choroidal lesion in the posterior pole. Despite treatment with high-dose oral prednisone for suspected ... Read more >>

Am J Ophthalmol Case Rep (American journal of ophthalmology case reports)
[2020, 17:100583]

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Multiple cerebral infarction diagnosed as Eosinophilic Granulomatosis with Polyangiitis by autopsy.

Kenichiro Hira, Hideki Shimura, Riyu Kamata, Masashi Takanashi, Akane Hashizume, Keiji Takahashi, Mizuho Sugiyama, Hiroshi Izumi, Nobutaka Hattori, Takao Urabe,

BACKGROUND:Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare systemic vasculitis of unknown cause involving the brain and accompanied by prominent eosinophilia. Intracardiac thrombosis is a major cardiac complication of EGPA that may cause thromboembolism. CASE PRESENTATION:A 53-year-old man presenting with abulia (consciousness disturbance) and left upper limb paralysis was admitted ... Read more >>

BMC Neurol (BMC neurology)
[2019, 19(1):288]

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An Atypical Case of Vasculitis: When 'Occult' 18FDG-PET Scan Findings Create a Classification Dilemma.

Elena Lopez-Francos, Sammy Place, Daphne Delplace, Frédéric Vandergheynst,

We describe a 66-year old patient with a recurrent ulcer on her right ankle. Biopsy revealed medium-vessel vasculitis consistent with cutaneous polyarteritis nodosa. There were no signs or symptoms suggestive of systemic vasculitis, but a 18FDG-PET scan showed areas of increased uptake around the large arteries and the pelvic and ... Read more >>

Eur J Case Rep Intern Med (European journal of case reports in internal medicine)
[2020, 7(1):001301]

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Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides: Insights from the DCVAS study.

Antje Bischof, Veronika K Jaeger, Robert D M Hadden, Raashid A Luqmani, Anne-Katrin Pröbstel, Peter A Merkel, Ravi Suppiah, Anthea Craven, Michael P Collins, Thomas Daikeler,

OBJECTIVE:Reported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN. METHODS:Data of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis ... Read more >>

Neurol Neuroimmunol Neuroinflamm (Neurology(R) neuroimmunology & neuroinflammation)
[2019, 6(6):]

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Takayasu arteritis in a rural hospital in Indonesia.

Michael Lusida, Mohammad Zakky Kurniawan, Johanes Nugroho,

Takayasu arteritis (TA) is a rare chronic granulomatous inflammation of the aorta or its branches and is prevalent all around the world. It causes stenosis of large arteries and ischaemic damage to target organs. There is usually a delay in recognising TA because of the rarity and unfamiliarity with the ... Read more >>

BMJ Case Rep (BMJ case reports)
[2020, 13(1):]

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