Extracorporeal membrane oxygenation rescue in adolescent with bronchiolitis obliterans ‐organizing pneumonia like Wegener's granulomatosis
We report a 17‐year‐old woman with bronchiolitis obliterans‐organizing pneumonia (BOOP)‐like granulomatosis with polyangiitis developing severe airway obliterations. Pending age, phase and grade of autoimmune treatment, and offering ECMO treatment may be crucial for survival but occasionally preface futility. ECMO‐treated patient with BOOP‐like GPA has never been described before.
We report a 17‐year‐old woman with bronchiolitis obliterans‐organizing pneumonia (BOOP)‐like granulomatosis with polyangiitis developing severe airway obliterations. Pending age, phase and grade of autoimmune treatment, and o...
Source: Clinical Case Reports - December 16, 2016 Category: Journals (General) Authors: Lars Falk, Lars Mikael Broman Tags: Case Report Source Type: research
Granulomatosis With Polyangiitis-Associated Hypertrophic Pachymeningitis Mimicking Spontaneous Intracranial Hypotension: A Case Report.
CONCLUSIONS: Hypertrophic pachymeningitis associated with granulomatosis with polyangiitis can present with positional headache and subdural hygroma, mimicking spontaneous intracranial hypotension. Granulomatosis with polyangiitis should be suspected when patients with spontaneous intracranial hypotension or hypertrophic pachymeningitis show atypical features.
PMID: 27943273 [PubMed - as supplied by publisher] (Source: Headache)
Source: Headache - December 9, 2016 Category: Neurology Authors: Jung YH, Lee MJ, Lee C, Cha J, Chung CS Tags: Headache Source Type: research
Bilateral acute angle-closure glaucoma as a first presentation of granulomatosis with polyangiitis (Wegener's)
We report a case of bilateral acute angle-closure glaucoma in a patient with undiagnosed granulomatosis with polyangiitis (Wegener's). A 59-year-old man presented with a severe headache, ocular pain, blurred vision, shortness of breath, and mild fever. Clinical examination revealed conjunctival chemosis, corneal edema, and shallow anterior chambers. Closed angles were observed bilaterally on gonioscopy. The patient was treated with intravenous mannitol, oral acetazolamide, and anti-glaucomatous eye drops. Over the following two days, his vision improved and intraocular pressures decreased. Subsequently, laser iridotomies w...
Source: Arquivos Brasileiros de Oftalmologia - December 9, 2016 Category: Opthalmology Source Type: research
Myeloperoxidase –Antineutrophil Cytoplasmic Antibody (ANCA)–Positive Granulomatosis With Polyangiitis (Wegener's) Is a Clinically Distinct Subset of ANCA‐Associated Vasculitis: A Retrospective Analysis of 315 Patients From a German Vasculitis Referral Center
ConclusionPatients with MPO‐ANCA−positive GPA show significantly different clinical courses compared to those with PR3‐ANCA−positive GPA or MPO‐ANCA−positive MPA, which should be considered in their clinical management. Classification according to ANCA specificity may improve the evaluation of relapse risk. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - November 27, 2016 Category: Rheumatology Authors: Jan H. Schirmer, Marvin N. Wright, Kristine Herrmann, Martin Laudien, Bernhard N ölle, Eva Reinhold‐Keller, Jan P. Bremer, Frank Moosig, Julia U. Holle Tags: Vasculitis Source Type: research
Transmembrane TNF-alpha reverse signaling leading to TGF-beta production is selectively activated by TNF targeting molecules: therapeutic implications
Publication date: Available online 22 November 2016 Source:Pharmacological Research Author(s): Zsuzsa Szondy, Anna Pallai Tumor necrosis factor (TNF)-α is a potent pro-inflammatory cytokine exerting pleiotropic effects on various cell types. It is synthesized in a precursor form called transmembrane TNF-α (mTNF-α) which, after being processed by metalloproteinases, is released in a soluble form to mediate its biological activities through Type 1 and 2 TNF receptors in TNF receptor expressing cells. In addition to acting in soluble form, TNF-α also acts in the transmembrane form both as a ligand by activating TNF re...
Source: Pharmacological Research - November 22, 2016 Category: Drugs & Pharmacology Source Type: research
Rituximab for treating multiple sclerosis: Off-label but on target
Physicians commonly use drugs off-label. A medication approved by the Food and Drug Administration and other regulatory agencies for one indication may be useful for treating others. An example is rituximab, which is a chimeric monoclonal antibody that targets CD20 on B cells. Rituximab lyses the targeted cells, resulting in prolonged depletion of circulating B cells. It is approved to treat B-cell malignancies, rheumatoid arthritis, Wegener granulomatosis, and microscopic polyarteritis. However, neurologists have successfully used rituximab off-label to treat neuroimmunologic diseases, including neuromyelitis optica, myas...
Source: Neurology - November 13, 2016 Category: Neurology Authors: Bourdette, D. Tags: Class IV, Multiple sclerosis EDITORIALS Source Type: research
Positive microbiology findings in non-cystic fibrosis bronchiectasis and co-morbidities
Conclusion: Mayor part of fungal findings represent only colonization. Management of non-CFB should consider other co-existingdiseases and patient's condition. (Source: European Respiratory Journal)
Source: European Respiratory Journal - November 7, 2016 Category: Respiratory Medicine Authors: Arsovski, Z., Kjaeva, B., Dokic, D., Goseva, Z., Pejkovska, S., Janeva, E., Arbutina, S., Debreslioska, A., Arsovska, A. Tags: 10.1 Respiratory Infections Source Type: research
Image Gallery: Symmetrical necrosis of the breast as an inaugural manifestation of granulomatosis with polyangiitis (Wegener granulomatosis).
PMID: 27790680 [PubMed - in process] (Source: The British Journal of Dermatology)
Source: The British Journal of Dermatology - October 31, 2016 Category: Dermatology Authors: Gallais Sérézal I, Jennische K Tags: Br J Dermatol Source Type: research
Development and validation of case ‐finding algorithms for the identification of patients with anti‐neutrophil cytoplasmic antibody‐associated vasculitis in large healthcare administrative databases
ConclusionCase‐finding algorithms accurately identify patients with GPA, EGPA, and MPA in administrative databases. These algorithms can be used to assemble population‐based cohorts and facilitate future research in epidemiology, drug safety, and comparative effectiveness. Copyright © 2016 John Wiley & Sons, Ltd. (Source: Pharmacoepidemiology and Drug Safety)
Source: Pharmacoepidemiology and Drug Safety - October 31, 2016 Category: Drugs & Pharmacology Authors: Antoine G. Sreih, Narender Annapureddy, Jason Springer, George Casey, Kevin Byram, Andy Cruz, Maya Estephan, Vince Frangiosa, Michael D. George, Mei Liu, Adam Parker, Sapna Sangani, Rebecca Sharim, Peter A. Merkel, Tags: Original Report Source Type: research
Granulomatosis With Polyangiitis ‐Associated Hypertrophic Pachymeningitis Mimicking Spontaneous Intracranial Hypotension: A Case Report
ConclusionsHypertrophic pachymeningitis associated with granulomatosis with polyangiitis can present with positional headache and subdural hygroma, mimicking spontaneous intracranial hypotension. Granulomatosis with polyangiitis should be suspected when patients with spontaneous intracranial hypotension or hypertrophic pachymeningitis show atypical features. (Source: Headache: The Journal of Head and Face Pain)
Source: Headache: The Journal of Head and Face Pain - October 31, 2016 Category: Neurology Authors: Young Hee Jung, Mi Ji Lee, Chungbin Lee, Jihoon Cha, Chin ‐Sang Chung Tags: Clinical Correspondence Source Type: research
Disease Activity, Glucocorticoid Exposure, and Rituximab Determine Body Composition Changes during Induction Treatment of ANCA ‐Associated Vasculitis
This article is protected by copyright. All rights reserved. (Source: Arthritis Care and Research)
Source: Arthritis Care and Research - September 30, 2016 Category: Rheumatology Authors: Zachary S. Wallace, Eli M. Miloslavsky, Matthew Cascino, Sebastian H. Unizony, Na Lu, Gary S. Hoffman, Cees G.M. Kallenberg, Carol A. Langford, Peter A. Merkel, Paul A. Monach, Philip Seo, Robert Spiera, E. William St. Clair, Ulrich Specks, Paul Brunetta, Tags: Original Article Source Type: research
Risk of Myocardial Infarction and Stroke in Patients With Granulomatosis With Polyangiitis (Wegener's): A Population ‐Based Study
ConclusionPatients with GPA have a significantly increased risk of MI and a non–statistically significant trend toward an increased risk of ischemic stroke. Monitoring for this complication and vigilance in modifying risk factors are particularly warranted in this patient population, especially early after the diagnosis of GPA. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - September 28, 2016 Category: Rheumatology Authors: J. Antonio Avi ña‐Zubieta, Alice Mai, Neda Amiri, Natasha Dehghan, Ju Ann Tan, Eric C. Sayre, Hyon K. Choi Tags: Full Length Source Type: research
Comparing Presenting Clinical Features in 48 Children With Microscopic Polyangiitis to 183 Children Who Have Granulomatosis With Polyangiitis (Wegener's): An ARChiVe Cohort Study
ConclusionYounger age at disease onset and, perhaps, both gastrointestinal manifestations and more severe kidney disease seem to characterize the clinical profile in children with MPA compared to those with GPA. Delay in diagnosis suggests that recognition of these systemic vasculitides is suboptimal. Compared with adults, initial treatment regimens in children were comparable, but the complete reversal of female‐to‐male disease prevalence ratios is a provocative finding. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - September 27, 2016 Category: Rheumatology Authors: David A. Cabral, Debra L. Canter, Eyal Muscal, Kabita Nanda, Dawn M. Wahezi, Steven J. Spalding, Marinka Twilt, Susanne M. Benseler, Sarah Campillo, Sirirat Charuvanij, Paul Dancey, Barbara A. Eberhard, Melissa E. Elder, Aimee Hersh, Gloria C. Higgins, Ad Tags: Vasculitis Source Type: research
Management Scheme For Cerebral Wegener Granulomatosis: An Unusual Pseudotumoral Skull Base pathology
Granulomatosis with polyangitis (Wegener's disease) is a rare ANCA (anti-neutrophil cytoplasmic antibody) positive vasculitis that seldom involves brain and meninges. We herein present a case of a 35-year-old woman presenting with granulomatosis with polyangitis involving the brain and the meninges. The histological diagnosis required a complete resection of the lesion. Despite the possibility of post operative wound infection surgery was mandatory to ascertain the diagnostic and to treat locally. (Source: World Neurosurgery)
Source: World Neurosurgery - September 22, 2016 Category: Neurosurgery Authors: Anne Laure Bernat, Etienne Lefevre, Damien S ène, Philippe Herman, Homa Adle Biassette, Sebastien Froelich Tags: Case Report Source Type: research
[Granulomatous polyangitis (Wegener granulomatosis): Clinical findings and results of long-term follow-up].
Conclusions: Diagnosis of GPA may be delayed by the nonspecific nature of its symptoms. URT involvement was associated with good prognosis, whereas cardiac involvement and ESRF requiring dialysis were associated with poor outcome. Plasmapheresis may increase the rate of renal recovery in the patients with ESRF requiring dialysis.
PMID: 28366156 [PubMed - in process] (Source: Tuberkuloz ve Toraks)
Source: Tuberkuloz ve Toraks - September 1, 2016 Category: Respiratory Medicine Authors: Şen N, Aydın Tufan M, Yıldız R, Ersözlü Bozkırlı ED, Yücel E Tags: Tuberk Toraks Source Type: research
