Pseudo-tumoral form of wegeners disease as an atypical manifestation of central nervous system involvement (p6.203)
Conclusions:As long as we know there is no report in literature of a pseudotumoral CSN involvement of GPA. This requires to exclude other pseudo-tumoral lesions as tumors or infectious. The prognosis is poor even when glucocorticoid treatment is started immediately.Disclosure: Dr. Crespo Arizmendi has nothing to disclose. Dr. Raina has nothing to disclose. Dr. Tkachuk has nothing to disclose. Dr. Bustos has nothing to disclose. Dr. Paviolo has nothing to disclose. (Source: Neurology)
Source: Neurology - April 17, 2017 Category: Neurology Authors: Arizmendi, M. C., Raina, G., Tkachuk, V., Bustos, A., Paviolo, J. P. Tags: General Neurology: Inflammation, Infections, and Autoimmunity Source Type: research
Th17 and Treg lymphocytes as cellular biomarkers of disease activity in Granulomatosis with Polyangiitis
Granulomatosis with Polyangiitis (GPA) (formerly known as Wegener's granulomatosis) is a vasculitis of unknown etiology affecting predominantly small‐ to medium‐sized vessels, usually involving the upper and lower respiratory tract and kidneys. Anti‐neutrophil cytoplasmic autoantibodies are probably the initial cause of the inflammatory process that leads to the typical necrotizing lesions. In this issue of the European Journal of Immunology, Szczeklik et al. [Eur. J. Immunol. 2017. 47: 724–733] report some interesting findings on the possible involvement of T‐cell subsets in the pathogenesis of the disease. Thi...
Source: European Journal of Immunology - April 7, 2017 Category: Allergy & Immunology Authors: Lorenzo Cosmi Tags: Commentary Clinical Source Type: research
Identification of Functional and Expression Polymorphisms Associated With Risk for Antineutrophil Cytoplasmic Autoantibody –Associated Vasculitis
ConclusionThis study reveals the association of susceptibility to GPA and MPA with functional gene variants that explain much of the genetic etiology of AAV, could influence and possibly be predictors of the clinical presentation, and appear to alter immune cell proteins and responses likely to be key factors in the pathogenesis of AAV. (Source: Arthritis and Rheumatism)
Source: Arthritis and Rheumatism - April 6, 2017 Category: Rheumatology Authors: Peter A. Merkel, Gang Xie, Paul A. Monach, Xuemei Ji, Dominic J. Ciavatta, Jinyoung Byun, Benjamin D. Pinder, Ai Zhao, Jinyi Zhang, Yohannes Tadesse, David Qian, Matthew Weirauch, Rajan Nair, Alex Tsoi, Christian Pagnoux, Simon Carette, Sharon Chung, Davi Tags: Full Length Source Type: research
Accessory left gastric artery aneurysms in granulomatosis with polyangiitis: a case report and literature review.
Authors: Tomosugi T, Takahashi T, Kawase Y, Yoshida K, Hayashi S, Sugiyama T, Shimizu M, Shoka M, Sawaki K, Onishi E, Hayashi N, Matsushita H, Okochi O
Abstract
Aneurysm formation is a potential complication of granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis. It is a very rare complication, but immediate diagnosis and therapy should be performed because an aneurysm can be life-threatening if it ruptures. An accessory left gastric artery (ALGA) is also a rare variant gastric artery that may obtain its blood supply from the left hepatic artery and left gastric artery. We ...
Source: Nagoya Journal of Medical Science - March 19, 2017 Category: Global & Universal Tags: Nagoya J Med Sci Source Type: research
Macula retinal vasculitis and choroiditis associated with granulomatosis with polyangiitis
Conclusion: Granulomatosis with polyangiitis can be associated with macular necrosis leading to fibrosis, unresponsive to immune-modulating therapy. (Source: Retinal Cases and Brief Reports)
Source: Retinal Cases and Brief Reports - March 18, 2017 Category: Opthalmology Tags: Case Report Source Type: research
Ulcerative granuloma of the eyelid as the initial manifestation of granulomatosis with polyangiitis (Wegener's granulomatosis): A case report.
Abstract
A 56-year-old-man presented a 2-month history of chalazion in the eyelids without response to treatment and with an inconclusive biopsy. Laboratory results confirmed the presence of Enterobacter cloacae and Streptococcus gordonii infection. Despite appropriate intravenous antibiotic treatment, clinical worsening was observed. Radical surgical excision and total tarsorrhaphy were performed. Following treatment, the patient was asymptomatic for 6 weeks until he developed acute renal failure, generalised arthralgia, acute hypertensive anterior uveitis, and dacryoadenitis. Lacrimal gland biopsy reveal...
Source: Orbit - March 16, 2017 Category: Opthalmology Authors: Brosa Morros H, Subirà O, Gomà Gàllego M, Paúles Villar MJ, Mascaró Zamora F, Abia Serrano M Tags: Orbit Source Type: research
Wegener Granulomatosis with Oral Involvement as Primary Manifestation: A Case Study
Discussion: Up to now, few reports have been published on Wegener disease with oral involvement and in most of these articles, Wegener was diagnosed after respiratory symptoms and kidney or other organs involvement. Only in few studies was Wegener diagnosis confirmed on the basis of oral symptoms and gingival involvement. Immediate and aggressive administration of immunotherapy treatments are required due to the fatal nature of the disease as the survival rate of patients with untreated WG is low and 90% of these patients die within 1 year after respiratory or kidney involvement . (Source: Journal of Dental Materials and Techniques)
Source: Journal of Dental Materials and Techniques - March 16, 2017 Category: Dentistry Source Type: research
Rare Presentation of Wegener ’s granulomatosis in the pituitary gland: Case Report and Literature Review STOCKTONB
CONCLUSION It is important to consider this rare complication of WG to ensure timely diagnosis and management. (Source: International Journal of Surgery Case Reports)
Source: International Journal of Surgery Case Reports - February 20, 2017 Category: Surgery Source Type: research
Characteristics of hypertrophic pachymeningitis in patients with granulomatosis with polyangiitis
AbstractHypertrophic pachymeningitis (HP) is an important neurologic complication of granulomatosis with polyangiitis (GPA, formerly Wegener ’s granulomatosis). The aim of this study is to investigate the clinical features, radiological findings, and diagnostic pitfalls of GPA-related HP. A retrospective chart review was performed to screen patients diagnosed with GPA at Samsung Medical Center between 1997 and 2016. Neurologic manifest ation, laboratory findings, neuroimaging data, and clinical course were evaluated in all patients. Characteristics of patients with HP were compared to those of patients without HP. Sixty-...
Source: Journal of Neurology - February 19, 2017 Category: Neurology Source Type: research
Surgical treatment of nasal septal perforations: SIR (Italian Society of Rhinology) experts opinion.
CONCLUSIONS: Given the great difficulty to make random studies about controversial topics and obtain statistically significant data related to that, expert opinion shall be of great value (expert opinion, level of evidence 5).
PMID: 28162219 [PubMed - as supplied by publisher] (Source: Acta Otorrinolaringologica Espanola)
Source: Acta Otorrinolaringologica Espanola - February 1, 2017 Category: ENT & OMF Authors: Passali D, Spinosi MC, Salerni L, Cassano M, Rodriguez H, Passali FM, Bellussi LM Tags: Acta Otorrinolaringol Esp Source Type: research
Fatal infection of Pneumocystis jiroveci pneumonia in a pemphigus patient treated with rituximab
This article is protected by copyright. All rights reserved. (Source: Journal of the European Academy of Dermatology and Venereology)
Source: Journal of the European Academy of Dermatology and Venereology - January 26, 2017 Category: Dermatology Authors: K.C. Wei, Y.H. Wang, W.H. Wang, W. Chen Tags: Letter to Editor Source Type: research
Value of non-identified ANCA (non-PR3, non-MPO) in the diagnosis of granulomatosis with polyangiitis (Wegener ’s granulomatosis)
. (Source: Acta Clinica Belgica)
Source: Acta Clinica Belgica - January 8, 2017 Category: Journals (General) Authors: M élanie Givaudan Fr édéric Vandergheynst Patrick Stordeur Annick Ocmant Christian Melot Val érie Gangji Muhammad S. Soyfoo Source Type: research
Acute urinary retention secondary to urethral involvement of granulomatosis with polyangiitis.
Abstract
Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a systemic necrotizing vasculitis of small- and medium-sized blood vessels, primarily affecting the upper and lower respiratory tracts, as well as the kidneys. Urogenital manifestations of GPA are exceedingly rare and usually respond well to systemic immunosuppressive therapy. Here, we present a case of a 36-year-old female presenting with acute urinary obstruction secondary to urethral GPA involvement in the immediate postpartum period. Special consideration should be given to ruling out malignancy in all patients with a...
Source: Canadian Urological Association Journal - December 31, 2016 Category: Urology & Nephrology Authors: Anderson PT, Gottheil S, Gabril M, Barra L, Power N Tags: Can Urol Assoc J Source Type: research
Pachymeningitis in granulomatosis with polyangiitis: case series with earlier onset in younger patients and literature review
The objective of this study is to describe the characteristics of patients with pachymeningitis (PM) in granulomatosis with polyangiitis (GPA) from Latin America, including three young patients. This is a retrospective case series. Patients were classified according to the ACR criteria, the 2012 Chapel Hill Consensus Conference Nomenclature and the EMA algorithm. Demographic, clinical, serological, and neuroimaging characteristics are described. Thirteen patients (nine females, four males) were identified. Mean age ± SD of PM diagnosis was 35.5 ± 20.4 years (median 48, range 8–71 years). Mean time ± SD betwee...
Source: Clinical Rheumatology - December 22, 2016 Category: Rheumatology Source Type: research
