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Atypical purpuric oedema of the nose during granulomatosis with polyangiitis.
We report the case of a 47-year-old woman presenting purpuric oedematous plaque with bullous detachment of the nose and hospitalised for the assessment of two suspicious neoplastic lung lesions discovered as a result of a recent stroke and repeated seromucosal otitis. Granulomatosis with polyangeitis was suspected because of multiple systemic lesions. The histopathology of skin lesions and laboratory investigation results were consistent with this diagnosis. A favourable outcome was achieved with corticosteroids and rituximab. DISCUSSION: The diagnosis of GPA is based on criteria established by the American College of...
Source: Annales de Dermatologie et de Cenereologie - April 14, 2020 Category: Dermatology Authors: Colas M, Bentz MH, Raffoul J, Devalland C, Dupond AS Tags: Ann Dermatol Venereol Source Type: research

Neurological Involvement in Primary Systemic Vasculitis
Conclusion Neurological involvement is a common complication of PSV (Table 1), and neurologists play an important role in the identification and diagnosis of PSV patients with otherwise unexplained neurological symptoms as their chief complaint. This article summarizes the neurological manifestations of PSV and hopes to improve neuroscientists' understanding of this broad range of diseases. TABLE 1 Table 1. Common CNS and PNS involvements of primary systemic vasculitis. Author Contributions SZ conceived the article and wrote the manuscript. DY and GT reviewed and edited the manuscript. All authors ...
Source: Frontiers in Neurology - April 25, 2019 Category: Neurology Source Type: research

Wegener's Granolomatosis Presenting as Wallenberg Syndrome: A Case Report
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia.
Source: Journal of Stroke and Cerebrovascular Diseases - April 22, 2019 Category: Neurology Authors: Maziar Emamikhah, Farzad Sina, Mahisa Mokhtari, Fatemeh Shirani, Mohammadreza Asadipanah Tags: Case Report Source Type: research

Wegener's Granulomatosis Presenting as Wallenberg Syndrome: A Case Report
Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis, a vasculitis affecting small and medium sized vessels usually affects the upper and lower respiratory tract, the kidneys, and the eyes. Neurologic manifestation in central nervous system (CNS) is less frequent than the peripheral and usually is in form of stroke. Few cases of lateral medullary ischemic stroke (Wallenberg syndrome) due to GPA have been reported. A 41 year-old female, presented with acute vertigo, nausea/vomiting, hiccups, dysphagia.
Source: Journal of Stroke and Cerebrovascular Diseases - April 22, 2019 Category: Neurology Authors: Maziar Emamikhah, Farzad Sina, Mahisa Mokhtari, Fatemeh Shirani, Mohammadreza Asadipanah Tags: Case Report 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

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 - 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

High risk of pulmonary embolism and deep venous thrombosis but not of stroke in granulomatosis with polyangiitis (Wegener’s)
Conclusion: The present study confirms that GPA patients have a markedly increased risk of venous thromboembolism. We did not observe an increased risk of stroke in our cohort. Thus, our observations demonstrate a differential impact of GPA and/or its treatment on the risk of various vascular events. © 2014 American College of Rheumatology.
Source: Arthritis Care and Research - July 29, 2014 Category: Rheumatology Authors: Mikkel Faurschou, Niels Obel, Bo Baslund Tags: Original Article Source Type: research

Lateral Medullary Stroke in Patient with Granulomatous Polyangiitis
We report a 41-year-old man with lateral medullary infarction who developed rapidly progressive renal failure. He was diagnosed with GPA based on positive serum c-ANCA and antiproteinase 3 antibodies and demonstration of pauci-immune crescentic glomerulonephritis on kidney biopsy. He was treated with Coumadin, pulse steroids, cyclophosphamide, and plasmapheresis. He had resolution of his neurologic deficits and improvement in renal function. This case report highlights the importance to consider GPA vasculitis in the differential diagnosis of stroke in patients with development of acute kidney injury.
Source: Journal of Stroke and Cerebrovascular Diseases - October 14, 2013 Category: Neurology Authors: Olga D. Taraschenko, Colum F. Amory, Jonathan Waldman, Era K. Hanspal, Gary L. Bernardini Tags: Case Reports Source Type: research

Ischemic stroke as a presenting feature of Wegener's granulomatosis.
We describe a 51-old-male who presented with complaints of slurred speech and left-sided weakness, along with bilateral pulmonary nodules on initial chest x-ray, which were associated with pulmonary symptoms in the past. A repeated computed tomography (CT) scan showed ischemia in the distribution of the right-middle cerebral artery. An open lung biopsy showed vasculitis with necrosis of a vessel wall, with a multinucleated giant cell, confirming the diagnosis of WG. The patient was successfully treated with cyclophosphamide and oral prednisone and he has continued in remission for one year. PMID: 24266130 [PubMed - in process]
Source: Connecticut Medicine - October 1, 2013 Category: Journals (General) Authors: Jethava A, Ali S Tags: Conn Med Source Type: research