580 results match your criteria: "Vasculitic Neuropathy"

Eosinophilic granulomatosis with polyangiitis (EGPA) is a chronic inflammatory disease belonging to the spectrum of small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCAs), also characterized by eosinophilic infiltration of target organs. Peripheral neuropathy (PN) affects about 2/3 of the patients as a presenting symptom and typically represents a vasculitic involvement. A few studies have addressed the role of intravenous immunoglobulin (IVIg) for the treatment of PN in EGPA.

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Vasculitic Myopathy: Clinical Characteristics and Long-Term Outcomes.

Neurology

December 2024

From the Department of Neurology (M.V.P., P.S., E.N.), and Division of Rheumatology (K.J.W., M.J.K.), Department of Internal Medicine, Mayo Clinic, Rochester, MN.

Article Synopsis
  • - The study investigates vasculitic myopathy, a rare manifestation of vasculitis, to characterize its clinical features, lab results, and treatment outcomes using a retrospective review of patient records from 1980 to 2022.
  • - Out of 25 identified patients, most presented with proximal weakness and pain, with 80% showing myopathy as the first sign of vasculitis; significant findings included inflammation in muscle biopsies and elevated blood markers in some cases.
  • - Key findings include high instances of perivascular inflammation in biopsies, with substantial destruction noted in vessel walls; a notable proportion of patients also displayed signs of peripheral neuropathy.
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Characteristics of Severe Asthma Clinic Patients With Eosinophilic Granulomatosis With Polyangiitis.

J Allergy Clin Immunol Pract

October 2024

Department of Respiratory and Critical Care Medicine, Singapore General Hospital, Singapore; Allergy Center, Singapore General Hospital, Singapore; Duke-NUS Medical School, Singapore.

Background: Eosinophilic granulomatosis with polyangiitis (EGPA) is a rare form of antineutrophil cytoplasm antibody (ANCA)-associated vasculitis associated with varying clinical presentations and overlapping multiorgan involvement. Asthma is a predominant feature of EGPA, typically in its prodromal phase, often severe, and precedes vasculitic complications. However, there is paucity of studies describing the prevalence and characteristics of EGPA in the asthma population.

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Introduction/aims: In vasculitic neuropathy (VN), it is not known whether nerve conduction studies (NCSs) showing low amplitude sensory nerve action potentials (SNAPs) or those with absent responses have a higher yield in detecting appropriate nerves for pathological confirmation. Our goal was to describe NCS findings of nerves prior to biopsy in patients with VN.

Methods: We performed a retrospective study between January 2000 and April 2021 in patients with VN who either met pathological definite criteria for VN or criteria for clinically probable VN and had NCS of the sural or superficial radial sensory nerves prior to biopsy of the same nerve.

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Neurobartonelloses: emerging from obscurity!

Parasit Vectors

October 2024

Intracellular Pathogens Research Laboratory, Comparative Medicine Institute, College of Veterinary Medicine, North Carolina State University, Raleigh, NC, USA.

Article Synopsis
  • Bartonella species are intracellular bacteria linked to various human diseases, often transmitted through infected bodily fluids or vectors, but infections have been reported without clear contact to animals or insects.
  • These bacteria are associated with neurological conditions, including neuroretinitis and encephalitis, primarily linked to species like Bartonella henselae and B. quintana, and can also cause peripheral neuropathies.
  • Recent studies have detected Bartonella DNA more frequently in patients with neuropsychiatric disorders, suggesting a potential connection between these infections and conditions such as schizophrenia and psychoses.
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Top 10 Clinical Pearls in Vasculitic Neuropathies.

Semin Neurol

September 2024

Department of Neurology, Virginia Commonwealth University, Richmond, Virginia.

Article Synopsis
  • Vasculitic neuropathies are inflammatory conditions affecting the peripheral nerves, often caused by systemic vasculitis or related disorders, leading to painful sensory and motor deficits.
  • Diagnosis involves extensive tests, including lab work, electrodiagnostics, and tissue biopsies, while treatment typically includes immunosuppressants and pain management.
  • Timely recognition and intervention are crucial to prevent serious complications, including permanent nerve damage or fatal outcomes.
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Article Synopsis
  • The peripheral nervous system and skeletal muscle are commonly affected in systemic vasculitis, leading to symptoms like myalgia, muscle weakness, and sensory deficits.
  • Vasculitic myopathy (VM) is marked by severe muscle pain but normal creatine kinase levels, and can be identified by MRI, while vasculitic neuropathy (VN) causes motor and sensory deficits, especially in the lower limbs.
  • Diagnosing these conditions typically involves histopathological examination of nerve and muscle biopsies, which is crucial for determining treatment strategies.
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[Peripheral neuroimmunological diseases - Neuropathological insights and clinical perspectives].

Nervenarzt

October 2024

Institut für Neuropathologie, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin, und Berlin Institute of Health (BIH), Charité-Universitätsmedizin, Berlin, Deutschland.

Article Synopsis
  • - The article focuses on peripheral neuroimmunological diseases, discussing the varying mechanisms, manifestations, and treatment advancements for conditions like idiopathic inflammatory myopathies and chronic inflammatory demyelinating polyneuropathy.
  • - It emphasizes the importance of muscle biopsies for accurate diagnoses, particularly in distinguishing between vasculitic myopathy and myositis, as well as identifying specific autoimmune characteristics through autoantibody testing.
  • - The text highlights that ongoing research and clinical trials aim to enhance understanding and treatment strategies for these diverse diseases, potentially leading to significant advancements in patient care.
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Anti-synthetase and myelodysplastic syndromes with deep morphea: an example of shared immunopathogenesis? A case-based review.

Rheumatol Int

November 2024

Department of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Mexico City, Mexico.

Article Synopsis
  • Anti-synthetase syndrome (AS) is a type of inflammatory muscle disease marked by specific autoantibodies and is often associated with myositis and lung issues; it sometimes includes skin lesions like those in dermatomyositis.
  • A case study of a 57-year-old man revealed a rare combination of AS, deep morphea, vasculitic neuropathy, and myelodysplastic syndrome, raising questions about shared underlying mechanisms among these conditions.
  • Despite receiving aggressive treatment, the patient experienced a severe decline and ultimately died from complications, underlining the complex interplay between AS and other inflammatory disorders.
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Ways to think about vasculitic neuropathy.

Curr Opin Neurol

October 2024

ANZAC Research Institute, Central Clinical School, University of Sydney, Sydney, Australia.

Purpose Of Review: Vasculitis as a pathomechanism for neuropathy can be isolated to the peripheral nervous system, a part of a systemic autoimmune condition or a component of another syndrome. This review aims to discuss the broad range of diagnoses in which vasculitic neuropathy can be encountered, highlight the progress in imaging techniques in identifying vasculitis, and the new drugs developed for other autoimmune diseases that may be applied to neurological conditions.

Recent Findings: Advances in imaging modalities, ultrasound, MRI and FDG-PET scanning for neuromuscular applications has redefined many aspects of vasculitic neuropathies.

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Vasculitic neuropathy is commonly associated with systemic vasculitis, leading to ischemic damage to the peripheral nerves and axonal degeneration. The typical clinical manifestation of vasculitic neuropathy is a sensory-dominant multiple mononeuropathy often accompanied by pain. Although vasculitic neuropathy is caused by various systemic diseases, ANCA-associated vasculitis, secondary systemic vasculitis linked to various collagen diseases, and non-systemic vasculitic neuropathy hold particular significance.

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Focal inflammatory neuropathies.

Handb Clin Neurol

May 2024

Division of Neuromuscular Medicine, Department of Neurology, Mayo Clinic, Rochester, MN, United States; Peripheral Neuropathy Research Laboratory, Mayo Clinic, Rochester, MN, United States. Electronic address:

Article Synopsis
  • The chapter discusses neuropathies characterized by inflammation or autoimmune responses affecting specific nerve roots, plexuses, and peripheral nerves.
  • It examines various types of focal autoimmune demyelinating neuropathies, vasculitic disorders, and other focal neuropathies related to conditions like diabetes and infections.
  • Key points include the clinical presentations, diagnostic methods, and treatment options for these conditions.
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Axillary and musculocutaneous neuropathies.

Handb Clin Neurol

May 2024

Neuroscience and Mental Health Institute and Division of Neurology, Department of Medicine, University of Alberta, Edmonton, AB, Canada. Electronic address:

This chapter covers axillary and musculocutaneous neuropathies, with a focus on clinically relevant anatomy, electrodiagnostic approaches, etiologic considerations, and management principles. Disorders of the lateral antebrachial cutaneous nerve, a derivative of the musculocutaneous nerve, are also reviewed. We emphasize the importance of objective findings, including the physical examination and electrodiagnostic evaluation in confirming the isolated involvement of each nerve which, along with the clinical history, informs etiologic considerations.

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Article Synopsis
  • Antineutrophil cytoplasmic antibody-related vasculitis (AAV) is a group of diseases that cause severe inflammation of small blood vessels, with subtypes including eosinophilic GPA, Microscopic Polyangiitis, and Granulomatosis with Polyangiitis.
  • A case report describes a 68-year-old man with AAV who experienced tingling and numbness in his legs, without respiratory symptoms or other organ involvement, confirmed by a nerve biopsy showing signs of vasculitic neuropathy.
  • After treatment with glucocorticoids and cyclophosphamide for six cycles, the patient's symptoms improved significantly, enhancing his quality of life.
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Objectives: This case series reports clinical features and outcome of four patients with non-systemic vasculitic neuropathy (NSVN) treated with the anti-CD20 agent rituximab.

Methods: Clinical, electrophysiological and biopsy data were retrospectively obtained and evaluated. Only patients with pathological definite or probable NSVN were included.

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[Modern Perspectives on Peripheral Neuropathology].

Brain Nerve

April 2024

Division of Neurology, Department of Internal Medicine, Saga University Faculty of Medicine.

Recent advances in genetic and antibody testing have limited pathological examination of peripheral nerve specimens. However, when examining peripheral neuropathological findings from a modern perspective, there is often an opportunity to comprehend previously unnoticed observations upon re-examining the same specimen. For example, electron microscopy studies have suggested that the components that distinguish between nodal regions and internodes play a pivotal role in the behavior of macrophages that initiate myelin phagocytosis in the demyelinating form of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP).

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Background: eosinophilic granulomatosis with polyangiitis (EGPA) is a rare multisystem inflammatory disease characterized by asthma, eosinophilia and granulomatous or vasculitic involvement of various organs. While the eye is uncommonly affected in patients with EGPA, multiple ophthalmic manifestations have been reported, which can result in serious visual impairment without timely treatment.

Case Report: we report the case of a 79-year-old woman with a history of asthma and nasal polyps who presented with low-grade fever, mild alteration of mental status, and fatigue.

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To investigate the clinical and electrophysiological characteristics of ANCA-associated vasculitic neuropathy (VN) and analyze the predictors of treatment outcomes. Retrospective case series. In all, 652 consecutive patients with ANCA-associated vasculitis were admitted to the First Medical Center of the Chinese PLA General Hospital between January 2006 and December 2022.

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Automated whole slide morphometry of sural nerve biopsy using machine learning.

Neuropathol Appl Neurobiol

April 2024

Department of Neurology and Neurological Science, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan.

Aim: The morphometry of sural nerve biopsies, such as fibre diameter and myelin thickness, helps us understand the underlying mechanism of peripheral neuropathies. However, in current clinical practice, only a portion of the specimen is measured manually because of its labour-intensive nature. In this study, we aimed to develop a machine learning-based application that inputs a whole slide image (WSI) of the biopsied sural nerve and automatically performs morphometric analyses.

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Membranous nephropathy has been associated with demyelinating polyneuropathies and antiglomerular membrane disease; however, an association with vasculitic neuropathy has not been described. This case describes a patient with biopsy-proven idiopathic membranous nephropathy and synchronous mononeuritis multiplex secondary to idiopathic small vessel vasculitis, who presented with lower limb microvascular ischaemia, peripheral neuropathy and active urinary sediment. Her extensive non-invasive screening for immunological disease and radiological investigations for occult malignancy were unremarkable.

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Article Synopsis
  • Mononeuritis multiplex is a rare but severe side effect associated with immune checkpoint inhibitors, particularly noted in three patients with mesothelioma in a French study.
  • The study involved three elderly male patients who exhibited severe neurological symptoms and were diagnosed with mononeuritis multiplex after receiving treatment with nivolumab and ipilimumab.
  • All patients were treated with corticosteroids, resulting in improvements, but one patient required additional therapy (rituximab and cyclophosphamide) after symptom recurrence upon tapering steroids.
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Peripheral neuropathy is a common manifestation of systemic vasculitis. The etiology of vasculitic peripheral neuropathy is generally classified into two groups: systemic and nonsystemic. In systemic vasculitic neuropathy (SVN), neuropathy is a consequence of a systemic disease, most commonly involving medium and small vessels throughout the body.

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Vasculitic neuropathy-related disability, pain, quality of life, and autonomic symptoms: a survey of 312 patients.

Rheumatology (Oxford)

October 2024

Nuffield Department of Orthopaedics, Rheumatology and Musculoskeletal Science, University of Oxford, Oxford, UK.

Objectives: To assess self-reported symptoms of neuropathy, disability, pain, health-related quality of life (HR-QOL) and autonomic dysfunction in patients with vasculitis.

Methods: Patients with vasculitis (with or without neuropathy) were invited by Vasculitis UK to complete an anonymous online survey.

Results: Three hundred and twelve patients (71% female) responded.

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