504 results match your criteria Vasculitic Neuropathy

A Case of Hepatitis C Related Mixed Cryoglobulinemia Syndrome.

J Community Hosp Intern Med Perspect 2022 12;12(2):53-56. Epub 2022 Apr 12.

Department of Internal Medicine, Trinitas Regional Medical Center, Elizabeth, New Jersey, USA.

Hepatitis C virus (HCV) is an RNA virus that preferentially infects hepatocytes and is transmitted through infected blood contact. Chronic hepatitis C can result in serious life-threatening conditions like fibrosis, cirrhosis, and liver cancer. Additionally, it can result in extrahepatic conditions including lymphoproliferative disease and mixed cryoglobulinemic vasculitis. Read More

View Article and Full-Text PDF

Early ultrastructural lesions of anti-neutrophil cytoplasmic antibody- versus complement-associated vasculitis.

Neuropathology 2022 Jun 15. Epub 2022 Jun 15.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

This study aims to describe electron microscopic findings of vasculitis associated with anti-neutrophil cytoplasmic antibody (ANCA) and complement. Sural nerve biopsy specimens were obtained from 10 patients with microscopic polyangiitis (MPA), a representative ANCA-associated vasculitis, and six patients with nonsystemic vasculitic neuropathy (NSVN), who were negative for ANCA but positive for complement deposition. In patients with MPA, attachment of neutrophils to epineurial vascular endothelial cells, migration of neutrophils to the extravascular space via the penetration of the endothelial layer, and release of neutrophil components to the extracellular space were observed. Read More

View Article and Full-Text PDF

Patterns of myelinated nerve fibers loss in transthyretin amyloid polyneuropathy and mimics.

Ann Clin Transl Neurol 2022 Jun 4. Epub 2022 Jun 4.

Department of Neurology, Peking University First Hospital, 8 Xishiku Street, Xicheng District, Beijing, 100034, China.

Objective: The present study was intended to analyze the characteristics of myelinated nerve fibers density (MFD) of transthyretin amyloid polyneuropathy (ATTR-PN) and other similar neuropathies.

Methods: A total of 41 patients with ATTR-PN, 58 patients of other common peripheral neuropathies, and 17 age-and gender-matched controls who visited the First Hospital of Peking University and performed sural nerve biopsy between June 2007 and August 2021 were included for analysis of MFD.

Results: Except the vasculitic neuropathy group, the total and small MFD of patients in the ATTR-PN group were significantly lower than those of other disease groups. Read More

View Article and Full-Text PDF

The role of sural nerve biopsy in diagnosis of vasculitis.

J Rheumatol 2022 Jun 1. Epub 2022 Jun 1.

Department of Rheumatology, Lund University, Lund, Sweden; Department of Translational Medicine - Hand Surgery, Lund University, Malmö, Sweden; Department of Medicine, Vasculitis and Lupus Research Group, University of Cambridge, Cambridge, United Kingdom;Department of Neuropathology, Addenbrooke's Hospital, Cambridge University Hospitals NHS Foundation Trust, Cambridge, United Kingdom. This study was supported by grants from the Swedish Research Council (Vetenskapsrådet: 2019-01655), Faculty of Medicine, Lund University (ALF-medel). Correspondence to Karl Gisslander, Department of Rheumatology, Department of Medicine, Lund University 221 85 Lund;

Objective: The diagnostic yield of sural nerve biopsy (SNB) in vasculitis is uncertain. Our aim was to document relevant characteristics of patients undergoing sural nerve biopsy in the investigation of vasculitis; determine the diagnostic yield; relate positive biopsy findings to patient demographic, laboratory, and clinical parameters; and to calculate the rate of surgical complications.

Methods: Patients with suspected vasculitis that underwent SNB as part of diagnostic evaluation at academic medical centres in Sweden and the United Kingdom were identified by searching local pathology databases and clinic registers. Read More

View Article and Full-Text PDF

Blood biomarkers of peripheral neuropathy.

Acta Neurol Scand 2022 May 25. Epub 2022 May 25.

Department of Neuromuscular Disease, Queen Square UCL Institute of Neurology and the National Hospital of Neurology and Neurosurgery, London, UK.

Traditionally, neurophysiology is the primary diagnostic and prognostic biomarker in peripheral neuropathy clinical practice; however, it may lack responsiveness in the context of slowly progressive neuropathies and where there is significant axonal damage. The development of ultrasensitive platforms for measuring serum proteins at the lower limit of detection of traditional ELISA techniques has transformed the field of blood biomarkers of peripheral neuropathy. A variety of blood biomarkers have been identified from inflammatory cytokines and apokines in diabetic neuropathy through to neuron-specific proteins such as neurofilament light chain, Schwann cell-specific proteins such as TMPRSS5 and microRNAs in other acquired and hereditary neuropathies. Read More

View Article and Full-Text PDF

Role of complement components in vasculitic neuropathy associated with systemic lupus erythematosus and rheumatoid arthritis.

Muscle Nerve 2022 May 17. Epub 2022 May 17.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, Japan.

Introduction/aims: The mechanism of complement-mediated neurological injury in vasculitic neuropathy associated with systemic lupus erythematosus (SLE) and rheumatoid arthritis (RA) is unknown. The current study aimed to investigate the local activation of the complement system in vasculitic neuropathy associated with SLE and RA.

Methods: We analyzed sural nerve biopsy specimens collected from patients with SLE (n = 12) and RA (n = 12). Read More

View Article and Full-Text PDF

Progressive multifocal fibrosing neuropathy: description of a novel disease.

Acta Neuropathol Commun 2022 03 16;10(1):34. Epub 2022 Mar 16.

Jefferson Institute of Molecular Medicine and Scleroderma Center, Thomas Jefferson University, Philadelphia, PA, 19107, USA.

Entrapment peripheral neuropathies are clinically characterized by sensory impairment and motor deficits. They are usually caused by mechanical injuries, but they are also a frequent manifestation of metabolic diseases, toxic agent exposure, or systemic fibrotic disorders. Here we describe the clinical, radiological, and histopathological features of a novel progressive fibrotic disorder characterized by progressive multifocal fibrosing neuropathy. Read More

View Article and Full-Text PDF

Pallid Disc Oedema in a Young Patient: Clinical and Diagnostic Challenge.

Neuroophthalmology 2022 20;46(2):95-98. Epub 2021 Apr 20.

Ophthalmology Unit, Royal Adelaide Hospital, Adelaide, Australia.

A mid-thirties male with end-stage renal failure receiving haemodialysis on a background of four failed renal transplants, post-transplant lymphoproliferative disorder,and autonomic dysfunction presented with acute vision change in his left eye. Over days his vision in that eye deteriorated from 20/25 to no light perception. Given his complex medical background he was extensively investigated for infective, inflammatory, infiltrative and vasculitic aetiologies to explain acute vision loss with pallid disc swelling. Read More

View Article and Full-Text PDF

Peripheral neuropathy and livedoid vasculopathy.

J Neurol 2022 Jul 15;269(7):3779-3788. Epub 2022 Feb 15.

Department of Neurology, Nerve-Muscle Unit, Referral Center for Neuromuscular Diseases AOC, University Hospital of Bordeaux (CHU Bordeaux, Pellegrin Hospital), Bordeaux, France.

Background: Livedoid vasculopathy (LV) is a chronic dermatosis associated with micro-thrombosis of the vessels of the dermis, leading to ischemic lesions and painful skin ulcerations of the lower limbs. This thrombosing occlusive vasculopathy, clearly distinct from 'classical vasculitis' (not related to alteration of vessel walls), may lead to peripheral neuropathy.

Objective: To clarify the main clinical, electrophysiological and pathological characteristics of peripheral neuropathy linked to LV. Read More

View Article and Full-Text PDF

COVID-19 and the peripheral nervous system. A 2-year review from the pandemic to the vaccine era.

J Peripher Nerv Syst 2022 03 14;27(1):4-30. Epub 2022 Mar 14.

Department of Clinical Neurosciences, Fondazione IRCCS Istituto Neurologico "Carlo Besta", Milan, Italy.

Increasing literature has linked COVID-19 to peripheral nervous system (PNS) diseases. In addition, as we move from the pandemic to the vaccination era, literature interest is shifting towards the potential association between COVID-19 vaccines and PNS manifestations. We reviewed published literature on COVID-19, COVID-19 vaccines and PNS manifestations between 1 January 2020 and 1 December 2021. Read More

View Article and Full-Text PDF

A diagnostic conundrum; Kimura's disease mimicking eosinophilic granulomatosis with polyangiitis.

SAGE Open Med Case Rep 2022 25;10:2050313X211070522. Epub 2022 Jan 25.

Department of Pathology, Faculty of Medicine, University of Colombo, Colombo, Sri Lanka.

Kimura's Disease (KD) is a benign, relapsing chronic inflammatory condition often seen in young Asian males, manifesting as recurrent swellings in skin and subcutaneous tissues especially in the head and neck region. Eosinophilic granulomatosis with polyangitis (EGPA) is a debilitating multisystem vasculitic condition which causes high morbidity due to cavitating lung lesions, neuropathy and renal impairment. Eosinophilia is common to both conditions. Read More

View Article and Full-Text PDF
January 2022

ANCA-Associated Vasculitic Neuropathies: A Review.

Neurol Ther 2022 Mar 19;11(1):21-38. Epub 2022 Jan 19.

Department of Neurology, Nagoya University Graduate School of Medicine, Nagoya, 466-8550, Japan.

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is a systemic disorder that frequently affects the peripheral nervous system and consists of three distinct conditions: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, previously Wegener's granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, previously Churg-Strauss syndrome). The neuropathic features associated with this condition usually include mononeuritis multiplex, which reflects the locality of lesions. Findings suggestive of vasculitis are usually found in the epineurium and occur diffusely throughout the nerve trunk. Read More

View Article and Full-Text PDF

Adalimumab-induced sensory vasculitic neuropathy.

BMJ Case Rep 2022 Jan 17;15(1). Epub 2022 Jan 17.

Department of Neurology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.

Vasculitis and other autoimmune conditions are known complications of tumour necrosis factor alpha (TNF-α) inhibitor use. By definition, TNF-α inhibitor induced vasculitis is a secondary systemic vasculitis. However, its phenotype is varied and can present as an isolated vasculitic neuropathy. Read More

View Article and Full-Text PDF
January 2022

Immune mechanisms, the role of complement, and related therapies in autoimmune neuropathies.

Norman Latov

Expert Rev Clin Immunol 2021 12 23;17(12):1269-1281. Epub 2021 Nov 23.

Department of Neurology, Weill Cornell Medical College, New York, USA.

Introduction: Autoimmune neuropathies have diverse presentations and underlying immune mechanisms. Demonstration of efficacy of therapeutic agents that inhibit the complement cascade would confirm the role of complement activation.

Areas Covered: A review of the pathophysiology of the autoimmune neuropathies, to identify those that are likely to be complement mediated. Read More

View Article and Full-Text PDF
December 2021

Leukocytoclastic vasculitis associated with multifocal sensory neuropathy responsive to intravenous immunoglobulins: a case report.

Sarcoidosis Vasc Diffuse Lung Dis 2021 30;38(3):e2021022. Epub 2021 Sep 30.

Unit of Dermatology, Department of Medicine DIMED, University of Padua, Padua, Italy.

Leukocytoclastic vasculitis (LCV) is a common form of small-vessel vasculitis, which commonly presents as palpable purpura or petechiae, caused by deposition of circulating immune complexes on vessels walls that attracts granulocytes which damage the vascular endothelium and leading to erythrocytes extravasation. The skin is the most commonly involved organ, but also renal, gastrointestinal, pulmonary, cardiovascular and neurological systems may be affected. Skin lesions may be the initial signs of systemic vasculitis. Read More

View Article and Full-Text PDF
September 2021

Fluid Biomarkers for Monitoring Structural Changes in Polyneuropathies: Their Use in Clinical Practice and Trials.

Neurotherapeutics 2021 10 18;18(4):2351-2367. Epub 2021 Oct 18.

Neurochemistry Lab, Department of Clinical Chemistry, Amsterdam Neuroscience, Amsterdam UMC, Vrije Universiteit, Amsterdam, the Netherlands.

Reliable and responsive tools for monitoring disease activity and treatment outcomes in patients with neuropathies are lacking. With the emergence of ultrasensitive blood bioassays, proteins released with nerve damage are potentially useful response biomarkers for many neurological disorders, including polyneuropathies. In this review, we provide an overview of the existing literature focusing on potential applications in polyneuropathy clinical care and trials. Read More

View Article and Full-Text PDF
October 2021

Cryoglobulinemic Vasculitic Mononeuritis Multiplex in a Teenager.

Pediatr Neurol 2021 11 23;124:1-2. Epub 2021 Jul 23.

Assistant Professor, Department of Pathology, The University of Texas at Austin, Dell Medical School, Dell Children's Medical School, Austin, Texas.

View Article and Full-Text PDF
November 2021

Multi-targeted therapy for refractory eosinophilic granulomatosis with polyangiitis characterized by intracerebral hemorrhage and cardiomyopathy: a case-based review.

Rheumatol Int 2021 Jul 21. Epub 2021 Jul 21.

Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

Eosinophilic granulomatosis with polyangiitis (EGPA) is a systemic autoimmune disorder classified under anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis, predominantly affecting small- to medium-sized vessels, characterized by asthma, eosinophilia, and necrotizing granulomatous inflammation. Most patients with EGPA experience peripheral neuropathy, whereas intracerebral hemorrhage is rare as EGPA-related presentation in central nervous system involvement, causing severe morbidity and mortality. Here, we present a 45-year-old man with refractory EGPA who developed intracerebral hemorrhage as the first manifestation, followed by cardiac involvement. Read More

View Article and Full-Text PDF

Vision loss in giant cell arteritis.

Pract Neurol 2022 Apr 8;22(2):138-140. Epub 2021 Jul 8.

Department of Ophthalmology and Vision Science, University of Toronto Faculty of Medicine, Toronto, Ontario, Canada

Almost two-thirds of patients with giant cell arteritis (GCA) develop ocular symptoms and up to 30% suffer permanent visual loss. We review the three most common mechanisms for visual loss in GCA, describing the relevant ophthalmic arterial anatomy and emphasising how ophthalmoscopy holds the key to a rapid diagnosis. The short posterior ciliary arteries supply the optic nerve head, while the central retinal artery and its branches supply the inner retina. Read More

View Article and Full-Text PDF

Vasculitic peripheral neuropathy in deficiency of adenosine deaminase 2.

Neuromuscul Disord 2021 09 14;31(9):891-895. Epub 2021 May 14.

Neurology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.

Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive inflammatory vasculopathy characterized by systemic vasculitis, early-onset stroke and livedo racemosa. We report a family cohort of 3 patients with ADA2 compound heterozygous mutation p.[Thr360Ala] and [Gly383Ser]. Read More

View Article and Full-Text PDF
September 2021

Vasculitis Presenting as Autonomic Failure.

Neurologist 2021 Jul 6;26(4):117-121. Epub 2021 Jul 6.

Departments of Neurology.

Introduction: Granulomatosis with polyangiitis (GPA) is a vasculitic process that can cause neurological dysfunction in addition to characteristic sinus and pulmonary manifestations. This case report highlights the spectrum of nervous system manifestations and includes rarely reported autonomic and pituitary involvement.

Case Report: A 62-year-old woman presented with orthostatic intolerance, tachycardia, dry mouth, and temperature sensitivity; subsequent autonomic reflex study demonstrated widespread postganglionic sympathetic sudomotor, cardiovagal, and cardiovascular adrenergic impairment reflective of autonomic neuropathy and overall autonomic failure. Read More

View Article and Full-Text PDF

The role of vascular inflammation markers in deficiency of adenosine deaminase 2.

Semin Arthritis Rheum 2021 08 28;51(4):839-844. Epub 2021 Apr 28.

Department of Pediatrics, Division of Rheumatology, Hacettepe University School of Medicine, Ankara, Turkey; Pediatric Rheumatology Unit, Translational Medicine Laboratories, Hacettepe University, Ankara, Turkey. Electronic address:

Objectives: The first objective was to assess the role of vascular inflammatory factors in the pathogenesis of deficiency of adenosine deaminase 2 (DADA2) and to compare these markers among DADA2 patients with different phenotypes. We also aimed to investigate differences between DADA2 patients with vasculitic features and classic polyarteritis nodosa (PAN) for the aforementioned markers.

Methods: The study included eighteen DADA2 patients, ten PAN patients, and eight healthy controls. Read More

View Article and Full-Text PDF

Nonsystemic vasculitic neuropathy: Presentation and long-term outcome from a French cohort of 50 patients.

Autoimmun Rev 2021 Aug 9;20(8):102874. Epub 2021 Jun 9.

Department of Neurology, Hôpital Henri Mondor, Créteil, France; Department of Internal Medicine, National Referral Center for Systemic and Autoimmune Diseases, Hôpital Cochin, Paris. Electronic address:

View Article and Full-Text PDF

Vasculitic neuropathy associated with IgG4-related kidney disease: A case report and literature review.

Clin Nephrol 2021 Sep;96(3):175-179

IgG4-related disease is an immune-mediated systemic inflammatory condition characterized by tissue infiltration of IgG4-positive plasma cells and elevated serum IgG4 concentrations. Peripheral neuropathy is an atypical manifestation of this disease. We describe an unusual case of vasculitic neuropathy in a patient with IgG4-related kidney disease. Read More

View Article and Full-Text PDF
September 2021

Efficacy of a fixed combination of palmitoylethanolamide and acetyl-l-carnitine (PEA+ALC FC) in the treatment of neuropathies secondary to rheumatic diseases.

Minerva Med 2021 Aug 31;112(4):492-499. Epub 2021 May 31.

Unit of Rheumatology, Città della Salute e della Scienza, Turin, Italy.

Background: The neurologic complications of rheumatic diseases (RDs) are highly variable, and their manifestations are linked to the pathogenesis and clinical phenotype of the specific RDs. In rheumatoid arthritis, for example, the peripheral nervous system is most commonly involved and mononeuritis multiplex, nerve entrapment and vasculitic sensorimotor neuropathies are not uncommon. Often the therapy for these disorders is not easy and is characterized by the use of different drugs. Read More

View Article and Full-Text PDF

Immune Axonal Neuropathies Associated With Systemic Autoimmune Rheumatic Diseases.

Front Pharmacol 2021 14;12:610585. Epub 2021 Apr 14.

Colentina-Research and Development Center, Colentina Clinical Hospital, Bucharest, Romania.

Immune axonal neuropathies are a particular group of immune-mediated neuropathies that occasionally accompany systemic autoimmune rheumatic diseases such as connective tissue dissorders and primary systemic vasculitides. Apart from vasculitis of vasa nervorum, various other mechanisms are involved in their pathogenesis, with possible therapeutic implications. Immune axonal neuropathies have highly heterogeneous clinical presentation and course, ranging from mild chronic distal sensorimotor polyneuropathy to severe subacute mononeuritis multiplex with rapid progression and constitutional symptoms such as fever, malaise, weight loss and night sweats, underpinning a vasculitic process. Read More

View Article and Full-Text PDF

The Effect of Lutein on Ischemia-reperfusion-induced Vasculitic Neuropathic Pain and Neuropathy in Rats.

In Vivo 2021 May-Jun;35(3):1537-1543

Department of Neurosurgery, Adatip Hospital, Istanbul, Turkey

Background/aim: Neuropathic pain and neuropathy is commonly seen after ischemia-reperfusion injuries. Our aim was to evaluate the effect of lutein on ischemia-reperfusion (I/R)-induced vasculitic neuropathic pain and neuropathy in rats.

Materials And Methods: An hour before anesthesia, 6 Albino Wistar male rats with I/R were orally administered with 1 mg/kg lutein (LIR group). Read More

View Article and Full-Text PDF

Chronic Immune-Mediated Polyneuropathies.

Clin Geriatr Med 2021 05 23;37(2):327-345. Epub 2021 Mar 23.

Department of Neurology, Virginia Commonwealth University, 1101 East Marshall Street, PO Box 980599, Richmond, VA 23298, USA. Electronic address:

This article discusses the chronic immune-mediated polyneuropathies, a broad category of acquired polyneuropathies that encompasses chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), the most common immune-mediated neuropathy, the CIDP variants, and the vasculitic neuropathies. Polyneuropathies associated with rheumatological diseases and systemic inflammatory diseases, such as sarcoidosis, will also be briefly covered. These patients' history, examination, serum studies, and electrodiagnostic studies, as well as histopathological findings in the case of vasculitis, confirm the diagnosis and differentiate them from the more common length-dependent polyneuropathies. Read More

View Article and Full-Text PDF

Multiple Myeloma and Vasculitic Neuropathy: An Unusual Presentation.

Cureus 2021 Mar 9;13(3):e13776. Epub 2021 Mar 9.

Internal Medicine, Nepalese Army Institute of Health Sciences, Kathmandu, NPL.

Multiple myeloma (MM) is a clonal proliferation of plasma cells in the bone marrow resulting in the production of paraproteins. It is more common in elderly adults and presents with nonspecific symptoms like bone pain, pathological fracture, fatigue, and signs of hypercalcemia. Peripheral neuropathy is an atypical presentation. Read More

View Article and Full-Text PDF

Peripheral nervous system manifestations of rheumatological diseases.

J Neurol Sci 2021 05 27;424:117421. Epub 2021 Mar 27.

University of Virginia, Department of Neurology, Charlottesville, VA, USA.

Rheumatological diseases result in immune-mediated injury to not only connective tissue, but often components of the peripheral nervous system. These overlap conditions can be broadly categorized as peripheral neuropathies and overlap myositis. The peripheral neuropathies are distinctive as many have unusual presentations such as non-length-dependent, small fiber neuropathies and sensory neuronopathies (both due to dorsal root ganglia dysfunction), multiple mononeuropathies (e. Read More

View Article and Full-Text PDF