scholarly journals Vasculitis and the peripheral nervous system

Rheumatology ◽  
2020 ◽  
Vol 59 (Supplement_3) ◽  
pp. iii55-iii59 ◽  
Author(s):  
Lionel Ginsberg
Keyword(s):  
Lower Limb ◽  
Vessel Wall ◽  
Early Recognition ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Chronic Neuropathic Pain ◽  
Vasculitic Neuropathy ◽  
Vasa Nervorum ◽  
Sensory Recovery ◽  
Systemic Process

Abstract Peripheral neuropathy is a common feature of systemic vasculitis and can also occur when vessel wall inflammation is confined to the vasa nervorum, as a tissue-specific condition—non-systemic vasculitic neuropathy (NSVN). Typically, the clinical picture in both systemic and non-systemic cases is of a lower limb predominant, distal, asymmetric or multifocal neuropathy, which is painful and subacute in onset. For NSVN, nerve biopsy is required to make the diagnosis, and nerve biopsy also has a role when vasculitic neuropathy is suspected and a systemic process has not yet declared itself. Early recognition of the disorder is important, because it is treatable, and without treatment potentially disabling, or even lethal if part of an undiagnosed systemic process. Treatment is generally with combination therapy (glucocorticoid plus other immunosuppressant), after which motor and sensory recovery are likely to occur, albeit slowly, but the patient may be left with chronic neuropathic pain.

scholarly journals Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

2019 ◽  
Vol 6 (6) ◽  
pp. e615 ◽  
Author(s):  
Antje Bischof ◽  
Veronika K. Jaeger ◽  
Robert D. M. Hadden ◽  
Raashid A. Luqmani ◽  
Anne-Katrin Pröbstel ◽  
...  
Keyword(s):  
Granulomatosis With Polyangiitis ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Eosinophilic Granulomatosis With Polyangiitis ◽  
Vasculitic Neuropathy ◽  
Diagnostic Certainty ◽  
Primary Systemic Vasculitis ◽  
Organ Manifestations ◽  
Eosinophilic Granulomatosis

ObjectiveReported prevalence of vasculitic neuropathy (VN) in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is highly variable, and associations with other organ manifestations have not been studied systematically while accounting for diagnostic certainty of VN.MethodsData of all patients with AAV within the Diagnostic and Classification criteria for primary systemic VASculitis study were analyzed cross-sectionally. VN was categorized as definite (histology proven), probable (multiple mononeuropathy or nerve biopsy consistent with vasculitis), or possible (all others). Associations with other organ manifestations were compared in patients with and without VN.ResultsNine hundred fifty-five patients (mean age 57 years, range 18–91 years, 51% female) were identified. Of these, 572 had granulomatosis with polyangiitis (GPA), 218 microscopic polyangiitis (MPA), and 165 eosinophilic granulomatosis with polyangiitis (EGPA). The prevalence of VN was 65% in EGPA, 23% in MPA, and 19% in GPA. Nerve biopsy was performed in 32/269 (12%) patients, demonstrating definite vasculitis in 17/32 (53%) of patients. VN was associated with myeloperoxidase-ANCA positivity (p = 0.004) and skin (p < 0.001), musculoskeletal, (p < 0.001) and cardiovascular (p = 0.005) involvement. Patients with VN were less likely to have renal (p < 0.001), eye (p < 0.001), and gastrointestinal (p = 0.023) involvement.ConclusionsOur study provides comprehensive insights into the prevalence and organ associations of VN in a large, systematically collected AAV cohort. VN is most commonly associated with skin, musculoskeletal, and cardiovascular manifestations. In routine clinical practice, diagnosis of VN is infrequently confirmed by the gold standard of nerve biopsy but rather supported by the clinical setting of active systemic AAV.

scholarly journals Peripheral nervous system lesion in systemic vasculitis - issues of diagnosis and treatment

2019 ◽  
Vol 91 (12) ◽  
pp. 63-69
Author(s):  
I Yu Golovach ◽  
Ye D Yehudina
Keyword(s):  
Blood Vessel ◽  
Lupus Erythematosus ◽  
Peripheral Nerves ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Antineutrophil Cytoplasmic Antibody ◽  
Underlying Disease ◽  
Pulse Therapy ◽  
Vasculitic Neuropathy ◽  
Viral Hepatitis C

Vasculitis is a clinically diverse group of diseases with histopathological signs of blood vessel inflammation, which contributes to vascular damage and ischemic damage to the affected tissues. Vasculitic neuropathy is a common complication of the primary systemic vasculitides, such as polyartertis nodosa and antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis, systemic diseases of the connective tissue - systemic lupus erythematosus and Sjogren syndrome, vasculitis associated with infection, most often viral hepatitis C and B and non - systemic vasculitis neuropathy. Vessels of medium and small caliber are involved in the pathological process in these diseases. With all vasculitis, except for those caused by the direct effect of the infectious trigger on the blood vessel walls, the main pathogenetic mechanism is an autoimmune process with the development of vasa nervorum vasculitis - small arteries and vessels that supply peripheral nerves, and the outcome - nerve ischemia. The classic clinical presentation is an acute or subacute painful multifocal neuropathy that has a predilection for the lower extremities, affects two or more named nerves, and progresses in a step wise manner. However, vasculitic neuropathy can manifest in a variety of ways, including asymmetric polyneuropathies and distal symmetric sensory neuropathies, and it also can be slowly progressive, particularly in cases of nonsystemic vasculitic neuropathy (NSVN), a form of vasculitis that clinically remains restricted to peripheral nerves. Nerve biopsy can help establish the diagnosis of a systemic vasculitis, particularly when other organ involvement is not clinically apparent, and is required for diagnosis of NSVN. Neuropathy due to systemic vasculitis should be treated in accordance with the recommendations for the treatment of the underlying disease. In NSVH, the main medicine of choice are glucocrticoids, and in severe/progressive cases, pulse therapy with cyclophosphamide.

Vasculitic Neuropathies

2019 ◽  
Vol 39 (05) ◽  
pp. 608-619 ◽  
Author(s):  
Nathaniel Beachy ◽  
Kelsey Satkowiak ◽  
Kelly Graham Gwathmey
Keyword(s):  
Peripheral Nerves ◽  
Early Recognition ◽  
Systemic Vasculitis ◽  
Vascular Supply ◽  
Connective Tissue Disorders ◽  
Drug Induced ◽  
Vasculitic Neuropathy ◽  
Primary Systemic Vasculitis ◽  
Substantial Morbidity ◽  
Nonsystemic Vasculitic Neuropathy

AbstractVasculitic neuropathies are disorders that result from inflammation in the peripheral nerves' vascular supply, resulting in ischemic injury. These disorders may be a result of systemic inflammation or may be confined to the peripheral nervous system. Causative etiologies include primary systemic vasculitis, vasculitis secondary to other conditions such as primary connective tissue disorders, infectious, paraneoplastic, and drug-induced conditions, and nonsystemic vasculitic neuropathy. Early recognition and treatment of these conditions is imperative to prevent substantial morbidity and mortality. The goal of this review is to provide an organization of the vasculitic neuropathies and an overview of principles of diagnosis and treatment for the clinical neurologist.

scholarly journals An update on vessel preparation in lower limb arterial intervention

2020 ◽  
Vol 3 (1) ◽  
Author(s):  
William Ormiston ◽  
Shelagh Dyer-Hartnett ◽  
Rukshan Fernando ◽  
Andrew Holden
Keyword(s):  
Lower Limb ◽  
Vessel Wall ◽  
Arterial Intervention ◽  
Arterial Disease ◽  
Long Term Outcomes ◽  
Pulsatile Pressure ◽  
Short And Long Term ◽  
Late Restenosis ◽  
Vessel Preparation

Abstract Background Plain balloon angioplasty has traditionally been used to treat lower limb arterial disease but can be limited by significant residual stenosis, vessel recoil, dissection, and by late restenosis. Appropriate vessel preparation may significantly improve short and long-term outcomes. We aim to give an overview of some of the devices currently available, or under investigation, for vessel preparation in the lower limb. Main text Vessel preparation devices include those that remove plaque (atherectomy devices) and those that modify plaque. The four groups of plaque removing atherectomy devices are defined by their plaque removal method: Directional, rotational orbital and excimer laser are categories of devices investigated for plaque modification. Intravascular lithotripsy devices generate sonic pulsatile pressure waves that pass into the vessel wall cracking calcified plaques whilst sparing soft tissue. This enables dilatation of calcified lesions at low pressure by conventional balloons and enables full stent expansion. Other balloon based vessel preparation devices were designed to modify plaque and produce more controlled, lower pressure luminal expansion without major dissections and potentially with less recoil than conventional angioplasty balloons. Scoring balloons have a helical nitinol element attached to the balloon that scores plaque facilitating uniform luminal enlargement. Further specialty balloons have been developed in recent years, including the Chocolate, Phoenix and Serranator balloons. Finally, the temporary Spur self-expanding retrievable nitinol stent has a series of radially aligned spurs that are driven into the vessel wall by post-dilatation, potentially improving drug delivery. Conclusion Lesion specific vessel preparation aims to improve both short and long term outcomes through improved penetration of anti-proliferative drug, maximising luminal gain, reducing the need for stent placement and minimising intimal injury. Some forms of vessel preparation appear to improve short term outcomes; long-term outcomes remain uncertain. An overview of some of the multiple devices available for vessel preparation is presented.

Deep dissecting haematoma in patients with dermatoporosis: implications for home nursing

2021 ◽  
Vol 26 (Sup3) ◽  
pp. S6-S13
Author(s):  
Valentina Vanzi ◽  
Elena Toma
Keyword(s):  
Wound Healing ◽  
Emergency Management ◽  
Lower Limb ◽  
Essential Role ◽  
Early Recognition ◽  
Wound Management ◽  
Home Nursing ◽  
High Prevalence ◽  
Clinical Emergency

Dermatoporosis is a chronic cutaneous insufficiency/fragility syndrome with a high prevalence in older adults. Dermatoporotic skin becomes thin and fragile and tends to tear. It may lead to deep dissecting haematomas (DDHs) as a final stage of dermatoporosis, which is a clinical emergency. Management can be challenging, as patients with lower-limb haematomas are frequently older and affected by multiple comorbidities, or are probably on medications that negatively influence wound healing. This article describes the essential role of nurses in prevention, early recognition and wound management of DDHs in patients with dermatoporosis.

scholarly journals Peripheral nerve biopsy: Is it still important for the early diagnosis of neural leprosy?

2021 ◽  
Author(s):  
Isabella Sabião Borges ◽  
João Victor Aguiar Moreira ◽  
Thales Junqueira Oliveira ◽  
Maria Fernanda Prado Rosa ◽  
Gabriel Nunes Melo Assunção ◽  
...  
Keyword(s):  
Peripheral Neuropathy ◽  
Early Diagnosis ◽  
Peripheral Nerve ◽  
Clinical Evidence ◽  
Early Recognition ◽  
Diagnostic Errors ◽  
Nerve Biopsy ◽  
Skin Lesions ◽  
National Reference Center ◽  
Neural Impairment

Background: The early recognition of neural impairment in leprosy represents a challenge in clinical practice and peripheral nerve biopsy may be required for diagnostic. Objective: Characterize the epidemiological, clinical, electroneuromyographic, laboratory and histopathological aspects of patients undergoing peripheral nerve biopsy during investigation of primary neural leprosy. Methods: 104 patients with peripheral neuropathy, referred to a national reference center leprosy, were biopsied. All patients had clinical evidence of peripheral neuropathy associated with the absence of skin lesions and were being investigated. Results: Of 104 biopsied, leprosy was confirmed in 89.4%. 66 were classified as primary neural leprosy and 27 as neural relapse or reinfection. All cases confirmed presented asymmetric neural impairment with predominance of sensory symptoms (88.2%), followed by muscular weakness and/or amyotrophy in 44.1% and pain in 34.4%. Neural thickening of one or more nerves was observed in 78.5% of the patients. The biopsied nerves were: ulnar (67.8%), superficial fibular (21.5%), sural (8.6%), radial (1.1%) and deep fibular (1.1%). 29% presented histopathological abnormalities and 4.4% acid fast bacilli. Nerve and superjacent skin qPCR were positive in 49.5% and 24.8% of cases, respectively. The patients with multiple mononeuropathy presented higher frequency of neural thickening (p<0.0001) and histopathological abnormalities (p=0.0077), but lower rates of positivity of ELISA anti-PGL-I (p=0.0100), qPCR in the peripheral blood (p=0.0157), and in the slit skin smear (p=0.0032). Conclusions: Peripheral nerve biopsy is an important tool in the investigation of primary neural cases, contributing to the early diagnosis and reducing diagnostic errors and the need for empirical treatment.

Pathology of Peripheral Nerve

2013 ◽  
pp. 313-342
Author(s):  
Jean-Michel Vallat ◽  
Douglas Anthony ◽  
Umberto De Girolami
Keyword(s):  
Peripheral Nerve ◽  
Systemic Vasculitis ◽  
Nerve Biopsy ◽  
Normal Morphology ◽  
Hematological Diseases ◽  
Hereditary Neuropathies ◽  
Classification Of Diseases ◽  
Inflammatory Neuropathies ◽  
A Current

This chapter gives a current classification of diseases of peripheral nerve and then describes and illustrates the pathology of peripheral nerve, particularly from the perspective of the nerve biopsy. After a description of the clinical indications for the biopsy, an introduction to the general laboratory techniques, and a brief review of the normal morphology of peripheral nerve, the general reactions of peripheral nerve to injury (primary axonal degeneration and primary segmental demyelination) are described. Neuropathies can be separated into acquired and hereditary neuropathies. Inflammatory neuropathies include immunopathological disorders of unknown cause (e.g. inflammatory demyelinating polyradiculoneuropathy), neuropathies due to infectious agents (e.g. leprosy), or those associated with systemic vasculitis. Neuropathy also occurs secondarily, in association with hematological diseases and neoplasms. Metabolic (diabetic) and toxic neuropathies are subsequently discussed. The chapter also gives an account of the importance of incorporating recent advances in molecular genetics in the evaluation of hereditary neuropathies (i.e. hereditary motor and sensory neuropathies, hereditary sensory and autonomic neuropathies, and familial amyloid polyneuropathies).

A 55-Year-Old Female with a History of Right Foot Drop and Left Hand Weakness

2016 ◽  
Author(s):  
Jeffrey A. Cohen ◽  
Justin J. Mowchun ◽  
Victoria H. Lawson ◽  
Nathaniel M. Robbins
Keyword(s):  
Systemic Vasculitis ◽  
Immunosuppressive Treatment ◽  
Abrupt Onset ◽  
Conclusive Evidence ◽  
Foot Drop ◽  
Mononeuritis Multiplex ◽  
Vasculitic Neuropathy ◽  
Left Hand ◽  
Serologic Markers ◽  
History Of

Vasculitic neuropathy often presents as a mononeuritis multiplex pattern. Ischemic nerve injury can lead to abrupt-onset, painful, and multifocal sensorimotor neuropathy. This chapter emphasizes the diagnostic considerations of vasculitic neuropathy, which includes the significant limitations of serologic markers in non-systemic vasculitic neuropathy. Nerve and muscle biopsy are important investigations to consider to make the diagnosis. Keys to management are also reviewed. It is important to manage systemic vasculitis with a rheumatologist. Nonsystemic vasculitis has a much better prognosis; immunosuppressive treatment is less aggressive, but it is recommended to have a rheumatologist’s input. There is no conclusive evidence on how to treat nonsystemic vasculitis. Mild cases may be treated with steroids alone.

015 Predicting a diagnosis of pathologically confirmed vasculitic neuropathy

2018 ◽  
Vol 89 (6) ◽  
pp. A7.2-A7
Author(s):  
Dev Nathani ◽  
Michael H Barnett ◽  
Judith Spies ◽  
John Pollard ◽  
Matthew C Kiernan
Keyword(s):  
Gold Standard ◽  
Clinical Laboratory ◽  
Axonal Neuropathy ◽  
Invasive Procedure ◽  
Nerve Biopsy ◽  
Nerve Conduction Studies ◽  
Vasculitic Neuropathy ◽  
Severity Of The Disease ◽  
Time Of Presentation ◽  
Sensitive Parameters

IntroductionNerve biopsy remains the gold standard to diagnose vasculitic neuropathy. Conversely, biopsy has imperfect sensitivity and entails risks associated with an invasive procedure. Methods to improve diagnostic accuracy remain important considerations given the severity of the disease, added with the risks associated with subsequent therapy, particularly in ill-defined cases.MethodsClinical, laboratory and neurophysiological parameters were analysed for all patients who subsequently underwent biopsy. Nerve and muscle biopsy reports were assigned pathologic categories of definite, probable, possible or absent vasculitis using standard guidelines. Correlations were assessed between pre-biopsy parameters and subsequent diagnosis of definite or probable vasculitic neuropathy (pathologically confirmed vasculitis).ResultsFrom a cohort of 207 patients who underwent nerve biopsy over 21 months, 70 were suspected of having vasculitic neuropathy prior to biopsy. Of the 70 patients, vasculitis was confirmed as definite (11.4%), probable (15.7%) or possible (10.0%) on neuropathological assessment. The most sensitive parameters for pathologically confirmed vasculitis were the presence of sensorimotor neuropathy (78%) and axonal neuropathy (67%) on nerve conduction studies (NCS) in the overall cohort. Pathologically absent vasculitis was most prevalent in patients with normal NCS (90%), chronic, symmetric symptoms (86%) and demyelinating findings on NCS (79%). The parameters with the strongest associations with pathologically confirmed vasculitis were positive autoantibody serology (50.0% vs 21.1%, p<0.01), anti-neutrophil cytoplasmic antibody (27.3% vs 6.4%, p<0.01), anti-myeloperoxidase antibody (22.7% vs 1.8%, p<0.005) and rheumatoid factor (22.7% vs 2.8%, p<0.005). In patients suspected to have vasculitis, 83.3% of anti-myeloperoxidase antibody positive patients had pathologically confirmed vasculitis. In patients not suspected to have vasculitis, acute symptoms had the strongest association with pathologically confirmed vasculitis (36.4% vs 10.5%, p<0.05).ConclusionSpecific characteristics of symptoms at the time of presentation, combined with the presence of autoantibodies and neurophysiological abnormalities, were predictive of a tissue diagnosis of vasculitic neuropathy.

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