Vasculitic Neuropathies

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.

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Peripheral neuropathy in antineutrophil cytoplasmic antibody-associated vasculitides

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000615 ◽

2019 ◽

Vol 6 (6) ◽

pp. e615 ◽

Cited By ~ 4

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.

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Vasculitis and the peripheral nervous system

Rheumatology ◽

10.1093/rheumatology/keaa075 ◽

2020 ◽

Vol 59 (Supplement_3) ◽

pp. iii55-iii59 ◽

Cited By ~ 2

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.

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Case 27

10.1093/med/9780190603434.003.0031 ◽

2018 ◽

Author(s):

Bashar Katirji

Keyword(s):

Differential Diagnosis ◽

Connective Tissue ◽

Peripheral Nerves ◽

Pathological Findings ◽

Mononeuritis Multiplex ◽

Vasculitic Neuropathy ◽

Peripheral Polyneuropathy ◽

Pathologic Findings ◽

Electrophysiological Findings ◽

Nonsystemic Vasculitic Neuropathy

Mononeuropathy multiplex is a disorder which distinguishes itself from peripheral polyneuropathy by involvement of specific individual peripheral nerves. Mononeuropathy multiplex is often asymmetrical and is classified, based on electrophysiologic and pathologic findings, into axonal and demyelinating types. Mononeuritis multiplex is a term that is used exclusively for cases of axonal multiple mononeuropathies due to peripheral nerve vasculitis. This may be associated with connective tissue disease or hepatitis B and C infection. This case outlines the clinical and electrophysiological findings in mononeuritis multiplex and discusses the differential diagnosis of systemic and nonsystemic vasculitic neuropathy. The causes and pathological findings that characterize vasculitic neuropathy are highlighted.

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

Terapevticheskii arkhiv ◽

10.26442/00403660.2019.12.000206 ◽

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.

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Faculty Opinions recommendation of Idiosyncratic drug-induced liver injury is associated with substantial morbidity and mortality within 6 months from onset.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.718329507.793496583 ◽

2014 ◽

Author(s):

Bruno Stieger

Keyword(s):

Liver Injury ◽

Morbidity And Mortality ◽

Drug Induced ◽

Drug Induced Liver Injury ◽

Substantial Morbidity

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Manage drug-induced parkinsonism through early recognition of the condition and discontinuation of the causative agent

Drugs & Therapy Perspectives ◽

10.1007/bf03262145 ◽

2012 ◽

Vol 28 (12) ◽

pp. 20-23

Keyword(s):

Causative Agent ◽

Early Recognition ◽

Drug Induced

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Infected Renal Cyst as a Complication of Dropped Gallstones during Laparoscopic Cholecystectomy

Case Reports in Gastrointestinal Medicine ◽

10.1155/2018/2478245 ◽

2018 ◽

Vol 2018 ◽

pp. 1-5

Author(s):

Chelsea Kennedy-Snodgrass ◽

Vivian Keenan ◽

Douglas S. Katz

Keyword(s):

Risk Factors ◽

Laparoscopic Cholecystectomy ◽

Ct Scan ◽

Renal Cyst ◽

Early Recognition ◽

Common Complication ◽

Abdominal Ct ◽

Abdominal Ct Scan ◽

Substantial Morbidity

Dropped gallstones are a relatively common complication, occurring in 3% to 32% of laparoscopic cholecystectomies performed, depending on various intraoperative risk factors. However, complications arising from dropped gallstones are relatively rare, occurring in fewer than 1% of such patients, and can include abscesses and inflammatory masses confined to the subhepatic space, presenting days to years later. We report a patient who developed an infected renal cyst as a result of dropped gallstones, which created a fistula from the duodenum to a previously simple right renal cyst, which was initially identified on an abdominal CT scan. Dropped gallstones can result in substantial morbidity in a minority of patients following cholecystectomy performed for cholecystitis, and a high clinical as well as radiological index of suspicion may be required for accurate early recognition and treatment.

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Drug Induced Steatohepatitis: An Uncommon Culprit of a Common Disease

BioMed Research International ◽

10.1155/2015/168905 ◽

2015 ◽

Vol 2015 ◽

pp. 1-14 ◽

Cited By ~ 44

Author(s):

Liane Rabinowich ◽

Oren Shibolet

Keyword(s):

Metabolic Syndrome ◽

Liver Disease ◽

Liver Injury ◽

Hepatic Steatosis ◽

Early Recognition ◽

Developed Countries ◽

Common Disease ◽

Drug Induced ◽

Altered Expression ◽

The Metabolic Syndrome

Nonalcoholic fatty liver disease (NAFLD) is a leading cause of liver disease in developed countries. Its frequency is increasing in the general population mostly due to the widespread occurrence of obesity and the metabolic syndrome. Although drugs and dietary supplements are viewed as a major cause of acute liver injury, drug induced steatosis and steatohepatitis are considered a rare form of drug induced liver injury (DILI). The complex mechanism leading to hepatic steatosis caused by commonly used drugs such as amiodarone, methotrexate, tamoxifen, valproic acid, glucocorticoids, and others is not fully understood. It relates not only to induction of the metabolic syndrome by some drugs but also to their impact on important molecular pathways including increased hepatocytes lipogenesis, decreased secretion of fatty acids, and interruption of mitochondrialβ-oxidation as well as altered expression of genes responsible for drug metabolism. Better familiarity with this type of liver injury is important for early recognition of drug hepatotoxicity and crucial for preventing severe forms of liver injury and cirrhosis. Moreover, understanding the mechanisms leading to drug induced hepatic steatosis may provide much needed clues to the mechanism and potential prevention of the more common form of metabolic steatohepatitis.

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