ANCA-Associated Vasculitic Neuropathies: A Review

Koike, Haruki; Nishi, Ryoji; Ohyama, Ken; Morozumi, Saori; Kawagashira, Yuichi; Furukawa, Soma; Mouri, Naohiro; Fukami, Yasuyuki; Iijima, Masahiro; Sobue, Gen; Katsuno, Masahisa

doi:10.1007/s40120-021-00315-7

Cited by 27 publications

(29 citation statements)

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“…Although the pathogenesis of ANCA-associated vasculitis is complex, it is widely accepted that ANCA plays an important role in the mechanisms of vasculitis through interactions between neutrophils and vascular endothelial cells. 10,11 In one study, passive transfer of ANCA induced vasculitis in mice. 12 Although neutrophils do not interact with resting endothelium under physiologic conditions, the binding of ANCA promotes firm adhesion of neutrophils to the endothelium via adhesion molecules.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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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 ANCAassociated 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. Such neutrophilassociated lesions were not observed in patients with NSVN. Nonetheless, morphological changes in epineurial vascular endothelial cells, such as increases in cytoplasmic organelles and cytoplasmic protrusions into the vascular lumen, were observed in patients with NSVN. Since these findings were observed where light microscopy-based findings suggestive of vasculitis (e.g., the disruption of vascular structures and fibrinoid necrosis) were absent, they were considered early lesions that preceded the formation of the so-called necrotizing vasculitis. In conclusion, this study enabled the visualization of distinctive early ultrastructural lesions associated with ANCA and complement. Further studies are needed to elucidate the molecular basis of the induction of these fine structural changes, which will contribute to the development of targeted therapies based on specific mechanisms of vasculitis.

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Section: Discussionmentioning

confidence: 99%

“…Although the pathogenesis of ANCA‐associated vasculitis is complex, it is widely accepted that ANCA plays an important role in the mechanisms of vasculitis through interactions between neutrophils and vascular endothelial cells 10,11 . In one study, passive transfer of ANCA induced vasculitis in mice 12 .…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2022

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“…nosinusitis respectivamente 30,49 . Se observó en comparación con la literatura menor compromiso oftalmológico y cutáneo, probablemente debido a la selección en su mayoría de pacientes internados, es decir formas más graves 42 .…”

Section: Discussionunclassified

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Young¹

2022

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Las vasculitis asociadas a anticuerpos anticitoplasma de neutrófilos (ANCA) comprenden un subgrupo de afección de pequeños vasos que comparten en forma variable la presencia de ANCA. Se analizaron todos los pacientes con vasculitis asociadas a ANCA que fueron atendidos en el Hospital Británico de Buenos Aires durante los años 2000 a 2016 con el objetivo de describir la casuística de nuestro centro. Se incorporaron un total de 26 pacientes: 11 (42.31%) granulomatosis con poliangeítis (GPA), 6 (23.08%) poliangeítis microscópica (MPA) y vasculitis limitada al riñón (VLR) y 3 (11.54%) granulomatosis eosinofílica con poliangeítis (EGPA). La edad media al diagnóstico fue de 51.73±18.85 años (18-78) con predominio de sexo femenino (57.61%). El compromiso inicial más frecuente fue renal (73.08%), seguido por compromiso pulmonar (42.31%) y rinosinusal (26.92%). Cinco pacientes (19.23%) debutaron con síndrome pulmón-riñón y 6 (23.08%) presentaron afectación de más de dos órganos al inicio. La clasificación clínica más común fue la generalizada (34.62%), seguido por la severa (30.77%), con una media de BVAS de 16.6. El 92.31% presentaron determinaciones de ANCA positivas y 76.92% biopsia diagnóstica positiva. Los 26 pacientes realizaron tratamiento de inducción, la adición de ciclofosfamida a glucocorticoides fue la terapia dual más frecuente. En forma global el 50% de los pacientes que ingresaron al estudio lograron remisión, el 61.53% luego de la inducción inicial y el resto luego del tratamiento de la recaída. La mortalidad global fue de 15.38%, ocurriendo durante la inducción inicial, no reportándose eventos durante la remisión o recaída. Del análisis de nuestra serie se desprende que a pesar del compromiso clínico severo es posible reducir la mortalidad con los nuevos esquemas de tratamiento.

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“…The manifestations are wide-ranging, both those induced by eosinophilic non-vasculitis, such as asthma, rhinosinusitis, peripheral and tissue eosinophilia, and cardiomyopathy, and that induced by vasculitis, such as purpura, glomerulonephritis, and mononeuritis multiplex [3]. The prevalence of peripheral neuropathy in EGPA patients is reportedly between 46 and 77% [4,5]. Peripheral neuropathy in EGPA patients is characterized by polyneuropathy and mainly affects the lower extremities, with peroneal nerve involvement being the most frequent and severe [4].…”

Section: Introductionmentioning

confidence: 99%

“…Mepolizumab, an antiinterleukin-5 agent, was the first FDA-approved agent, and other biologic agents have since been developed for the treatment of EGPA [3]. Although most patients experience a reduction in pain and improvement in their strength within several weeks to months of the initiation of immunotherapies, some suffer from residual neuropathic symptoms for a long time, even after EGPA remission [5].…”

Section: Introductionmentioning

confidence: 99%

Successful treatment with spinal cord stimulation for pain due to eosinophilic granulomatosis with polyangiitis: a case report

et al. 2023

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Background Although most patients of eosinophilic granulomatosis with polyangiitis (EGPA) experience a reduction in pain within several weeks to months of the initiation of immunotherapies, some suffer from residual neuropathic symptoms for a long time. Case presentation A 28-year-old woman diagnosed with EGPA visited. She had been treated with steroid pulse therapy, intravenous immunoglobulin, and mepolizumab (antiinterleukin-5 agent). Her symptoms other than peripheral neuropathy improved, but posterior lower thigh pain and weakness of the lower legs worsened. At the initial visit, she used crutches and complained of numb pain in both posterior lower thighs, especially the left one. She also presented with left foot drop and reported a decreased tactile sensation on the lateral sides of both lower thighs. We performed spinal cord stimulation (SCS) at the L1 level on both sides. Her pain remarkably decreased, her tactile sensation improved, her muscle strength increased, and she was able to walk without crutches. Conclusions We herein report the first case of lower extremity pain being successfully treated with SCS in an EGPA patient who did not respond well to drug therapy. Because the cause of pain in EGPA is neuropathy induced by vasculitis, there is ample ability for SCS to improve this pain. When pain is neuropathic, whatever the cause, SCS may be worth trying, even for pain from disorders other than EGPA.

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ANCA-Associated Vasculitic Neuropathies: A Review

Cited by 27 publications

References 77 publications

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

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

Vasculitis asociadas a ANCA: análisis de 26 pacientes

Successful treatment with spinal cord stimulation for pain due to eosinophilic granulomatosis with polyangiitis: a case report

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