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Central and Peripheral Nervous System Complications of Vasculitis Syndromes from Pathology to Bedside: Part 2—Peripheral Nervous System

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Abstract

Purpose of Review

Peripheral nervous system vasculitides (PNSV) are a heterogeneous group of disorders with a clinical subset that may differ in prognosis and therapy. We provide a comprehensive update on the clinical assessment, diagnosis, complications, treatment, and follow-up of PNSV.

Recent Findings

Progress in neuroimaging, molecular testing, and peripheral nerve biopsy has improved clinical assessment and decision-making of PNSV, also providing novel insights on how to prevent misdiagnosis and increase diagnostic certainty. Advances in imaging techniques, allowing to clearly display the vessel walls, have also enhanced the possibility to differentiate inflammatory from non-inflammatory vascular lesions, while recent histopathology data have identified the main morphological criteria for more accurate diagnosis and differential diagnoses. Overall, the identification of peculiar morphological findings tends to improve diagnostic accuracy by defining a clearer boundary between systemic and non-systemic neuropathies. Therefore, the definition of epineurium vessel wall damage, type of vascular lesion, characterization of lymphocyte populations, antibodies, and inflammatory factors, as well as the identification of direct nerve damage or degeneration, are the common goals for pathologists and clinicians, who will both benefit for data integration and findings translation. Nevertheless, to date, treatment is still largely empiric and, in some cases, unsatisfactory, thus often precluding precise prognostic prediction. In this context, new diagnostic techniques and multidisciplinary management will be essential in the proper diagnosis and prompt management of PNSV, as highlighted in the present review.

Summary

Thirty to fifty percent of all patients with vasculitis have signs of polyneuropathy. Neuropathies associated with systemic vasculitis are best managed according to the guidelines of the underlying disease because appropriate workup and initiation of treatment can reduce morbidity. Steroids, or in severe or progressive cases, cyclophosphamide pulse therapy is the standard therapy in non-systemic vasculitic neuropathies. Some patients need long-term immunosuppression. The use of novel technologies for high-throughput genotyping will permit to determine the genetic influence of related phenotypes in patients with PNSV.

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Authors and Affiliations

  1. Department of Advanced Medical and Surgical Sciences (DAMSS), University of Campania “Luigi Vanvitelli”, Piazza L. Miraglia 2, 80138, Naples, Italy

    Gelsomina Mansueto

  2. Clinical Department of Laboratory Services and Public Health—Legal Medicine Unit, University of Campania “Luigi Vanvitelli”, Via Luciano Armanni 5, 80138, Naples, Italy

    Gelsomina Mansueto

  3. Pathology-Unit of Federico II University, Via S. Pansini 3, 80131, Naples, Italy

    Gelsomina Mansueto

  4. Department of Surgery and Medical-Surgical Specialties, University of Catania, Via Santa Sofia 78, 95123, Catania, Italy

    Giuseppe Lanza

  5. Clinical Neurophysiology Research Unit, Oasi Research Institute-IRCCS, Via Conte Ruggero 73, 94018, Troina, Italy

    Giuseppe Lanza

  6. Pathology Unit, Sant’Anna E San Sebastiano Hospital, 81100, Caserta, Italy

    Jessica Falleti & Pasquale Orabona

  7. School of Medicine, University of New Mexico, Albuquerque, NM, USA

    Danielle Alaouieh & Emily Hong

  8. Neurological Service, SS Annunziata Hospital, Viale Mazzini 100, 67039, Sulmona, L’Aquila, Italy

    Sara Girolami & Mario Di Napoli

  9. Mental and Physical Health and Preventive Medicine Department, University of Campania “Luigi Vanvitelli”, Via Luciano Armanni 5, 80138, Naples, Italy

    Marco Montella

  10. Department of Biomedical and Biotechnological Sciences, University of Catania, Via Santa Sofia 97, 95123, Catania, Italy

    Francesco Fisicaro

  11. AOU “Luigi Vanvitelli”, Via Santa Maria Di Costantinopoli 104, 80138, Naples, Italy

    Anna Galdieri

  12. Department of Human Genetics, Punjabi University, Patiala, 147002, Punjab, India

    Puneetpal Singh

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Gelsomina Mansueto, Giuseppe Lanza, Jessica Falleti, Pasquale Orabona, Danielle Alaouieh, Emily Hong, Sara Girolami, Marco Montella, Francesco Fisicaro, Anna Galdieri, Puneetpal Singh, and Mario Di Napoli each declare no potential conflicts of interest.

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Mansueto, G., Lanza, G., Falleti, J. et al. Central and Peripheral Nervous System Complications of Vasculitis Syndromes from Pathology to Bedside: Part 2—Peripheral Nervous System. Curr Neurol Neurosci Rep 23, 83–107 (2023). https://doi.org/10.1007/s11910-023-01249-3

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