Поражение периферической нервной системы при системных васкулитах – вопросы диагностики и лечения

Головач И.Ю., Егудина Е.Д. Поражение периферической нервной системы при системных васкулитах – вопросы диагностики и лечения. Терапевтический архив. 2019; 91 (12): 63–69. DOI: 10.26442/00403660.2019.12.000206

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Golovach I.Yu., Yehudina Ye.D. Peripheral nervous system lesion in systemic vasculitis – issues of diagnosis and treatment. Therapeutic Archive. 2019; 91 (12): 63–69. DOI: 10.26442/00403660.2019.12.000206

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Аннотация

Васкулиты представляют собой клинически разнообразную группу заболеваний с гистопатологическими признаками воспаления кровеносных сосудов, которое способствует поражению сосудов и ишемическому повреждению пораженных тканей. Наиболее часто васкулитная нейропатия является осложнением таких первичных системных васкулитов, как узелковый полиартериит, и ассоциированных с антинейтрофильными цитоплазматическими антителами (AНЦA) васкулитов, системных заболеваний соединительной ткани – системной красной волчанки и синдрома Шегрена, васкулита, ассоциированного с инфекцией (чаще всего это вирусные гепатиты С и В) и несистемной васкулитной нейропатии (НСВН). При этих заболеваниях в патологический процесс вовлекаются сосуды среднего и малого калибра. При всех васкулитах, кроме тех, которые обусловлены непосредственным влиянием инфекционного триггера на стенки сосудов, основной патогенетический механизм – это аутоиммунный процесс с развитием васкулита vasa nervorum (мелких артерий и сосудов, которые кровоснабжают периферические нервы), а исход – ишемия нервов. Классическая клиническая картина ишемической нейропатии – это острая или подострая болезненная мультифокальная нейропатия с преимущественным поражением нервных структур нижних конечностей, поражающая два или более крупных нерва и прогрессирующая поэтапно. Однако васкулитная нейропатия может манифестировать различно, включая асимметричные полинейропатии и дистальные симметричные сенсорные нейропатии; кроме того, она может быть медленно прогрессирующий, особенно в случаях НСВН, которая клинически остается ограниченной периферическими нервами. Биопсия нерва может помочь установить диагноз системного васкулита, особенно когда поражение других органов не является клинически очевидным, и требуется для диагностики НСВН. Нейропатии, обусловленные системным васкулитом, следует лечить в соответствии с рекомендациями по лечению основного заболевания. При НСВН основными препаратами выбора являются глюкокортикоиды, а в тяжелых/прогрессирующих случаях используется пульс-терапия с циклофосфамидом.

Ключевые слова: васкулит, несистемная васкулитная нейропатия, васкулитная нейропатия, лечение.

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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.

Keywords: vasculitis, nonsystemic vasculitic neuropathy, vasculitic neuropathy, treatment.

Список литературы

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3. Collins MP, Dyck PJB, Gronseth GS, et al. Peripheral Nerve Society. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of nonsystemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst. 2010;15:176-84. doi: 10.1111/j.1529-8027.2010.00281.x
4. Gorson KC. Vasculitic neuropathies: an update. Neurologist 2007;13(1):12-9. doi:10.1097/01.nrl.0000252942.14928.17
5. Imboden JB. Involvement of the peripheral nervous system in polyarteritis nodosa and antineutrophil cytoplasmic antibodies-associated vasculitis. Rheum Dis Clin North Am. 2017;43(4):633-9. doi: 10.1016/j.rdc.2017.06.011
6. Cattaneo L, Chierici E, Pavone L, et al. Peripheral neuropathy in Wegener’s granulomatosis, Churg–Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry. 2007;78(10):1119-23. doi: 10.1136/jnnp.2006.111013
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8. Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg–Strauss): a study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Autoimmun Rev. 2017;16(1):1-9. doi: 10.1016/ j.autrev.2016.09.018
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10. Gwathmey KG, Burns TM, Collins MP, Dyck PJ. Vasculitic neuropathies. Lancet Neurol. 2014;13(1):67-82. doi: 10.1016/S1474-4422(13)70236-9
11. Pagnoux C, Seror R, Henegar C, et al., French Vasculitis Study Group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616-26. doi: 10.1002/art.27240
12. Bouiller K, Audia S, Devilliers H, et al. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: a retrospective study in 112 patients. Medicine (Baltimore). 2016;95(28):e4238. doi: 10.1097/MD.0000000000004238
13. Collins MP. The vasculitic neuropathies: an update. Curr Opin Neurol. 2012;25(5):573-85. doi: 10.1097/WCO.0b013e3283580432
14. Morozumi S, Koike H, Tomita M, et al. Spatial distribution of nerve fiber pathology and vasculitis in microscopic polyangiitis-associated neuropathy. J Neuropathol Exp Neurol. 2011;70(3):340-8. doi: 10.1097/NEN.0b013e3182172290
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21. Игнатова Т.М., Козловская Л.В., Гордовская Н.Б., Чернова О.А., Милованова С.Ю., Новиков П.И., Некрасова Т.П., Бекетова Т.В., Мухин Н.А. Криоглобулинемический васкулит, ассоциированный с вирусом гепатита С: 20-летний опыт лечения. Терапевтический архив. 2017;89(5):46-52 [Ignatova TM, Kozlovskaya LV, Gordovskaya NB, Chernova OA, Milovanova SYu, Novikov PI, Nekrasova TP, Beketova TV, Mukhin NA. Hepatitis C virus-associated cryoglobulinemic vasculitis: A 20-year experience with treatment. Therapeutic Archive. 2017;89(5):46-52 (In Russ.)]. doi: 10.17116/ terarkh201789546-52
22. Maneiro J, Lopez-Canoa N, Salgado E, Gomez-Reino J. Maintenance therapy of lupus nephritis with mycophenolate or azathioprine: systematic review and meta-analysis. Rheumatology (Oxford). 2014;53:834-8. doi: 10.1093/rheumatology/ket429
23. Stone J, Merkel P, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221-32. doi: 10.1056/NEJMoa0909905
24. Blaes F. Diagnosis and therapeutic options for peripheral vasculitic neuropathy. Ther Adv Musculoskelet Dis. 2015;7(2):45-55. doi: 10.1177/1759720X14566617
25. Chiche L, Bataille S, Kaplanski G, Jourde N. The place of immunotherapy in the management of HCV-induced vasculitis: an update. Clin Dev Immunol. 2012:315167. doi: 10.1155/2012/315167
26. Wludarczyk A, Szczeklik W. Neurological manifestations in ANCA-associated vasculitis – assessment and treatment. Exp Rev Neurother. 2016;16(8):861-63. doi: 10.1586/14737175.2016.1165095
27. Lubana SS, Singh N, Sanelli-Russo S, Abrudescu A. Non-Systemic Vasculitic Neuropathy: An Enigmatic Clinical Entity. Am J Case Rep. 2015;16:449-53. doi: 10.12659/AJCR.894601
28. Collins MP, Periquet-Collins I. Nonsystemic vasculitic neuropathy: update on diagnosis, classification, pathogenesis, and treatment. Front Neurol Neurosci. 2009;26:26-66. doi: 10.1159/000212368
29. Uceyler N, Geng A, Reiners K, et al. Nonsystemic vasculitic neuropathy: single-center follow-up of 60 patients. J Neurol. 2015;262:2092-100. doi: 10.1007/s00415-015-7813-5
30. De Vita S, Quartuccio L, Isola M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64:843-53. doi: 10.1002/art.34331

________________________________________________

1. Jennette JC, Falk RJ, Bacon PA, et al. 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum. 2013;65(1):1-11. doi: 10.1002/art.37715
2.[Shostak NA, Klimenko AA. Systemic vasculitis: novelty in classification, diagnosis, and treatment. Clinicist. 2015;(2):8-12 (In Russ.)]. doi: 10.17 650/1818-8338-2015-9-2-8-12
3. Collins MP, Dyck PJB, Gronseth GS, et al. Peripheral Nerve Society. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of nonsystemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst. 2010;15:176-84. doi: 10.1111/j.1529-8027.2010.00281.x
4. Gorson KC. Vasculitic neuropathies: an update. Neurologist 2007;13(1):12-9. doi:10.1097/01.nrl.0000252942.14928.17
5. Imboden JB. Involvement of the peripheral nervous system in polyarteritis nodosa and antineutrophil cytoplasmic antibodies-associated vasculitis. Rheum Dis Clin North Am. 2017;43(4):633-9. doi: 10.1016/j.rdc.2017.06.011
6. Cattaneo L, Chierici E, Pavone L, et al. Peripheral neuropathy in Wegener’s granulomatosis, Churg–Strauss syndrome and microscopic polyangiitis. J Neurol Neurosurg Psychiatry. 2007;78(10):1119-23. doi: 10.1136/jnnp.2006.111013
7. Vrancken AFJE, Said G. Vasculitic neuropathy. Handb Clin Neurol. 2013;115:463-83. doi: 10.1016/B978-0-444-52902-2.00026-6
8. Cottin V, Bel E, Bottero P, et al. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg–Strauss): a study of 157 patients by the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg–Strauss). Autoimmun Rev. 2017;16(1):1-9. doi: 10.1016/ j.autrev.2016.09.018
9. Iudici M, Pagnoux C, Quartier P, et al. French Vasculitis Study Group. Childhood- versus adult-onset ANCA-associated vasculitides: a nested, matched case-control study from the French Vasculitis Study Group Registry. Autoimmun Rev. 2018;17(2):108-14. doi: 10.1016/j.autrev.2017.11.014
10. Gwathmey KG, Burns TM, Collins MP, Dyck PJ. Vasculitic neuropathies. Lancet Neurol. 2014;13(1):67-82. doi: 10.1016/S1474-4422(13)70236-9
11. Pagnoux C, Seror R, Henegar C, et al., French Vasculitis Study Group. Clinical features and outcomes in 348 patients with polyarteritis nodosa: a systematic retrospective study of patients diagnosed between 1963 and 2005 and entered into the French Vasculitis Study Group Database. Arthritis Rheum. 2010;62(2):616-26. doi: 10.1002/art.27240
12. Bouiller K, Audia S, Devilliers H, et al. Etiologies and prognostic factors of leukocytoclastic vasculitis with skin involvement: a retrospective study in 112 patients. Medicine (Baltimore). 2016;95(28):e4238. doi: 10.1097/MD.0000000000004238
13. Collins MP. The vasculitic neuropathies: an update. Curr Opin Neurol. 2012;25(5):573-85. doi: 10.1097/WCO.0b013e3283580432
14. Morozumi S, Koike H, Tomita M, et al. Spatial distribution of nerve fiber pathology and vasculitis in microscopic polyangiitis-associated neuropathy. J Neuropathol Exp Neurol. 2011;70(3):340-8. doi: 10.1097/NEN.0b013e3182172290
15. Naddaf E, Dyck PJ. Vasculitic neuropathies. Curr Treat Options Neurol. 2015;17(10):374. doi: 10.1007/s11940-015-0374-1
16. Hadden RD, Collins MP, Živković SA, et al. Vasculitic peripheral neuropathy: case definition and guidelines for collection, analysis, and presentation of immunisation safety data. Vaccine. 2017;35(1):1567-78. doi: 10.1016/j.vaccine.2015.11.047
17. Collins MP, Hadden RD. The nonsystemic vasculitic neuropathies. Nat Rev Neurol. 2017;13:302-16. doi: 10.1038/nrneurol.2017.42
18. Graf J, Imboden J. Vasculitis and peripheral neuropathy. Curr Opin Rheumatol. 2019;31:40-5. doi: 10.1097/BOR.0000000000000559
19. De Virgilio A, Greco A, Magliulo G, Gallo A, Ruoppolo G, Conte M, et al. Polyarteritis nodosa: A contemporary overview. Autoimmun Rev. 2016;15(6):564-70. doi: 10.1016/j.autrev.2016.02.015
20. Lacomis D, Zivkovic SA. Approach to vasculitic neuropathies. J Clin Neuromusc Dis. 2007;9:265-76. doi: 10.1097/CND.0b013e31815202b3
21. [Ignatova TM, Kozlovskaya LV, Gordovskaya NB, Chernova OA, Milovanova SYu, Novikov PI, Nekrasova TP, Beketova TV, Mukhin NA. Hepatitis C virus-associated cryoglobulinemic vasculitis: A 20-year experience with treatment. Therapeutic Archive. 2017;89(5):46-52 (In Russ.)]. doi: 10.17116/ terarkh201789546-52
22. Maneiro J, Lopez-Canoa N, Salgado E, Gomez-Reino J. Maintenance therapy of lupus nephritis with mycophenolate or azathioprine: systematic review and meta-analysis. Rheumatology (Oxford). 2014;53:834-8. doi: 10.1093/rheumatology/ket429
23. Stone J, Merkel P, Spiera R, et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med. 2010;363:221-32. doi: 10.1056/NEJMoa0909905
24. Blaes F. Diagnosis and therapeutic options for peripheral vasculitic neuropathy. Ther Adv Musculoskelet Dis. 2015;7(2):45-55. doi: 10.1177/1759720X14566617
25. Chiche L, Bataille S, Kaplanski G, Jourde N. The place of immunotherapy in the management of HCV-induced vasculitis: an update. Clin Dev Immunol. 2012:315167. doi: 10.1155/2012/315167
26. Wludarczyk A, Szczeklik W. Neurological manifestations in ANCA-associated vasculitis – assessment and treatment. Exp Rev Neurother. 2016;16(8):861-63. doi: 10.1586/14737175.2016.1165095
27. Lubana SS, Singh N, Sanelli-Russo S, Abrudescu A. Non-Systemic Vasculitic Neuropathy: An Enigmatic Clinical Entity. Am J Case Rep. 2015;16:449-53. doi: 10.12659/AJCR.894601
28. Collins MP, Periquet-Collins I. Nonsystemic vasculitic neuropathy: update on diagnosis, classification, pathogenesis, and treatment. Front Neurol Neurosci. 2009;26:26-66. doi: 10.1159/000212368
29. Uceyler N, Geng A, Reiners K, et al. Nonsystemic vasculitic neuropathy: single-center follow-up of 60 patients. J Neurol. 2015;262:2092-100. doi: 10.1007/s00415-015-7813-5
30. De Vita S, Quartuccio L, Isola M, et al. A randomized controlled trial of rituximab for the treatment of severe cryoglobulinemic vasculitis. Arthritis Rheum. 2012;64:843-53. doi: 10.1002/art.34331

Авторы

И.Ю. Головач 1, Е.Д. Егудина 2

1 Клиническая больница «Феофания» Государственного управления делами, Киев, Украина;
2 Клиника современной ревматологии, Киев, Украина

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I.Yu. Golovach 1, Ye.D. Yehudina 2

1 “Feofaniya” Clinical hospital State Management of Affairs, Kyiv, Ukraine;
2 Clinic of modern rheumatology, Kyiv, Ukraine

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