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ANCA-associated vasculitis presenting with isolated neurological manifestations in a patient with cocaine abuse: a case report and literature review

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Abstract

Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of rare conditions predominantly affecting small vessels of skin, musculoskeletal, pulmonary, renal, and rarely central and peripheral nervous systems. Isolated neurological manifestations of AAV are uncommon and challenging to diagnose. Cocaine has been reported as a potential trigger for the development of AAV. There are only a few case reports of isolated neurological involvement in cocaine-induced AAV with poorly characterized histopathological features. We present a unique case of AAV with isolated neurological manifestations presenting with multiple cranial neuropathies, leptomeningeal enhancement on imaging and histopathologic evidence of small-vessel vasculitis in the leptomeninges and brain and extensive dural fibrosis in a patient with cocaine abuse. The patient’s progressive neurological deficits were controlled after starting immunosuppression with rituximab and prednisone. We also reviewed the literature to provide the diagnostic overview of AAV and evaluate intervention options. To our knowledge, this is the first case of AAV with isolated neurological manifestations and histopathologic evidence of small-vessel vasculitis in a patient with cocaine abuse. Patients with multiple cranial neuropathies and meningeal involvement should be screened for AAV, especially if they have a history of cocaine abuse.

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References

  1. Jennette JC et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65(1):1–11. https://doi.org/10.1002/art.37715

    Article  CAS  PubMed  Google Scholar 

  2. Segelmark M (2020) Serological testing in small vessel vasculitis. Rheumatology (Oxford) 59(Suppl 3):51–54. https://doi.org/10.1093/rheumatology/kez633

    Article  CAS  Google Scholar 

  3. Kitching AR et al (2020) ANCA-associated vasculitis. Nat Rev Dis Primers 6(1):71. https://doi.org/10.1038/s41572-020-0204-y

    Article  PubMed  Google Scholar 

  4. Woller S, Sood A, Maclean AKW (2023) Propylthiouracil-induced vasculitis. Proc (Bayl Univ Med Cent) 36(5):647–649. https://doi.org/10.1080/08998280.2023.2230830

    Article  PubMed  Google Scholar 

  5. Wludarczyk A, Szczeklik W (2016) Neurological manifestations in ANCA-associated vasculitis - assessment and treatment. Expert Rev Neurother 16(8):861–863. https://doi.org/10.1586/14737175.2016.1165095

    Article  CAS  PubMed  Google Scholar 

  6. Pendolino AL et al (2021) Olfactory dysfunction in antineutrophil cytoplasmic antibody-associated vasculitides: A review of the literature. World J Methodol 11(2):15–22. https://doi.org/10.5662/wjm.v11.i2.15

    Article  PubMed  PubMed Central  Google Scholar 

  7. Kubal AA, Perez VL (2010) Ocular manifestations of ANCA-associated vasculitis. Rheum Dis Clin North Am 36(3):573–586. https://doi.org/10.1016/j.rdc.2010.05.005

    Article  PubMed  Google Scholar 

  8. Catala M, Kubis N (2013) Gross anatomy and development of the peripheral nervous system. Handb Clin Neurol 115:29–41. https://doi.org/10.1016/B978-0-444-52902-2.00003-5

    Article  PubMed  Google Scholar 

  9. Subesinghe S, van Leuven S, Yalakki L, Sangle S, D’Cruz D (2018) Cocaine and ANCA associated vasculitis-like syndromes - a case series. Autoimmun Rev 17(1):73–77. https://doi.org/10.1016/j.autrev.2017.11.011

    Article  CAS  PubMed  Google Scholar 

  10. García-Pérez D et al (2020) Snorting the brain away: cerebral damage as an extension of cocaine-induced midline destructive lesions. J Neuropathol Exp Neurol 79(12):1365–1369. https://doi.org/10.1093/jnen/nlaa097

    Article  PubMed  Google Scholar 

  11. Trimarchi M, Bussi M, Sinico RA, Meroni P, Specks U (2013) Cocaine-induced midline destructive lesions - an autoimmune disease? Autoimmun Rev 12(4):496–500. https://doi.org/10.1016/j.autrev.2012.08.009

    Article  CAS  PubMed  Google Scholar 

  12. Hamilton K, Sabiq F, Percy J, Hirsch-Reinshagen V (2022) Focal chronic meningitis with lymphoid follicle-like structures associated with cocaine-induced midline destructive lesion: a case report. J Neuropathol Exp Neurol 81(7):577–580. https://doi.org/10.1093/jnen/nlac038

    Article  PubMed  Google Scholar 

  13. Albert L, DeMattia JA (2011) Cocaine-induced encephalocele: case report and literature review. Neurosurgery 68(1):E263–E266. https://doi.org/10.1227/NEU.0b013e3181fead2e

    Article  PubMed  Google Scholar 

  14. Lee KC, Ladizinski B, Nutan FNU (2012) Systemic complications of levamisole toxicity. J Am Acad Dermatol 67(4):791–792. https://doi.org/10.1016/j.jaad.2011.11.969

    Article  CAS  PubMed  Google Scholar 

  15. Chung C et al (2011) Characteristic purpura of the ears, vasculitis, and neutropenia–a potential public health epidemic associated with levamisole-adulterated cocaine. J Am Acad Dermatol 65(4):722-725.e2. https://doi.org/10.1016/j.jaad.2010.08.024

    Article  PubMed  PubMed Central  Google Scholar 

  16. Gupta S, Sethi P, Duvesh R, Sethi HS, Naik M, Rai HK (2021) Optic perineuritis. BMJ Open Ophth 6(1):e000745. https://doi.org/10.1136/bmjophth-2021-000745

    Article  Google Scholar 

  17. Santoriello D, et al. (2021) “Anti-neutrophil cytoplasmic antibody associated glomerulonephritis complicating treatment with hydralazine.” Kidney Int. https://doi.org/10.1016/j.kint.2021.03.029

  18. Graf J (2013) Rheumatic manifestations of cocaine use. Curr Opin Rheumatol 25(1):50–55. https://doi.org/10.1097/BOR.0b013e32835b4449

    Article  CAS  PubMed  Google Scholar 

  19. Naranjo CA et al (1981) A method for estimating the probability of adverse drug reactions. Clin Pharmacol Ther 30(2):239–245. https://doi.org/10.1038/clpt.1981.154

    Article  CAS  PubMed  Google Scholar 

  20. Neynaber S et al (2008) PR3-ANCA-positive necrotizing multi-organ vasculitis following cocaine abuse. Acta Derm Venereol 88(6):594–596. https://doi.org/10.2340/00015555-0514

    Article  PubMed  Google Scholar 

  21. Tervaert JWC, Stegeman CA (2004) A difficult diagnosis. Lancet 364(9442):1313–1314. https://doi.org/10.1016/S0140-6736(04)17183-3

    Article  PubMed  Google Scholar 

  22. Rowshani AT, Schot LJ, ten Berge IJM (2004) c-ANCA as a serological pitfall. Lancet 363(9411):782. https://doi.org/10.1016/S0140-6736(04)15694-8

    Article  PubMed  Google Scholar 

  23. Robson JC et al (2022) 2022 American college of rheumatology/European alliance of associations for rheumatology classification criteria for granulomatosis with polyangiitis. Ann Rheum Dis 81(3):315–320. https://doi.org/10.1136/annrheumdis-2021-221795

    Article  PubMed  Google Scholar 

  24. Trimarchi M et al (2001) Cocaine-induced midline destructive lesions: clinical, radiographic, histopathologic, and serologic features and their differentiation from Wegener granulomatosis. Medicine (Baltimore) 80(6):391–404. https://doi.org/10.1097/00005792-200111000-00005

    Article  CAS  PubMed  Google Scholar 

  25. Perez Alamino R, Espinoza LR (2013) Vasculitis mimics: cocaine-induced midline destructive lesions. Am J Med Sci 346(5):430–431. https://doi.org/10.1097/MAJ.0b013e3182972014

    Article  PubMed  Google Scholar 

  26. Bachi K, Mani V, Jeyachandran D, Fayad ZA, Goldstein RZ, Alia-Klein N (2017) Vascular disease in cocaine addiction. Atherosclerosis 262:154–162. https://doi.org/10.1016/j.atherosclerosis.2017.03.019

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  27. Simsek S et al (2006) Sino-nasal bony and cartilaginous destruction associated with cocaine abuse, S. aureus and antineutrophil cytoplasmic antibodies. Neth J Med 64(7):248–251

    CAS  PubMed  Google Scholar 

  28. Pendergraft WF et al (2004) Autoimmunity is triggered by cPR-3(105–201), a protein complementary to human autoantigen proteinase-3. Nat Med 10(1):72–79. https://doi.org/10.1038/nm968

    Article  CAS  PubMed  Google Scholar 

  29. Van Gool IC, Kers J, Bakker JA, Rotmans JI, Teng YKO, Bauer MP (2022) Antineutrophil cytoplasmic antibodies in infective endocarditis: a case report and systematic review of the literature. Clin Rheumatol 41(10):2949–2960. https://doi.org/10.1007/s10067-022-06240-w

    Article  PubMed  PubMed Central  Google Scholar 

  30. Wiesner O et al (2004) Antineutrophil cytoplasmic antibodies reacting with human neutrophil elastase as a diagnostic marker for cocaine-induced midline destructive lesions but not autoimmune vasculitis. Arthritis Rheum 50(9):2954–2965. https://doi.org/10.1002/art.20479

    Article  CAS  PubMed  Google Scholar 

  31. Nolan AL, Jen K-Y (2015) Pathologic manifestations of levamisole-adulterated cocaine exposure. Diagn Pathol 10:48. https://doi.org/10.1186/s13000-015-0279-z

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  32. Weng C-H, Liu Z-C (2019) Drug-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. Chin Med J 132(23):2848–2855. https://doi.org/10.1097/CM9.0000000000000539

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  33. Gao Y, Zhao M-H (2009) Review article: drug-induced anti-neutrophil cytoplasmic antibody-associated vasculitis. Nephrology (Carlton) 14(1):33–41. https://doi.org/10.1111/j.1440-1797.2009.01100.x

    Article  CAS  PubMed  Google Scholar 

  34. Chung SA et al (2021) 2021 American college of rheumatology/vasculitis foundation guideline for the management of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheumatol 73(8):1366–1383. https://doi.org/10.1002/art.41773

    Article  CAS  PubMed  Google Scholar 

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Acknowledgements

We would like to thank the patient for allowing us permission to report and publish this case.

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There is no funding to be reported for this manuscript.

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Authors and Affiliations

  1. Department of Neurology, University of North Carolina School of Medicine, 170 Manning Drive, CB 7025, Chapel Hill, NC, 27599, USA

    Fazila Aseem, Freshta Khoshbakht, Anahit C. Mehrabyan, Bushra Javed, Irena Dujmovic Basuroski & Monica M. Diaz

  2. Department of Neurology, University of Washington School of Medicine, Seattle, WA, 98195, USA

    Victor Lin

  3. Department of Medicine, Division of Rheumatology, Allergy, and Immunology, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA

    Abigail L. Gilbert & Alfredo C. Rivadeneira

  4. Department of Pathology and Laboratory Medicine, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA

    J. Charles Jennette & Thomas W. Bouldin

  5. Department of Radiology, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA

    Yueh. Z. Lee

  6. Department of Neurosurgery, University of North Carolina School of Medicine, Chapel Hill, NC, 27599, USA

    Kelly Chamberlin & Aaron Gelinne

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  1. Fazila Aseem
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Correspondence to Monica M. Diaz.

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Aseem, F., Lin, V., Gilbert, A.L. et al. ANCA-associated vasculitis presenting with isolated neurological manifestations in a patient with cocaine abuse: a case report and literature review. Clin Rheumatol 43, 1401–1407 (2024). https://doi.org/10.1007/s10067-024-06919-2

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