Cutaneous manifestations of ANCA-associated vasculitis and immunosuppressive therapy: cause-effect relationships (a case report)

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Abstract


Granulomatosis with polyangiitis, formerly known as Wegener’s granulomatosis, is an autoimmune ANCA-associated systemic vasculitis characterized by extensive damage to multiple organs and systems. Besides a typical clinical triad of ENT, lungs, and kidneys injury, various types of skin lesions can be found in 10–50% of cases. A severe course of the disease and low survival of patients often requires using aggressive treatment in a form of combined immunosuppressive therapy. On the one hand, it generally improves the prognosis, and on the other is itself associated with numerous complications. One of them is a secondary infection. Skin is the second most common localization of infection after the respiratory system. Preceding skin lesions caused by vasculitis may increase the risk of infection. Thus, patients with ANCA-associated vasculitis should be carefully observed for cutaneous manifestation, both before and during the immunosuppressive therapy.


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About the authors

Madina A. Kitova

Municipal Out-Patient Department No 93

Email: madina8141@mail.ru

Russian Federation, Saint Petersburg

rheumatologist, Municipal out-patient department №93

Maksim V. Maksimov

Pavlov University

Email: bolec@sampo.ru

Russian Federation, Saint Petersburg

PhD (Medicine), associate professor, Hospital Therapy Department

 

Valeriy N. Marchenko

Pavlov University

Email: marchvn@mail.ru
ORCID iD: 0000-0002-2440-7222
SPIN-code: 1711-4150
Scopus Author ID: 57206419660

Russian Federation, Saint Petersburg

PhD (Medicine), Professor, Hospital Therapy Department

Elena A. Bruchkus

Pavlov University

Email: bruklen@listl.ru

Russian Federation, Saint Petersburg

Head of the Second Cardiology Department, Scientific Research Institute of Rheumatology and Allergology of the Scientific and Clinical Research Center

Denis A. Davydov

Pavlov University

Author for correspondence.
Email: davydov.rheum@gmail.com
ORCID iD: 0000-0002-5524-1616
SPIN-code: 1132-5294
Scopus Author ID: 57217159189

Russian Federation, Saint Petersburg

resident, Hospital Therapy Department

References

  1. Баранов А.А. Системные васкулиты // Ревматология: национальное руководство / под ред. Е.Л. Насонова, В.А. Насоновой. – М.: ГЭОТАР-Медиа, 2008. – С. 528–567. [Baranov AA. Sistemnye vaskulity. In: Revmatologiya. Natsional’noe rukovodstvo. Ed. by E.L. Nasonov, V.A. Nasonova. Moscow: GEOTAR-Media; 2008. P. 528-567. (In Russ.)]
  2. Jennette JC. Overview of the 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Clin Exp Nephrol. 2013;17(5):603-606. https://doi.org/10.1007/s10157-013-0869-6.
  3. Sinico RA, Guillevin L. Anti-Neutrophil Cytoplasmic Antibody (ANCA) Associated Vasculitis. Cham, Switzerland: Springer; 2020. 328 р.
  4. Weiner M, Segelmark M. The clinical presentation and therapy of diseases related to anti-neutrophil cytoplasmic antibodies (ANCA). Autoimmunity Reviews. 2016;15(10): 978-982. https://doi.org/10.1016/j.autrev.2016.07.016.
  5. Lutalo PM, D’cruz DP. Diagnosis and classification of granulomatosis with polyangiitis (aka Wegener’s granulomatosis). J Autoimmun. 2014;48-49:94-98. https://doi.org/ 10.1016/j.jaut.2014.01.028.
  6. Grygiel-Górniak B, Limphaibool N, Perkowska K, Puszczewicz M. Clinical manifestations of granulomatosis with polyangiitis: Key considerations and major features. Postgrad Med. 2018;130(7):581-596. https://doi.org/10.1080/00325481.2018.1503920.
  7. Marzano AV, Raimondo MG, Berti E, et al. Cutaneous manifestations of ANCA-associated small vessels vasculitis. Clin Rev Allergy Immunol. 2017;53(3):428-438. https://doi.org/10.1007/s12016-017-8616-5.
  8. Suwanchote S, Rachayon M, Rodsaward P, et al. Anti-neutrophil cytoplasmic antibodies and their clinical significance. Clin Rheumatol. 2018;37(4):875-884. https://doi.org/10.1007/s10067-018-4062-x.
  9. Бекетова Т.В. Стандартная индукционная терапия АНЦА-ассоциированных системных васкулитов: мифы и реальность // Фарматека. – 2015. – № 7. – C. 7–13. [Beketova TV. Standard induction therapy of ANCA-associated systemic vasculitis: myths and realities. Farmateka. 2015;(7):7-13. (In Russ.)]
  10. Derhovanessian A, Tazelaar H, Belperio J, Lynch J. Granulomatosis with polyangiitis (wegener’s granulomatosis): Evolving concepts in treatment. Semin Respir Crit Care Med. 2018;39(4):434-458. https://doi.org/10.1055/ s-0038-1660874.
  11. Чудинов А.Л., Беляева И.Б., Мазуров В.И., Инамова О.В. Рациональная фармакотерапия АНЦА-ассоциированных системных васкулитов // Эффективная фармакотерапия. – 2019. – Т. 15. – № 6. – C. 16–20. [Chudinov AL, Belyayeva IB, Mazurov VI, Inamova OV. Rational pharmacotherapy of ANCA-associated systemic vasculitis. Effective pharmacotherapy. 2019;15(6):16-20. (In Russ.)]
  12. Walsh M, Merkel PA, Peh C-A, et al. Plasma exchange and glucocorticoids in severe anca-associated vasculitis. N Engl J Med. 2020;382(7):622-631. https://doi.org/10.1056/nejmoa1803537.
  13. Little MA, Nightingale P, Verburgh CA, et al. Early mortality in systemic vasculitis: Relative contribution of adverse events and active vasculitis. Ann Rheum Dis. 2009;69(6):1036-1043. https://doi.org/10.1136/ard.2009.109389.
  14. Charlier C, Henegar C, Launay O, et al. Risk factors for major infections in Wegener granulomatosis: analysis of 113 patients. Ann Rheum Dis. 2008;68(5):658-663. https://doi.org/10.1136/ard.2008.088302.
  15. Yang L, Xie H, Liu Z, et al. Risk factors for infectious complications of ANCA-associated vasculitis: A cohort study. BMC Nephrol. 2018;19(1):138. https://doi.org/ 10.1186/s12882-018-0933-2.
  16. Wall N, Harper L. Complications of long-term therapy for ANCA-associated systemic vasculitis. Nat Rev Nephrol. 2012;8(9):523-532. https://doi.org/10.1038/nrneph.2012.107.
  17. King C, Harper L. Avoidance of harm from treatment for ANCA-associated vasculitis. Curr Treatm Opt Rheumatol. 2017;3(4):230-243. https://doi.org/10.1007/s40674-017-0082-y.

Supplementary files

Supplementary Files Action
1.
Fig. 1. Crusted ulcerative defects of the left shin

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Fig. 2. Weeping ulcer in the left inframammary fold

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3.
Fig. 3. Fibrin-covered ulcer in the sacral region

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4.
Fig. 4. Ulcer with detritus behind the left ear

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Copyright (c) 2020 Kitova M.A., Maksimov M.V., Marchenko V.N., Bruchkus E.A., Davydov D.A.

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