Modern Pediatrics. Ukraine. (2023). 6(134): 149-160. doi 10.15574/SP.2023.134.149
Okhotnikova O. M.¹, Tkachova Т. М.¹, Іvanova Т. P.², Оshljanska О. А.¹, Usova О. І.¹, Sharikadze О. V.¹, Gristchenko О. M.², Shkljarska H. V.², Denysenko А. Y.², Rudenko S. M.², Semenenko L. V.^2
1Shupyk National University of Health Care of Ukraine, Kyiv
²National Children’s Specialized Hospital “OKHMATDYT” of the Ministry of Health of Ukraine, Kyiv

For citation: Okhotnikova OM, Tkachova ТМ, Іvanova ТP, Оshljanska ОА, Usova ОІ, Sharikadze ОV et al. (2023). A Features of the modern course of granulomatosis with polyangiitis (Wegener's granulomatosis) in children on the example of a clinical case in a teenage patient. Modern Pediatrics. Ukraine. 6(134): 149-160. doi 10.15574/SP.2023.134.149.
Article received: Jul 04 2023. Accepted for publication: Oct 06, 2023.

The article introduces readers to a rare disease from the group of systemic vasculitis – Wegener’s granulomatosis. The prevalence of this disease in European countries ranges from 4.9 to 10.5 cases per 1 million adult population. A feature of the disease is the pathology of the upper respiratory tract at the beginning of the inflammatory process, which leads to long and difficult treatment of patients by otolaryngologists, as a result of which the correct diagnosis is delayed and the prognosis of the disease worsens. The disease is especially rare among children.
Purpose – to share the experience of diagnosis and treatment of a teenager with this extremely rare pathology. Complications of drug therapy and ways to solve them are considered in detail.
The conclusion of this experience is an understanding of the severity of the course of the disease and the need to use aggressive therapy already in its early stages in order to save the patient’s life.
The study was conducted in accordance with the principles of the Declaration of Helsinki. Informed consent of the parents and the child was obtained for the study.
No conflict of interests was declared by the authors.
Keywords: Wegener’s granulomatosis, adolescent, systemic vasculitis, diagnosis, treatment, complications.

REFERENCES

1. Beketova TV. (2012). Hranulematoz z polianhiitom, patohenetychno pov'iazanyi z ANTsA: osoblyvosti klinichnykh proiaviv. Nauchno-praktycheskaia revmatolohyia. 50 (6): 19-28.

2. Cottin V, Cordier J-F, Richeldi L. (2015). Orphan lung diseases (the clinical guide to rare lung disease). Springer: 611. https://doi.org/10.1007/978-1-4471-2401-6

3. Duzhyi I.D, Byzov VV. (2003). Trudnoshchi diahnostyky y uskladnennia pry hranulematozi Vehenera. Lik. Sprava. 7: 94-97.

4. Jennette JC, Falk RJ, Bacon PA et al. (2013). Revised international Chapel Hill consensus conference nomenclature of vasculitis. Arthritis Rheum. 65: 1-11.

5. Kovalenko VM, Shuba NM. (2013). Natsionalnyi pidruchnyk z revmatolohii. Za redaktsiieiu V.M. Kovalenka, N.M. Shuby. Kyiv: 320-328.

6. Lane S.E. (2005). Epidemiology of systemic vasculitis. Curr. Rheumatol. Rep. 7: 270-275. https://doi.org/10.1007/s11926-005-0036-5; PMid:16045829

7. Markov HY. (2010). Dyahnostyka y lechenye hranulematoza Vehenera. Vestnyk otorynolarynholohyy. 1: 19-20.

8. Ministerstvo zdravoohraneniya Rossii. (2017). Federalniye klynycheskye rekomendatsyy; Hranulematoz Vehenera (vozrastnaia hruppa – dety): 65.

9. Nasonov L. (2010). Klynycheskye rekomendatsyy. Moskva: HEOTAR-Medya: 752.

10. Sterlynh DzhV. (2001). Sekreti revmatolohyy. Per. s anhl. pod obshchei red. d-ra med. nauk, prof. V.N. Khyrmanova. Moskva: BYNOM, Sankt-Peterburh, Nevskyi Dyalekt: 750.

11. Watts R.A et al. (2001). Epidemiology of vasculitis in Europe. Ann. Rheum. Dis. 60 (12): 1156-1157. https://doi.org/10.1136/ard.60.12.1156a; PMid:11760724 PMCid:PMC1753455

12. Zerbino DD, Zemba OO. (2013). Hranulematoz Vehenera: uskladnennia ta prychyny smerti. Ukrainskyi medychnyi chasopys. 5 (97); IX/X.

13. Zerbyno DD, Chopiak VV. (2009). Klassyfykatsyy vaskulytov y krytycheskaia otsenka problemi. Sertse i sudyny. 4 (28): 93-100.