Eosinophilic granulomatosis with polyangiitis (Churg–Strauss syndrome) as a multi-organ pathology in the practice of a district therapist and related specialists

Objective. To demonstrate the role of a primary care specialist, a district therapist, in the diagnosis and subsequent monitoring of a rare and difficult-to-recognize disease, eosinophilic granulomatosis with polyangiitis (EGPA), characterized by multiple organ damage, using a clinical case example.

Materials and methods. This article presents a clinical case of a 72-year-old patient with EGPA. The stages of diagnostic search are described, including the patient's initial visit to a district therapist, analysis of the patient's medical history, physical examination, laboratory and instrumental tests, verification of the diagnosis by a rheumatologist, as well as the treatment and monitoring of the patient's condition.

Results. A case of a patient with ECPA manifestation after a long remission of bronchial asthma, with a predominance of neurological symptoms (ischemic stroke, polyneuropathy), subclinical damage to the heart (complete blockade of the left bundle branch, hydropericard), and kidneys is described. Presented case demonstrates the role of a primary care specialist – a district therapist – in the diagnosis and subsequent monitoring of a rare and difficult-to-recognize disease, eosinophilic granulomatosis with polyangiitis (EGPA), characterized by multiple organ damage. Timely and adequate therapy led to clinical and laboratory remission.

Conclusion. This clinical case demonstrates the importance of the district therapist's alertness regarding rare diseases such as EGPAs, especially in the presence of bronchial asthma and eosinophilia. The need for a comprehensive examination of patients with multiple organ pathology is emphasized. Timely diagnosis and adequate therapy contribute to achieving remission. In the future, it is important to monitor the patient to prevent relapses and treat associated diseases. Despite the fact that the diagnosis and treatment of EGPAs are the responsibility of a rheumatologist, the early diagnosis and referring the patients to a specialist depend largely on the district therapist.

1. Beketova T.V. New ACR/EULAR2022 classification criteria for ANCA-associated vasculitis. Rheumatology Science and Practice. 2023; 61 (5): 531–536. (In Russ.).

2. Lanham JG, Elkon KB, Pusey CD, Hughes GR. Systemic vasculitis with asthma and eosinophilia: a clinical approach to the Churg–Strauss syndrome. Medicine (Baltimore). 1984 Mar; 63 (2): 65–81. https://doi.org/10.1097/00005792-198403000-00001

3. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, Calabrese LH, Edworthy SM, Fauci AS, Leavitt RY. et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990 Aug; 33 (8): 1094–100. https://doi.org/10.1002/art.1780330806

4. Murkamilov I.T., Aitbaev K.A., Raiymzhanov Z.R., Ymankulov D.S., Yusupova T.F., Yusupov F.A. ANCA-associated vasculitis: focusing on eosinophilic granulomatosis with polyangiitis (Churg– Strauss eosinophilic vasculitis). Bulletin of Science and Practice. 2024; 10 (2): 203–23. (In Russ.).

5. Adasheva T.V., Nesterenko O.I., Zadionchenko V.S., Kharkova M.S., Samorukova E.I. Clinical case of Churg–Strauss syndrome: diagnostic challenges, therapeutic approach. Arkhiv vnutrennei meditsiny. 2016; 5 (31): 63–9. (In Russ.).

6. Isaikin A.I., Chernenko O.A., Pozhidaev K.A., Smirnova D.S., Ilina K.A. Peripheral and central nervous system involvement in Churg–Strauss syndrome (eosinophilic granulomatosis with polyangiitis). Neurological Journal. 2017; 22 (5): 267–74. (In Russ.).

7. Mikhailova Z.D., Pochinka I.G., Vostokova A.A., Cherepanova V.V. Hypereosinophilia in a cardiologist's practice: the path to diagnosis. Kardiologiia: novosti, mneniia, obuchenie. Cardiology: News, Opinions, Training. 2019; 7 (2): 71–8. (In Russ.).

8. Kirichenko M.V., Shutemova E.A., Manokhin V. Yu., Afanasyeva I.P. Difficulties in the diagnosis of eosinophilic granulomatosis with polyangiitis. Vestnik Ivanovskoi meditsinskoi akademii. Bulletin of the Ivanovo Medical Academy. 2018; 23 (3): 44–8. (In Russ.).

9. Grayson PC, Monach PA, Pagnoux C, Cuthbertson D, Carette S, Hoffman GS, Khalidi NA, Koening CL, Langford CA, Maksimowicz-McKinnon K, Seo P, Specks U, Ytterberg SR, Merkel PA; Vasculitis Clinical Research Consortium. Value of commonly measured laboratory tests as biomarkers of disease activity and predictors of relapse in eosinophilic granulomatosis with polyangiitis. Rheumatology (Oxford). 2015 Aug; 54 (8): 1351–9. https://doi.org/10.1093/rheumatology/keu427

10. Grayson PC, Ponte C, Suppiah R, Robson JC, Craven A, Judge A, Khalid S, Hutchings A, Luqmani RA, Watts RA, Merkel PA; DCVAS Study Group. 2022 American College of Rheumatology/European Alliance of Associations for Rheumatology Classification Criteria for Eosinophilic Granulomatosis With Polyangiitis. Arthritis Rheumatol. 2022 Mar; 74 (3): 386–392. https://doi.org/10.1002/art.41982

11. Bayrashevskaya A.V., Degtyareva N.D., Radenska-Lopovok S.G. ANTsA-assotsiirovannye vaskulity. ANCA-associated small-vessel vasculitides. Arkh Patol. 2022; 84 (1): 50–58. (In Russ.).

12. Bagisheva N.V., Trukhan D.I., Viktorova I.A., Alekseenko V.A., Kononenko A. Yu., Nikolaeva N. Yu. Difficulties in the diagnosis of eosinophilic granulomatosis with polyangiitis (Churg– Strauss syndrome). Consilium Medicum. 2018; 20 (11): 55–60. (In Russ.).