Wegener’s granulomatosis in ophthalmic practice. Clinical case

Wegener’s granulomatosis (WG) or granulomatosis with polyangiitis is a primary systemic necrotizing vasculitis of small and medium-sized vessels with the development of foci of granulomatous inflammation. This is a severe, progressive, presumably autoimmune disease associated with antineutrophil cytoplasmic antibodies, which is detected mainly in people over 50 years of age. In the absence of timely treatment — immunosuppressive therapy (steroids and cytostatics to suppress disease activity, maintain remission and treat relapses), it can lead to the death of the patient. In the WG clinic, it begins with symptoms characteristic for respiratory infection, the lesions of the upper respiratory tract (rhinitis, nasopharyngitis, etc.), eyes, lungs (fibrosis, pleurisy, etc.) and kidneys (glomerulonephritis) prevail. One of the organs that are quite often (in about half of patients) affected by this disease, and, mainly, already in the early stages is the eye. With its defeat, in some cases, WG can even begin, but more and more often the eye is involved against the background of the development and long-term course of the inflammatory process of the upper respiratory tract and/or the first developed purulent inflammation of the middle ear in adult patients and nasal sinuses. In WG, any part of the eye can be affected, most often the anterior (most often scleritis, keratitis, less often — a corneal ulcer, etc.) and much less often — the posterior, and both in the form of a slight inflammation of the membranes of the eye to severe involvement of all its structures and orbit (pseudotumor ) with the occurrence of low vision and even complete blindness. Difficulties in the diagnosis of WG usually arise due to the variety of forms and variants of the clinical course in the early stages of the disease, when ophthalmic changes are still of little specificity, and systemic ones are practically absent. Our clinical case of WG in a 62-year-old patient indicates that the diagnosis of this disease is far from simple and takes a very long period of time. Although the presence of episleritis/scleritis with a refractory course of the pathological process, especially if the patient had a history of bilateral purulent otitis media suffered by the patient and an infiltrate in the lungs, regarded, despite the biopsy during the first analysis, as a manifestation of atypical pneumonia, should already have alerted doctors to regarding the diagnosis of WG. Unfortunately, only severe dysphagia prompted the patient to be urgently hospitalized, to examine her as much as possible with a second revision of the previously taken lung biopsy material and to make the right decision in favor of the diagnosis of WG with the appointment of subsequent adequate treatment.

1. Greco A, Marinelli C, Fusconi M, et al. Clinic manifestations in granulomatosis with polyangiitis. International Journal of Immunopathology and Pharmacology. 2016; 29 (2): 151–9. doi 10.1177/0394632015617063

2. Wegener F. Ueber eine eigenartige rhinogene Granulomatose mit besonderer Beteiligung des Arteriensystems und der Nieren. Beitr Pathol Anat. 1939; 102: 36–68. https://doi.org/10.1016/S0005-8165(76)80082-0

3. Godman GC, Churg J. Wegener’s granulomatosis: pathology and review of the literature. AMA Arch Pathol. 1954 Dec; 58 (6): 533–53. PMID: 13217569.

4. Hodosh E.M., Krut’ko V.S., Efremova O.A. Granulomatosis with polyangiitis (Wegener’s) and systemic vasculitis: a multifaceted view of the problem (literature review). Nauchnye vedomosti Belgorodskogo gosudarstvennogo universiteta. Seriya: Medicina. Farmaciya. 2017; 19 (268): 40–53 (In Russ.).

5. Serebryakova E.N., Volosnikov D.K., Pishchalnikov A.Yu., et al. Wegener’s granulomatosis (granulomatosis with polyangiitis). Difficult patient. 2021: 19 (4): 39–44 (In Russ.). doi: 10.224412/2074-1005-2021-4-39-44

6. Levina T.M., Romanov M.D., Kireeva E.M. Features of diagnosis and treatment of Wegener’s granulomatosis. News of higher educational institutions. Volga region. Medical sciences. 2019; 1 (49): 15–26 (In Russ.). doi 10.21685/2072-3032-2019-1-2

7. Pearce FA, Lanyon PC, Watts RA, et al. Novel insights into the aetiology of granulomatosis with polyangiitis-a case-control study using the Clinical Practice Research Datalink. Rheumatology (Oxford). 2018 Jun 1; 57 (6): 1002–10. doi: 10.1093/rheumatology/kex512

8. Lane SE, Watts RA, Bentham G, Innes NJ, Scott DG. Are environmental factors important in primary systemic vasculitis? A case-control study. Arthritis Rheum. 2003 Mar; 48 (3): 814–23. doi: 10.1002/art.10830

9. Falk RJ, Terrell RS, Charles LA, et al. Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci USA. 1990 Jun; 87 (11): 4115–9. doi: 10.1073/pnas.87.11.4115

10. Lavrishcheva Yu.V., Kaledinova Y.S., Yakovenko A.A., Artemev I.A. Severe course of quickly progressing glomerulonephritis in a patient with ANTSA-associated vasculitis: review and case report. Translational medicine. 2020; 7 (3): 55–62 (In Russ.). https://doi.org/10.18705/2311-4495-2020-7-3-55-62

11. Borovoj V.N., Lis E.S. A clinical case with a fatal outcome: Wegener’s granulomatosis, generalized form. Smolenskij medicinskij al’manakh. 2020; 3: 245–9 (In Russ.).

12. Burluckaya A.V., Savel’eva N.V., Taran N.S. ANCA-associirovannyj vaskulit u podrostka 14 let: klinicheskij sluchaj. Kubanskij nauchnyj medicinskij vestnik. 2020; 27 (5): 184–94 (In Russ.).

13. Stamp LK, Chapman PT, Francis J, et al. Association between environmental exposures and granulomatosis with polyangiitis in Canterbury, New Zealand. Arthritis Res Ther. 2015 Nov 24; 17: 333. doi: 10.1186/s13075-015-0852-6

14. Gurizheva M.V., Nahusheva A.R., Muskhanov E.A., et al. Rare systemic vasculitides in clinical practice: diagnostic difficulties. Trudnyj pacient. 2019; 17: 11–12: 36–40 (In Russ.).

15. Vereshchagina O.E., Karpishchenko1 S.A., Teplova E.O. Synechiae of the nasal cavity as a manifestation of granulomatosis with polyangiitis: clinical case. Russkij medicinskij zhurnal. 2020; 28 (4) 34–6 (In Russ.).

16. Federal clinical guidelines for the diagnosis and treatment of systemic vasculitis. Moscow; 2015 (In Russ.).