Ocular Manifestations of Granulomatosis With Polyangiitis.

Sponsor
Military Institute od Medicine National Research Institute
Study ID
NCT07664709
Status
Recruiting
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Conditions

  • Churg-Strauss Syndrome (CSS)
  • GPA
  • Microscopic Polyangiitis (MPA)
  • Vasculitis

Eligibility Criteria

Sex
ALL
Age
18 Years - N/A
Healthy Volunteers
Not accepted

Interventions

  • Optical coherence tomography angiography — DIAGNOSTIC_TEST
    standardized OCTA protocol for macula 3x3 standardized OCTA protocol for optic disc 4.5x4.5

Study Details

The current state of knowledge on ANCA-associated vasculitis (AAV) indicates that it is a group of autoimmune diseases in which small blood vessels in various organs are affected. Disease entities included in this group are granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome). These are rare diseases, with an incidence in Europe of approximately 20-25 cases per million people per year. There is a slight predominance among men, and the risk of developing the disease increases with age. ANCA antibodies play a role in the pathogenesis of the disease, and inflammation within small vessels leads to damage of the vessel walls, resulting either in rupture or occlusion of the vessel lumen. Consequently, vital organs such as the kidneys, lungs, heart, nervous system, upper respiratory tract, gastrointestinal tract, and eyes may be affected. If the disease is not diagnosed, untreated, or treated improperly, it can lead to irreversible failure of these organs and even death. Despite appropriate treatment, AAV diseases tend to relapse; therefore, therapy consists of two phases: induction therapy and maintenance therapy. Current EULAR/EDTA guidelines for induction treatment of AAV recommend the use of cyclophosphamide (CYC) or rituximab (RTX) in combination with glucocorticosteroids in cases of severe disease. If remission is achieved after induction therapy, maintenance treatment should be initiated with drugs such as azathioprine, mycophenolate mofetil, methotrexate, or rituximab, combined with a low dose of glucocorticosteroids. Maintenance therapy should last no less than two years. The study will focus on ophthalmological evaluation of patients diagnosed with ANCA-associated vasculitis. In this disease, all structures of the eye may be involved. The most common ocular manifestations include scleritis, keratitis, proptosis, inflammation of orbital tissues, nasolacrimal duct obstruction, and orbital involvement leading to proptosis, double vision, and restricted eye movement. Until recently, the disease was often fatal. However, advances in diagnostics and current pharmacological treatment options, combined with appropriately aggressive immunosuppressive therapy, have significantly improved survival, enhanced patients' quality of life, and reduced mortality. Early diagnosis and prompt initiation of appropriate therapy are crucial.

Key Dates

Start date
Mar 1, 2024
Status verified
Apr 2026
Primary completion
Dec 31, 2026
Completion
Dec 31, 2026

Study Design

Enrollment
70 participants (estimated)

Arms

  • Arm: ANCA-associated vasculitis patients referred for ophthalmic assessment
    For the study, patients referred for ophthalmologic consultation from the Nephrology Clinic of the Military Institute of Medicine National Research Institute with newly diagnosed or relapsing ANCA-positive vasculitis will be enrolled. Estimated number of patients: 60. Examinations will be performed for initial ophthalmic evaluation. If more frequent visits are required (whenever ophthalmic involvement is present), additional follow-ups will be scheduled as clinical status requires. The study is planned for a minimum duration of three years. Ophthalmic examinations will include: visual acuity, intraocular pressure, and anterior and posterior segment examinations. Depending on the clinical situation and reported symptoms, additional tests will be performed * OCT and OCT angiography * neurological assessment - pupillary light reflexes, visual field, color vision * exophthalmometry, ocular motility testing * fluorescein angiography * photographic documentation * imaging studies

Primary Outcome Measure

Statistical analysis of ocular structures involvement [ Time Frame: from 01/2024 until 12/2026 ]

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