Ocular Manifestations of Granulomatosis With Polyangiitis.

NCT07664709 | Recruiting

• 2 other identifiers: 8/2024/KBMIMNRI 626
• Study Type: observational
• Target: 70
• Locations: 1 country
• Sites: 1

Brief Summary

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.

Conditions

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

Keywords

ocular symptoms; octa; ocular manifestations; scleritis GPA; gpa ocular involvement

Outcome Measures

Primary Outcomes (1)

• Statistical analysis of ocular structures involvement

  Based on data from other populations in these rare disorders, all ocular structures can be affected. The most common ocular manifestations include scleritis, keratitis, proptosis, orbital tissue inflammation, nasolacrimal duct obstruction, orbital involvement with proptosis, diplopia, and restricted ocular motility. The epidemiology data will be collected and presented in percenage data

  from 01/2024 until 12/2026

Secondary Outcomes (1)

• Vessel structure and density measured with OCT angiography compared to control group.

  1/2024-12/2026

Study Arms (1)

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

Diagnostic Test: Optical coherence tomography angiography

Interventions

Optical coherence tomography angiography DIAGNOSTIC_TEST

standardized OCTA protocol for macula 3x3 standardized OCTA protocol for optic disc 4.5x4.5

ANCA-associated vasculitis patients referred for ophthalmic assessment

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

ANCA positive vasculitis with any systemic symptoms Patients either with early onset of the disease or under treatment

You may qualify if:

• ANCA positive vasculitis age 18- no limit patients with onset od the disease and patients already under treatment

You may not qualify if:

• no consent for ophthalmic examination
• inability to udergo ophthalmic examination

Sponsors & Collaborators

• Military Institute od Medicine National Research Institute: lead

Study Sites (1)

Military Institute of Medicine National Research Institute

Warsaw, 04-141, Poland

RECRUITING

Related Publications (1)

• Byszewska A, Skrzypiec I, Rymarz A, Niemczyk S, Rekas M. Ocular Involvement of Granulomatosis with Polyangiitis. J Clin Med. 2023 Jul 2;12(13):4448. doi: 10.3390/jcm12134448.

  PMID: 37445483 BACKGROUND

MeSH Terms

Conditions

Vasculitis; Microscopic Polyangiitis; Churg-Strauss Syndrome

Condition Hierarchy (Ancestors)

Vascular Diseases; Cardiovascular Diseases; Cerebral Small Vessel Diseases; Cerebrovascular Disorders; Brain Diseases; Central Nervous System Diseases; Nervous System Diseases; Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis; Systemic Vasculitis; Skin Diseases, Vascular; Skin Diseases; Skin and Connective Tissue Diseases; Autoimmune Diseases; Immune System Diseases; Granuloma; Lymphoproliferative Disorders; Lymphatic Diseases; Hemic and Lymphatic Diseases

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: PROSPECTIVE
• Target Duration: 1 Day
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: June 17, 2026
• First Posted: June 24, 2026
• Study Start: March 1, 2024
• Primary Completion (Estimated): December 31, 2026
• Study Completion (Estimated): December 31, 2026
• Last Updated: June 24, 2026

Record last verified: 2026-04

Data Sharing

• IPD Sharing: Will not share

Locations

PL (1)