Ignite Creation Date:
2024-07-17 @ 11:10 AM
Last Modification Date:
2024-10-26 @ 3:34 PM
Study NCT ID:
NCT06500728
Status:
RECRUITING
Last Update Posted:
2024-07-15
First Post:
2024-07-07
Brief Title:
Visual Involvement in Giant Cell Arteritis
Sponsor:
ASST Fatebenefratelli Sacco
Organization:
ASST Fatebenefratelli Sacco
Study Overview
Official Title:
Visual Involvement in Giant Cell Arteritis
Status:
RECRUITING
Status Verified Date:
2024-07
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
Visu-GCA
Brief Summary:
This observational study aims to enhance the description of the different ways Giant Cell Arteritis GCA affects vision The latest technology and knowledge are used to improve how we diagnose and predict patient outcomes GCA is the most frequent vasculitis an inflammation of vessels in older adults It involves large and medium-sized arteries and causes ischemic alterations such as stroke and blindness through damage of extracranial arteries
The primary objective is to compare the frequency of the various ocular findings between the main alterations of arteritic and non-arteritic aetiology such as Arteritic Anterior Ischemic Optic Neuropathy A-AION Vs Non-Arteritic Anterior Ischemic Optic Neuropathy NA-AION or Central Retinal Artery Occlusion CRAO from GCA Vs from other causes through a comprehensive clinical and instrumental evaluation
The primary objective is to compare the frequency of the various ocular findings between the main alterations of arteritic and non-arteritic aetiology such as Arteritic Anterior Ischemic Optic Neuropathy A-AION Vs Non-Arteritic Anterior Ischemic Optic Neuropathy NA-AION or Central Retinal Artery Occlusion CRAO from GCA Vs from other causes through a comprehensive clinical and instrumental evaluation
Detailed Description:
Giant-cell arteritis GCA is an idiopathic inflammatory condition affecting medium- and large-sized arteries This condition typically affects females over 50 with a peak incidence in the eighth decade of life It is a rare disease with an annual incidence in southern Europe including Italy of approximately 12100000 inhabitants aged 50 years This clinical condition has considerable heterogeneity among patients with different clinical phenotypes recognised predominantly involving the large vessel LV-GCA and the medium-sized arteries of the cephalic district cGCA The main complications of cephalic involvement are ischaemic events such as stroke and optic involvement Visual involvement is a minor but the main prognostic factor in patients with GCA as it can lead to irreversible vision loss Patients with visual involvement often exhibit other disease features but with a less intense inflammatory response compared to subjects without visual involvement Ocular involvement occurs with a wide clinical spectrum of ocular manifestations from amaurosis fugax and diplopia to permanent loss of visual capacity This irreversible or partially reversible visual impairment is mainly linked to three mechanisms
Arteritic anterior ischaemic optic neuropathy A-AION which is present in 90 of cases of irreversible vision loss it is secondary to ischaemic involvement of the short posterior ciliary arteries that supply the optic nerve head direct ophthalmoscopy shows typically an oedematous and pale optic disc with a resolution in cupping characteristics like in glaucomatous optic neuropathy after 4 weeks In the presence of cilio-retinal artery the vascular territory of this arterial variation could be involved This ophthalmological image is being considered for differential diagnosis with non-arteritic anterior ischemic optic neuropathy NA-AION NA-AION is caused by a compartment syndrome that occurs at the level of the optic nerve head This is triggered by even transient hypoperfusion that causes ischemic swelling in an area with little room to expand at the level of the lamina cribrosa As the therapy is completely different the differentiation between A-AION and NA-AION is crucial Hayreh et al differ these conditions according to the extra-ocular features of GCA and the ophthalmological characteristics presence of pallorpapillary haemorrhages cilio-retinal occlusion if arising from a territory with choroidal ischaemia and evidence of choroidal ischaemia or delayed choroidal perfusion
In 15 of cases internal retinal ischaemia occurred during GCA due to involvement of the central retinal artery CRAO or one of its branches BRAO Direct ophthalmoscopy shows peripheral retinal whitening in contrast to the cherry-red macula Sub-occlusive involvement of the retinal vasculature provides necrotic spots of certain retinal layers providing superficial cottony exudates and deep paracentral acute middle maculopathy PAMM PAMM was first described in 2013 A single study about 52 patients with visual GCA observed PAMM in 26 of patients either isolated or in association with other forms of visual involvement However this diagnosis requires evaluation by Optical Coherence Tomography OCT
In 5 of cases of GCA posterior ischemic optic neuropathy PION occurs due to altered circulation in the retrobulbar optic nerve No typical retinal or optic nerve changes are evident on direct ophthalmoscopy
Various unmet needs in the ophthalmological literature could impact on the management of patients with acute visual impairment in suspected GCA
1 Semeiological aspects of the rare visual phenotypes are described in studies with sample sizes ranging between 8 and 85 subjects Multicenter studies with the largest sample sizes and ten-year recruitment do not explain deeply all ophthalmological pictures meanwhile small studies interfere with a clear description of the entire spectrum of ophthalmic semeiotic manifestations highlighting only the most frequent phenotypes and making diagnosis very complex to date In this center among 60 patients enrolled in the last 3 years 5 patients with PAMM and 2 with atypical A-AION were described complicating the diagnostic-therapeutic procedure
2 Types of enrolled GCA patients are a limiting aspect due to the evolution of vasculitis definition diagnosis and management ie last updated in American College of Rheumatology guidelines of 2022 and awareness about diagnosis delay and visual prognosis Therefore it is important to define ophthalmological manifestations and frequency distributions in enrolled patients according to recent criteria and current clinical practices
3 Many studies with an ophthalmological focus were published several years ago New ophthalmological clinical pictures have been recognised such as PAMM in 2013 and ophthalmology-focused instrumental technology has advanced considerably such as high-OCT Optical Coherence Resonance OCR and angio-Optical Coherence Tomography OCT-A Moreover considering that extra-ocular manifestations of GCA may be absent in approximately 20 of patients with visual involvement it is particularly important to update semeiological knowledge and predictive and prognostic values
4 PAMM in GCA was described in a few epidemiological studies with a small sample size Although PAMM could represent the second most frequent form of visual impairment in GCA OCT is not yet widely used andor practised in comparison with traditional methods such as ophthalmoscopy and fluoroangiography
This observational study aims to improve the ophthalmological description of different visual involvement phenotypes in GCA This will be achieved by utilizing state-of-the-art technology and nosographic knowledge to improve patient diagnosis and prognostic stratification
The primary objective is a comparison of the frequency of the various semeiological findings by multi-parametric evaluation among the main pathological ocular alterations of arteritic and non-arteritic aetiology eg A-AION Vs NA-AION CRAO from arteritis Vs CRAO from other causes The second objectives are
Prevalence of the GCA visual phenotypes including the potential co-existence of several of them eg paracellular retinal ischemia as in PAMM choroidal and papillary ischemia
Integration of clinical findings with ophthalmological methods such as visual acuity campimetry retinal fluorangiography OCT and OCT-A in various pathological visual conditions
Temporal evolution of the visual acuity and semeiological findings after therapy and correlation with prognosis
This prospective study enrolls patients referred to the emergency room or ophthalmology outpatient clinic for new-onset visual symptoms for which they will perform the clinical laboratory and instrumental examinations required by existing clinical practice For patients with suspected GCA venous blood samples 18 ml per sample are scheduled at baseline and at times 7 days 3 months and 6 months
Clinical management and treatment will follow international recommendations per the 2021 American College of Rheumatology ACR and 2018 European Alliance of Associations for Rheumatology EULAR guidelines due to the progression of acute visual impairment leading to permanent visual loss Ophthalmological assessment will be scheduled at baseline T0 which is repeated after 48-72 hours T1 7 2 days T2 4 1 weeks T3 12 2 weeks T4 and 26 2 weeks T5 At each time point the evaluation includes an assessment of visual acuity fundus and visual field The ophthalmologist frequently recommends fluorescein FAG and indocyanine green angiography ICGA OCT with high-resolution technique and OCT-A In addition to the ophthalmological assessment patients will also undergo an internal and immuno-rheumatological evaluation to address the management and treatment of the underlying condition causing the visual impairment Internal or immuno-rheumatology follow-up will depend on the underlying diagnosis and follow normal clinical practice
In case of suspected ocular flare-up the ophthalmologist may consider performing a full or partial ophthalmological work-up based on clinical need The clinical-instrumental data obtained from the ophthalmological assessments will be collected in a standardised electronic database according to the variables described in the case report form CRF
Arteritic anterior ischaemic optic neuropathy A-AION which is present in 90 of cases of irreversible vision loss it is secondary to ischaemic involvement of the short posterior ciliary arteries that supply the optic nerve head direct ophthalmoscopy shows typically an oedematous and pale optic disc with a resolution in cupping characteristics like in glaucomatous optic neuropathy after 4 weeks In the presence of cilio-retinal artery the vascular territory of this arterial variation could be involved This ophthalmological image is being considered for differential diagnosis with non-arteritic anterior ischemic optic neuropathy NA-AION NA-AION is caused by a compartment syndrome that occurs at the level of the optic nerve head This is triggered by even transient hypoperfusion that causes ischemic swelling in an area with little room to expand at the level of the lamina cribrosa As the therapy is completely different the differentiation between A-AION and NA-AION is crucial Hayreh et al differ these conditions according to the extra-ocular features of GCA and the ophthalmological characteristics presence of pallorpapillary haemorrhages cilio-retinal occlusion if arising from a territory with choroidal ischaemia and evidence of choroidal ischaemia or delayed choroidal perfusion
In 15 of cases internal retinal ischaemia occurred during GCA due to involvement of the central retinal artery CRAO or one of its branches BRAO Direct ophthalmoscopy shows peripheral retinal whitening in contrast to the cherry-red macula Sub-occlusive involvement of the retinal vasculature provides necrotic spots of certain retinal layers providing superficial cottony exudates and deep paracentral acute middle maculopathy PAMM PAMM was first described in 2013 A single study about 52 patients with visual GCA observed PAMM in 26 of patients either isolated or in association with other forms of visual involvement However this diagnosis requires evaluation by Optical Coherence Tomography OCT
In 5 of cases of GCA posterior ischemic optic neuropathy PION occurs due to altered circulation in the retrobulbar optic nerve No typical retinal or optic nerve changes are evident on direct ophthalmoscopy
Various unmet needs in the ophthalmological literature could impact on the management of patients with acute visual impairment in suspected GCA
1 Semeiological aspects of the rare visual phenotypes are described in studies with sample sizes ranging between 8 and 85 subjects Multicenter studies with the largest sample sizes and ten-year recruitment do not explain deeply all ophthalmological pictures meanwhile small studies interfere with a clear description of the entire spectrum of ophthalmic semeiotic manifestations highlighting only the most frequent phenotypes and making diagnosis very complex to date In this center among 60 patients enrolled in the last 3 years 5 patients with PAMM and 2 with atypical A-AION were described complicating the diagnostic-therapeutic procedure
2 Types of enrolled GCA patients are a limiting aspect due to the evolution of vasculitis definition diagnosis and management ie last updated in American College of Rheumatology guidelines of 2022 and awareness about diagnosis delay and visual prognosis Therefore it is important to define ophthalmological manifestations and frequency distributions in enrolled patients according to recent criteria and current clinical practices
3 Many studies with an ophthalmological focus were published several years ago New ophthalmological clinical pictures have been recognised such as PAMM in 2013 and ophthalmology-focused instrumental technology has advanced considerably such as high-OCT Optical Coherence Resonance OCR and angio-Optical Coherence Tomography OCT-A Moreover considering that extra-ocular manifestations of GCA may be absent in approximately 20 of patients with visual involvement it is particularly important to update semeiological knowledge and predictive and prognostic values
4 PAMM in GCA was described in a few epidemiological studies with a small sample size Although PAMM could represent the second most frequent form of visual impairment in GCA OCT is not yet widely used andor practised in comparison with traditional methods such as ophthalmoscopy and fluoroangiography
This observational study aims to improve the ophthalmological description of different visual involvement phenotypes in GCA This will be achieved by utilizing state-of-the-art technology and nosographic knowledge to improve patient diagnosis and prognostic stratification
The primary objective is a comparison of the frequency of the various semeiological findings by multi-parametric evaluation among the main pathological ocular alterations of arteritic and non-arteritic aetiology eg A-AION Vs NA-AION CRAO from arteritis Vs CRAO from other causes The second objectives are
Prevalence of the GCA visual phenotypes including the potential co-existence of several of them eg paracellular retinal ischemia as in PAMM choroidal and papillary ischemia
Integration of clinical findings with ophthalmological methods such as visual acuity campimetry retinal fluorangiography OCT and OCT-A in various pathological visual conditions
Temporal evolution of the visual acuity and semeiological findings after therapy and correlation with prognosis
This prospective study enrolls patients referred to the emergency room or ophthalmology outpatient clinic for new-onset visual symptoms for which they will perform the clinical laboratory and instrumental examinations required by existing clinical practice For patients with suspected GCA venous blood samples 18 ml per sample are scheduled at baseline and at times 7 days 3 months and 6 months
Clinical management and treatment will follow international recommendations per the 2021 American College of Rheumatology ACR and 2018 European Alliance of Associations for Rheumatology EULAR guidelines due to the progression of acute visual impairment leading to permanent visual loss Ophthalmological assessment will be scheduled at baseline T0 which is repeated after 48-72 hours T1 7 2 days T2 4 1 weeks T3 12 2 weeks T4 and 26 2 weeks T5 At each time point the evaluation includes an assessment of visual acuity fundus and visual field The ophthalmologist frequently recommends fluorescein FAG and indocyanine green angiography ICGA OCT with high-resolution technique and OCT-A In addition to the ophthalmological assessment patients will also undergo an internal and immuno-rheumatological evaluation to address the management and treatment of the underlying condition causing the visual impairment Internal or immuno-rheumatology follow-up will depend on the underlying diagnosis and follow normal clinical practice
In case of suspected ocular flare-up the ophthalmologist may consider performing a full or partial ophthalmological work-up based on clinical need The clinical-instrumental data obtained from the ophthalmological assessments will be collected in a standardised electronic database according to the variables described in the case report form CRF
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None