Stromal Choroiditis Research Articles

Review on imaging methods in non-infectious posterior uveitis, principles, relevance, and practical clinical applications to disease entities

The work-up and diagnosis of posterior uveitis rely heavily on multiple imaging methods that have become available beyond the mere photographic imaging and fluorescein angiography (FA) used to image uveitis in the past. Global assessment and precise follow-up of posterior uveitis were achieved with the development of indocyanine green angiography (ICGA) since the mid-1990ties that, together with FA, made it possible to perform dual FA and ICGA giving information on both the retina and the choroidal compartment. Further non-invasive imaging methods were developed subsequently that contributed to additional valuable information completing the dual FA/ICGA basic appraisal of uveitis, including (1) optical coherence tomography (OCT) giving a quasi-histological morphology of retinal structures of the posterior pole, (2) enhanced-depth imaging OCT (EDI-OCT) allowing to image the choroidal compartment and (3) blue light fundus autofluorescence (BAF) showing the integrity or damage of the retinal pigment epithelium, the photoreceptors and the outer retina. OCT-angiography (OCT-A) became available more recently and presented the advantage to image the retinal and choroidal circulations without needing dye injections, necessary for dual FA/ICGA. This review article will illustrate the principles, relevance and practical applications of these different imaging methods used in uveitis by examining the main categories of non-infectious posterior uveitis entities including (1) retinal inflammatory disorders, inflammatory diseases of the outer retina and of the choriocapillaris (choriocapillaritis) and stromal choroiditis.

Benefits and Limitations of OCT-A in the Diagnosis and Follow-Up of Posterior Intraocular Inflammation in Current Clinical Practice: A Valuable Tool or a Deceiver?

Purpose: Optical coherence tomography angiography (OCT-A) has been applied to uveitis and intraocular inflammation since its availability after 2014. The imaging of retinal and choroidal vascularization without the use of dyes was a major development and represented a potentially valuable tool in ocular research. In addition to such use, OCT-A is often put forward as being able to potentially replace invasive methods needing dye injection, such as fluorescein angiography (FA) and indocyanine green angiography (ICGA). The aim of this review was to establish whether OCT-A was sufficiently useful in everyday routine clinical practice to monitor disease evolution and to perform treatment adjustments to the extent that it could reliably replace the standard dye methods. Methods: Selective literature review and analysis of own data and experience. Results: OCT-A is a technologically high-grade imaging modality allowing to analyze retinal circulation in inflammatory diseases of the posterior pole with a high sensitivity useful for research purposes. However, there is no evidence that it reaches equal effectiveness in the routine management of posterior uveitis involving the retina. OCT-A is unable to show leakage. In choriocapillaritis involving pre-capillary vessels, it shows capillary drop-out but does not seem to have an advantage over ICGA except that it can be repeated easily, not being invasive, and so allows a closer follow-up. It is, however, less useful in end-choriocapillary non-perfusion, such as in MEWDS. For choroidal stromal inflammation, OCT-A is ill-suited as it only shows inconsistent secondary circulatory changes produced by choroidal foci. OCT-A seems to be useful in the diagnosis and follow-up of inflammatory chorioneovascularisation (iCNV), although dye exams are more precise in showing the activity of the iCNV. Conclusion: In summary, OCT-A is a very sensitive modality for the retinal circulation in uveitis for research purposes; it is sometimes useful for close follow of choriocapillary drop-out but not in end-capillary non-perfusion. Its use for monitoring purposes in stromal choroiditis, however, is questionable. Its claim to possibly replace classical angiographic work-up for the practical management of posterior uveitis is largely overrated.

Sympathetic ophthalmia in a phthisical eye with B-cell proliferation

Sympathetic ophthalmia is a dreaded ocular condition resulting in bilateral panuveitis following penetrating injury in one eye or associated with surgeries and laser procedures. We describe a case of a 13-year-old girl who presented with blurring of vision in the right eye for the past 2 years following penetrating trauma in the left eye 9 years back. Histopathology of left enucleated phthisical eye showed diffuse stromal choroiditis with B-cell proliferation on immunohistochemistry, thus confirming chronicity of the disease.

Precise, simplified diagnostic criteria and optimised management of initial-onset Vogt-Koyanagi-Harada disease: an updated review.

Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune stromal choroiditis. This review aimed to provide a novel perspective of the disease. We took into account recent developments in the understanding of the disease and crucial progress in investigational modalities of the choroid, which has led to new, simpler diagnostic criteria. We analysed recent novel notions in the literature and new diagnostic tools for VKH. We identified the following updates for VKH disease: (1) A crucial differentiation between the acute initial-onset and the chronic forms of the disease; (2) the integration of new, precise imaging methods to assess choroidal inflammation; (3) the promotion of simplified, more reliable diagnostic criteria for acute initial-onset of the disease, based on the sine qua non presence of diffuse choroiditis, detected with indocyanine green angiography (ICGA) and/or Enhanced Depth Imaging OCT (EDI-OCT); and (4) treatment optimisation through early, vigorous, sustained corticosteroid and nonsteroidal immunosuppression, as the first line of treatment for initial-onset VKH disease, and monitoring subclinical choroidal inflammation during follow-ups. Several studies have shown that most patients could discontinue treatment without an inflammation relapse. ICGA and EDI-OCT represented the methods of choice for precisely monitoring disease evolution. Simplified, precise, new diagnostic criteria allow early diagnosis of VKH. In VKH disease, inflammation exclusively originates in the choroidal stroma. Therefore, in many cases, early, sustained treatment, with dual corticosteroid and nonsteroidal immunosuppressive therapy can result in full "healing", which obviates chronic, uncontrolled, subclinical choroidal inflammation.

Classification of Non-Infectious and/or Immune Mediated Choroiditis: A Brief Overview of the Essentials.

The choroid was poorly accessible to imaging investigation until the last decade of the last century. With the availability of more precise imaging methods such as indocyanine green angiography (ICGA) and, later, optical coherence tomography (OCT), enhanced depth OCT (EDI-OCT), and OCT angiography (OCTA), appraisal of choroidal inflammation has substantially gained in accuracy. This allowed to precisely determine which structures were touched in the different non-infectious choroiditis entities and made it possible to classify this group of diseases, ICGA signs, mainly hypofluorescent lesions, were identified and described. Previous publications have divided angiographic findings into two main sets of signs: (1) irregular “geographic” hypofluorescent areas corresponding to choriocapillaris non-perfusion and (2) round more regular, hypofluorescent dark dots more evenly distributed in the fundus corresponding to more deep choroidal stromal foci. These distinct findings allowed to subdivide and classify choroiditis into choriocapillaritis and stromal choroiditis. Additional signs were identified from EDI-OCT and OCTA examination supporting the classification of choroiditis into choriocapillaritis and stromal choroiditis. Results: Diseases involving principally the choriocapillaris included Multiple Evanescent White Dot Syndrome (MEWDS), Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE), Idiopathic Multifocal Choroiditis (MFC), and Serpiginous Choroiditis (SC) as well as mixed forms. Diseases primarily involving the choroidal stroma included HLA-A29 Birdshot Retinochoroiditis (BRC), Vogt-Koyanagi-Harada disease (VKH), Sympathetic Ophthalmia (SO), and Sarcoidosis chorioretinitis (SARC). Thanks to new imaging investigations of the choroid, it is now possible to classify and understand the diverse clinicopathological mechanisms in the group of non-infectious choroiditis entities.

White dot syndromes : Principles, diagnostics, and treatment

The white dot syndromes include a group of diseases which are characterized by multiple yellowish-white foci in the outer retina, retinal pigment epithelium, and choroid. For clinicians and researchers alike they present significant diagnostic and therapeutic challenges. White dot syndromes include primary inflammatory choriocapillaropathies, such as acute posterior multifocal placoid pigment epitheliopathy (APMPPE)/acute multifocal ischemic choriocapillaropathy (AMIC), multiple evanescent white dot syndrome (MEWDS)/acute idiopathic blind spot enlargement (AIBSE), multifocal choroiditis (MFC), punctate inner choroidopathy (PIC), serpiginous choroiditis (SC), acute zonal occult outer retinopathy (AZOOR), and acute macular neuroretinopathy (AMN). Among the primary stromal choroiditis is birdshot retinochoroidopathy (BSRC); however, the pathogenesis of these disorders is largely unknown. Immunological reactions to previous viral infections with a genetic disposition seem to be acommon denominator.

Vogt-Koyanagi-Harada Disease and Birdshot Retinochoroidopathy, Similarities and Differences: A Glimpse into the Clinicopathology of Stromal Choroiditis, a Perspective and a Review.

The purpose of this work was to give a comprehensive and updated review on two primary stromal choroiditis entities, Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC). Their appraisal has become much more precise thanks to new investigational methods, such as indocyanine green angiography (ICGA) and enhanced depth imaging optical coherence tomography (EDI-OCT), which give substantially improved imaging access to the choroid. In this review, we focus on the crucial changes brought by this progress in the understanding, diagnosis, and management of these disorders. Application of these methods makes it possible to reach an early diagnosis, therefore allowing early treatment, which has led to a profound improvement in outcomes when compared to previous management.

Sensitivity of indocyanine green angiography compared to fluorescein angiography and enhanced depth imaging optical coherence tomography during tapering and fine-tuning of therapy in primary stromal choroiditis: A case series

PurposeTo investigate indocyanine green angiography (ICGA), fluorescein angiography (FA), and enhanced depth imaging optical coherence tomography measured choroidal thickness (EDI-OCT-CT) in the follow-up of inflammatory activity in stromal choroiditis [Vogt-Koyanagi-Harada disease (VKH) and birdshot retinochoroiditis (BRC)] under treatment in order to monitor tapering of therapy or readjustment of therapy in case of subclinical disease recurrence. MethodsPatients with initial onset disease and/or treatment-naive stromal choroiditis (VKH & BRC) at entry, quiet under therapy, and having had a follow-up of at least four years monitored with dual FA and ICGA and EDI-OCT-CT measurements were analyzed retrospectively. ICGA and FA scores and EDI-OCT-CT values were correlated with therapy, and significant changes of each modality were correlated with disease evolution. ResultsOf the 31 VKH and 29 BRC patients seen from 1995 to 2017 in our center, four patients (2 VKH and 2 BRC patients) fulfilled the inclusion criteria. During tapering, two patients (both VKH) showed no significant ICGA, FA, and EDI-OCT-CT changes (mean follow-up time 5.6 years) and allowed for safe tapering. In the other two (BRC) patients (mean follow-up time 6.25 years), a total of seven significant subclinical changes were demonstrated by ICGA alone after therapy modifications due to side-effects or during attempted tapering of therapy, while FA and EDI-OCT-CT remained unchanged. ConclusionsICGA was the most sensitive monitoring modality of stromal choroiditis, able to identify subclinical recurrences following change of therapy and inversely treatment responses after readjusted therapy, events otherwise missed by FA and EDI-OCT. ICGA proved efficient for safe therapy tapering or for timely adjustment of therapy in stromal choroiditis when necessary.

Fine‐tuning of therapy in stromal choroiditis using indocyanine green angiography (ICGA)

Fine‐tuning of therapy in stromal choroiditis using indocyanine green angiography (ICGA)

Choroiditis (non‐infectious)

SummaryChoroiditis (non‐infectious) Non‐infectious choroiditis is classically subdivided into at least two main categories, including choriocapillaritis and stromal choroiditis. In the latter, the site of inflammation is situated in the choroidal stroma. When inflammation exclusively originates from the choroidal stroma which is the target of an immune reaction such as in Vogt‐Koyanag‐Harada disease (VKH), sympathetic Ophthalmia (SO) or birdshot retinochoroiditis (BRC), the term of primary stromal choroiditis is used. When the choroidal stroma is a chance and random location of a systemic disease such as sarcoidosis, the term of secondary stromal choroiditis is used. In the choriocapillaritis group the lesion process is an inflammatory non perfusion of the choriocapillaris, a mechanism occurring or suspected to occur in diseases such as multiple evanescent white dots syndrome (MEWDS), (idiopathic) multifocal choroiditis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The difference of these two mechanism will be further presented with the help of practical examples presented in the same fashion as the “viva voce” EBO examination.

Primary stromal choroiditis

SummaryOptimal outcome measures are necessary to monitor adequately the diverse uveitis entities and follow‐up modalities should be tailored according to the disease process. Stromal choroiditis entities are diseases predominantly involving the choroidal stroma, such as Vogt‐Koyanagi‐Harada disease (VKH) and birdshot retinochoroiditis (BRC), both considered as primary stromal choroiditis because the disease process starts within the stroma of the choroid. Sarcoidosis and tuberculous chorioretinitis are considered as secondary choroiditis, as the choroid is the innocent bystander structure where a systemic disease causes lesions Consequently a follow‐up modality capable of optimally evaluating this structure should be privileged in the assessement at presentation and for monitoring disease evolution. Among imaging modalities, the ones best accounting for choroidal stromal involvement are indocyanine green angiography (ICGA) and measurement of choroidal thickness by enhanced depth imaging optical coherence tomography (EDI‐OCT). ICGA is more suited for acute and subacute stages of disease, while EDI‐OCT useful to follow medium and long‐term evolution. The precision obtained with these modalities will be demonsrated.

Contribution of dual fluorescein and indocyanine green angiography to the appraisal of posterior involvement in birdshot retinochoroiditis and Vogt-Koyanagi-Harada disease.

To assess the levels of retinal and choroidal involvement in initial-onset birdshot retinochoroiditis (BRC) and Vogt-Koyanagi-Harada (VKH) disease, two stromal choroiditis entities. This retrospective study included patients diagnosed with BRC and VKH, seen during initial-onset disease at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified, using an established dual fluorescein angiography (FA) and indocyanine green angiography (ICGA) scoring system for uveitis, and the FA/ICGA score ratios were compared between diseases. Among 1793 patients with uveitis seen from 1995 to 2015, 7 newly diagnosed BRC patients and 4 patients with newly diagnosed VKH disease had sufficient data for study inclusion. Patients with BRC and VKH at initial onset had mean FA angiographic scores of 16.91±3.42 and 4.06±1.87; mean ICGA angiographic scores of 21.34±3.49 and 25.75±3.88; and mean FA/ICGA ratios of 0.79±0.21 and 0.16±0.09, respectively. This study showed the differential involvements of the retina and choroid in BRC and VKH. The choroid was preponderantly involved in both diseases; thus, ICGA is essential for disease assessment and follow-up. However, these diseases also differed substantially. The origin of inflammation was primarily in the choroid in VKH and in both the choroid and retina in BRC. We recommend dual FA and ICGA for evaluating posterior uveitis, when choroiditis is suspected.

Inflammatory choroiditis

SummaryNon‐infectious choroiditis is classically subdivided into at least two main categories, including choriocapillaritis and stromal choroiditis. In the latter, the site of inflammation is situated in the choroidal stroma. When inflammation exclusively originates from the choroidal stroma which is the target of an immune reaction such as in Vogt‐Koyanag‐Harada disease (VKH), sympathetic Ophthalmia (SO) or birdshot retinochoroiditis (BRC), the term of primary stromal choroiditis is used. When the choroidal stroma is a chance and random location of a systemic disease such as sarcoidosis, the term of secondary stromal choroiditis is used. In the choriocapillaritis group the lesion process is an inflammatory non perfusion of the choriocapillaris, a mechanism occurring or suspected to occur in diseases such as multiple evanescent white dots syndrome (MEWDS), multifocal choroiditis, acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The difference of these two mechanism will be further presented with the help of practical examples presented in the same fashion as the ‘viva voce’ EBO examination.

Contribution of dual fluorescein and indocyanine green angiography to the appraisal of posterior involvement in birdshot retinochoroiditis and Vogt‐Koyanagi‐Harada disease

PurposeTo assess the levels of retinal versus choroid involvement in two stromal choroiditis entities, birdshot retinochoroiditis (BRC) and Vogt‐Koyanagi‐Harada (VKH) disease in initial onset non treated disease.MethodsThis retrospective study included patients diagnosed with BRC and VKH, seen during initial onset disease at the Centre for Ophthalmic Specialized Care, Lausanne, Switzerland. Angiographic signs were quantified, using an established dual FA and ICGA scoring system for uveitis, and the FA/ICGA ratios were compared between diseases.ResultsAmong 1,739 patients with uveitis seen from 1995 to 2014, 7 newly diagnosed BRC patients and 4 patients with newly diagnosed VKH disease were included in the study. Patients with BRC and VKH had mean FA angiographic scores of 16.64 ± 3.75 and 4.25 ± 1.83; mean ICGA angiographic scores of 21.00 ± 3.48 and 25.25 ± 3.84; and mean FA/ICGA ratios of 0.79 ± 0.21 and 0.17 ± 0.09, respectively.ConclusionsThis study showed the differential involvements of the retina and choroid in BRC and VKH. Choroid was predominantly involved in both diseases, which clearly indicated that ICGA was the method of choice, and that it should be performed in conjunction with FA. Also, the FA/ICGA ratio highlighted the fundamental differences between BRC and VKH. VKH had a purely choroidal origin of inflammation, and BRC had both retinal and choroidal origins of inflammation.

EDI‐OCT is less suited for close monitoring of primary stromal choroiditis when compared to Indocyanine green angiography

PurposeInvestigate the performance, utility, and precision, of enhanced depth imaging optical coherence tomography thickness (EDI‐OCT‐T), versus indocyanine green angiography (ICGA), in tracking any fluctuation in the activity of primary stromal choroiditis (PSC) in response to therapy during long‐term follow‐up.MethodsPatients with a diagnosis of PSC; Vogt Koyanagi Harada (VKH) or birdshot retinochoroiditis (BRC), with untreated initial disease with long‐term follow‐up including both ICGA and EDI‐OCT, were recruited at the Centre for Ophthalmic Specialized care, Lausanne, Switzerland. Angiography signs were quantified according to established dual FA and ICGA scoring systems for uveitis. Changes in ICGA score, and EDI‐OCT‐T, in response to therapy, were assessed.ResultsAmong 1829 uveitis patients seen from 1995 to 2015, 59 patients (3.2%) were diagnosed with PSC of which 4 patients (2 BRC and 2 VKH) fulfilled the restrictive inclusion criteria. Mean EDI‐OCT‐T decreased from 672 ± 101 μm at entry to 358.5 ± 44.5 μm in a mean of 26.5 months at stabilization. Mean ICGA scores decreased from 28 ± 4.2 at entry, to 5 ± 7 at stabilization. Only ICGA was sufficiently sensitive and reactive to detect disease recurrencies and efficacy or absence of effect of successive treatment changes, detected in 7 instances during follow‐up, but not recorded by EDI‐OCT.ConclusionsThis pilot study showed that ICGA was the more sensitive methodology; able to promptly identify evolving subclinical and occult choroidal disease and flag occult recurrence and/or therapeutic responses that were otherwise missed by EDI‐OCT. Although EDI‐OCT‐T showed a linear decrease, these changes were too sluggish to be relied upon for close follow‐up and timely adjustment of therapy.

Stromal choroiditis in East (VKH) and West (birdshot)

SummaryBackground and aimBoth Vogt‐Koyanagi‐Harada (VKH) disease and birdshot retinochoroiditis (BRC) are primary stromal choroiditis entities, meaning that the initial inflammatory process necessarily starts in the choroidal stroma. The aim here was to point out similarities and differences between these two stromal choroiditis entities and to show that early treatment is able to modify the phenotypes of both conditions.Patients and methodsTwo groups of patients respectively of VKH and BRC cases were analyzed and followed clinically and angiographically using dual fluorescein (FA) and indocyanine green (ICGA) angiography.Results: 13 of 28 BRC patients and 11 of 24 VKH patients had enough follow‐up data to be included in the study. Early diagnosis and treatment in both VKH (9/11) and BRC (6/13), allowed to avoid sunset glow fundus in the former and BRC fundus lesions in the latter. The main similarities and differenced will be pointed out.ConclusionVKH and BRC differ substantially from each other. However choroidal stromal inflammation is characterized by a similar process and responds equally to therapy.

Inflammatory choroiditis

SummaryWhite dot syndromes (WDS) is a term introduced around 1995 to describe posterior uveitis syndromes that were poorly understood such as MEWDS, APMPPE, multifocal choroiditis (MFC), serpiginous choroiditis (SC), birdshot retinochoroiditis (BRC) and many others depending on the extension with which the term is used. Unfortunatelly the term is of no utility as it is purely based on the the white dots most posterior uveitis exhibit and as it emcompasses entities that look alike but have nothing in common as far as mechanism is concerned. Thanks to indocyanine green angiography (ICGA) it became possible to get away from this pot‐pourri terminology and allowed to sort out choroiditis entities according to the pathophysiological mechanism subdividing choroiditis into diseases of the choriocapillaris (primary choriocapillaritis) including MEWDS, APMPPE, MFC, SC and atypical and overlapping entities on one side and stromal choroiditis on the other side including Vogt‐Koxanangi‐Harada disease (VKH), BRC, sarcoid and tubercular choroiditis. The appraisal of these diseases and the rationale of their new classification will be explained and examples will be given to illustrate this new comprehensive approach that should make WDS obsolete.

White dot syndromes

Abstract"White dot syndromes" (WDS)is a term introduced around 1995 to describe posterior uveitis syndromes that were poorly understood such as MEWDS, APMPPE, multifocal choroiditis (MFC), serpiginous choroiditis (SC), birdshot retinochoroiditis (BRC) and many others depending on the extension with which the term is used. Unfortunatelly the term is of no utility as it is purely based on the the white dots most posterior uveitis exhibit and as it emcompasses entities that look alike but have nothing in common as far as mechanism is concerned. Thanks to indocyanine green angiography (ICGA) it became possible to get away from this pot‐pourri terminology and allowed to sort out choroiditis entities according to the pathophysiological mechanism subdividing choroiditis into diseases of the choriocapillaris (primary choriocapillaritis) including MEWDS, APMPPE, MFC, SC and atypical and overlapping entities on one side and stromal choroiditis on the other side including Vogt‐Koxanangi‐Harada disease (VKH), BRC, sarcoid and tubercular choroiditis. The appraisal of these diseases and the rationale of their new classification will be explained and examples will be given to illustrate this new comprehensive approach that should make WDS obsolete.

Early and sustained treatment modifies the phenotype of birdshot retinochoroiditis

In this single-centre retrospective case review, we investigate the long-term follow-up of birdshot retinochoroiditis (BRC) patients, analysing the impact of early, vigorous, and prolonged treatment on the evolution of indocyanine green angiography (ICGA) signs and fundus appearance. Treatment delay was calculated for each BRC patient, and patients were classified into two groups--treatment delay of <10 months (early-treatment group) and treatment delay of >10 months (delayed-treatment group). Fundus photographs and ICGA frames from the initial visit and from the last follow-up visit were assessed. Fundus photographs were evaluated for the presence of at least three circumpapillary, typical, rice-shaped birdshot lesions in one eye, inferior or nasal to the optic disc. ICGA pictures were evaluated for the presence of lesions (hypofluorescent dark dots, fuzziness). Differences were compared between the two groups and between the first visit and the last follow-up visit. In the early-treatment group, 5/6 patients had no characteristic BRC fundus lesions, but 7/7 patients in the delayed-treatment group displayed typical lesions. At last follow-up, 5/6 early-treatment patients showed no fundus lesions, and 6/7 delayed-treatment patients retained their fundus lesions. At presentation, all 13 patients exhibited lesions on ICGA. At last follow-up, ICGA lesions had completely disappeared in 4/6 early-treatment patients and 3/7 delayed-treatment patients. Thus, early and sufficiently dosed inflammation-suppressive treatment can prevent the appearance of typical BRC fundus lesions. It is therefore crucial to perform ICGA to detect otherwise occult stromal choroiditis in suspected BRC cases and to initiate adequate therapy immediately.

Pathophysiology of the development of intraocular inflammation in VKH

AbstractChoroiditis has recently been classified and subdivided into choriocapillaritis when the impact of inflammation is at the level of the choriocapillsris and into stromal choroiditis when the inflammation is situated in the choroidal stroma. The choroidal stroma can be the chance localization of an infectious or non‐infectious systemic disease such as tuberculosis or sarcoidosis and is then termed secondary stromal choroiditis. The situation is different in Vogt‐Koyanagi‐Harada disease (VKH)(and birdshot retinochoroiditis) where the target of the inflammatory reaction is selectively situated in the choroidal stroma and should therefore be called primary stromal choroiditis. The choroidal inflammation develops first silently while it is still confined to the stroma. Probably this phase corresponds to the proromal stage. It is only when the inflammation spills over, usually in an explosive fashion, into neighbouring compartments such as the retina, optic disc and vitreous that the disease becomes clinically apparent.The fact that the choroidal stroma is the primum movens means that the follw‐up of choroidaql inflammation by ICGA is crucial and that therapy has a good impact as long as sustained ICGA assisted therapy is applied