Evidence and Consensus Based Guidelines for Imaging in Serpiginous Choroiditis - Multimodal imaging in Uveitis (MUV) Taskforce: Report 4

Purpose: To develop imaging and consensus-based guidelines for the application of multimodal imaging in serpiginous choroiditis (SC). Design: Consensus agreement guided by literature search, and an expert committee using a nominal group technique (NGT). Methods: An expert committee gathered cases of non-infectious SC based on pre-defined informatics-driven inclusion criteria. These criteria focused primarily on high-quality imaging, including color fundus photographs (CFP), optical coherence tomography (OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA). A structured NGT was applied to achieve consensus-based recommendations on imaging use for specific disease characteristics, biomarkers of activity, and complications. These recommendations were further voted upon by members of the full task force. Results: CFP and FAF are key imaging modalities for the diagnosis of SC. SC is characterized by amoeboid/serpentine choroiditis lesions on CFP, with hypo-autofluorescence in older inactive lesions and hyper-autofluorescence along the broad active edges. FAF is the most important tool for assessing disease activity. ICGA findings, particularly at the leading active edge, and OCT further aid in disease characterization. Early hypofluorescence of the active edge with uniform hyperfluorescent staining in the late phase of FFA are key features that help differentiate SC from other placoid chorioretinopathies. FFA, OCT and OCTA are useful in identifying underlying choroidal neovascularization, with OCTA being an important modality. Conclusions: MUV imaging criteria for SC extend the Standardization of Uveitis Nomenclature (SUN) classification, by providing guidelines for the use of multimodal imaging. These criteria also provide a framework for evaluation of disease activity and complications

Evidence and Consensus-based Multimodal Imaging Guidelines in Birdshot Chorioretinopathy - Multimodal imaging in Uveitis (MUV) Taskforce Report 8

Purpose: To develop consensus-based imaging guidelines for diagnosing and monitoring birdshot chorioretinopathy (BSCR). Design: Consensus-based approach guided by literature and an expert committee using a nominal group technique (NGT). Methods: An expert committee of five international uveitis specialists reviewed 15 well-documented representative BSCR cases with comprehensive imaging data. Cases with active and inactive disease were included. Imaging, including color fundus photography (CFP), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), fundus autofluorescence (FAF), and OCT angiography (OCTA) were reviewed. Using a structured NGT approach, consensus-based recommendations were developed for specific disease characteristics, biomarkers of activity, and complications. The recommendations were voted upon by members of the full task force. Results: For the diagnosis of BSCR, CFP, FFA, and ICGA were identified as the key imaging modalities. ICGA was identified as a key imaging modality for assessing the presence of choroidal lesions. FFA was deemed crucial for monitoring retinal vascular leakage and assessing the treatment response. OCT, while not essential for diagnosis, was valuable for detecting complications such as cystoid macular edema and retinal thinning. The committee did not reach a consensus on the role of FAF and OCTA for the diagnosis or monitoring of BSCR. Conclusions: The MUV consensus-based imaging guidelines for BSCR expand the Standardization of Uveitis Nomenclature (SUN) classification criteria by reaffirming the critical role of ICGA and providing a standardized guidelines for using other imaging modalities in the diagnosis and monitoring of BSCR. These guidelines are expected to facilitate monitoring of disease activity and complications using multimodal imaging

Evidence and Consensus Based Guidelines for Imaging in Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) - Multimodal imaging in Uveitis (MUV) Taskforce: Report 7

Purpose: To develop consensus-based guidelines on use and interpretation of multimodal imaging in acute posterior multifocal placoid pigment epitheliopathy (APMPPE). Design: Consensus agreement led by literature, and an expert committee using a nominal group technique (NGT). Methods: The expert committee for APMPPE performed a thorough review of representative cases of APMPPE. The cases were used to develop guidelines for the diagnosis and follow-up of APMPPE using color fundus photography (CFP), optical coherence tomography (OCT), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), fundus autofluorescence (FAF), and OCT angiography (OCTA). Structured NGT-based discussions were used to achieve consensus-based recommendations on imaging characteristics, disease activity, and complications, and subsequently were adopted by a vote of the entire taskforce. Results: Diagnosis of active APMPPE is characterized by distinctive imaging findings on CFP, and hyper-reflectivity of the ellipsoid zone (EZ), external limiting membrane (ELM), and outer nuclear layer (ONL) on OCT. Choriocapillaris non-perfusion, detectable via early-phase FFA, ICGA or OCTA, is crucial. In the early stages of APMPPE, OCT findings may be unremarkable, making FFA, ICGA, and/or OCTA relevant for the diagnosis. Based on the imaging findings, APMPPE can be classified into four stages of activity: choroidal, chorioretinal, transitional, and resolved. Following diagnosis, OCT and OCTA can be used to monitor lesion activity and identify potential complications. Conclusions: MUV imaging criteria enable the identification of key diagnostic features for APMPPE, extending the Standardization of Uveitis Nomenclature (SUN) classification. These consensus-based guidelines provide a framework for evaluating disease activity and complications, enhancing diagnostic accuracy and guiding clinical management

Evidence and Consensus based Guidelines for Imaging in Non-Infectious Posterior Uveitis - Methodology of the Multimodal Imaging in Uveitis (MUV) Project: Report 2: Methodology of the Multimodal Imaging in Uveitis (MUV)

The Multimodal Imaging in Uveitis (MUV) project is a comprehensive initiative aimed at developing guidelines for the use of multimodal imaging (MMI) in diagnosing and managing non-infectious posterior uveitis (NIPU). This project seeks to develop standardized guidelines and a minimal imaging set leading to evidence and consensus-based imaging guidelines that are applicable across diverse clinical settings. This manuscript describes the overall goals and methodology of the project.The MUV project was structured into seven phases: (1) a global survey to assess current practices and the need for standardized MMI criteria, (2) study design planning to define research questions and establish expert committees, (3) systematic review of the literature, (4) evidence and consensus-building on imaging guidelines through the Nominal Group Technique (NGT), (5) agreement with proposed guidelines by task force and development of consensus statements for use of MMI in the diagnosis and monitoring of NIPU, (6) standardizing the endpoints of inflammation on imaging in NIPU and (7) prospective validation of MMI criteria, using formal consensus techniques, to achieve supermajority agreement.The initial survey revealed that nearly 90% of uveitis and retina specialists already incorporate MMI into their diagnostic process for NIPU, with strong support for standardized practices. The NGT phase formulated and achieved statements on clinical utility of MMI, findings that were further substantiated by the systematic review. The final guidelines, proposed by the NGT and approved by the task force, offer a standardized framework for utilizing MMI in NIPU. This was a key aspect of this patient-focused update.The MUV project introduces a formalized set of guidelines for MMI use in NIPU, enhancing and extending the Standardization of Uveitis Nomenclature (SUN) classification framework. The comprehensive guidelines developed through this initiative will standardize the use of MMI in clinical practice, which should lead to optimal use of imaging for more accurate diagnoses, better monitoring of disease activity, and improved management of complications

Evidence and Consensus-based Imaging Guidelines in Birdshot Chorioretinopathy: Multimodal Imaging in Uveitis (MUV) Taskforce Report 8

To develop consensus-based imaging guidelines for diagnosing and monitoring birdshot chorioretinopathy (BSCR). Consensus-based approach guided by literature and an expert committee using a nominal group technique (NGT). An expert committee of 5 international uveitis specialists reviewed 15 well-documented representative BSCR cases with comprehensive imaging data. Cases with active and inactive disease were included. Imaging, including color fundus photography (CFP), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), optical coherence tomography (OCT), fundus autofluorescence (FAF), and OCT angiography (OCTA) were reviewed. Using a structured NGT approach, consensus-based recommendations were developed for specific disease characteristics, biomarkers of activity, and complications. The recommendations were voted upon by members of the full task force. For the diagnosis of BSCR, CFP, FFA, and ICGA were identified as the key imaging modalities. ICGA was identified as a key imaging modality for assessing the presence of choroidal lesions. FFA was deemed crucial for monitoring retinal vascular leakage and assessing the treatment response. OCT, while not essential for diagnosis, was valuable for detecting complications such as cystoid macular edema and retinal thinning. The committee did not reach a consensus on the role of FAF and OCTA for the diagnosis or monitoring of BSCR. The MUV consensus-based imaging guidelines for BSCR expand the Standardization of Uveitis Nomenclature (SUN) classification criteria by reaffirming the critical role of ICGA and providing a standardized guidelines for using other imaging modalities in the diagnosis and monitoring of BSCR. These guidelines are expected to facilitate monitoring of disease activity and complications using multimodal imaging

Evidence and Consensus-based Imaging Classification Criteria in Multiple Evanescent White Dot Syndrome - Multimodal imaging in Uveitis (MUV) Taskforce Report 6

: To develop imaging and consensus-based guidelines on the application of multimodal imaging in multiple evanescent white dot syndrome (MEWDS). : Consensus agreement guided by literature, and an expert committee using a nominal group technique (NGT). : The expert committee employed a structured NGT with multiple rounds of discussion, conflict resolution, and anonymous voting to: (1) establish imaging criteria for diagnosing and monitoring MEWDS using color fundus photography (CFP), optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA); and (2) develop consensus-based recommendations for assessing specific characteristics in patients with MEWDS. These formal recommendations were derived from a structured NGT using illustrative cases of MEWDS and were further voted upon by the entire task force. : The diagnosis of acute MEWDS is supported by distinct multimodal features on CFP, multi-focal disruption of the ellipsoid/interdigitation zone with overlying outer retinal hyper-reflectivity with OCT, and hyper-autofluorescent spots with FAF (short-wave blue/green). In complex cases, wreath-like lesions on FFA and the absence of early hypofluorescence on ICGA help differentiate MEWDS from other chorioretinopathies. The lack of specific choroidal changes on OCT and preserved signal on OCTA on retinal and inner choroidal slabs also aid in diagnosis. : Multimodal imaging is essential for diagnosing MEWDS and differentiating it from other non-infectious uveitis types, extending the Standardization of Uveitis Nomenclature (SUN) classification. These imaging criteria enable detailed assessment of disease activity and offer valuable insights into MEWDS pathogenesis