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Viruses belonging to the herpes family group, including HSV (herpes simplex virus) 1 and 2, VZV (varicella zoster virus) and CMV (cytomegalovirus) are the leading causes of necrotizing retinitis. These viral retinal necroses generally manifest in three forms, depending on the patient's immune status: acute retinal necrosis (ARN), progressive outer retinal necrosis (PORN) and CMV retinitis. Although specific, effective drug treatments are available today, early treatment initiation is essential to avoid sight-threatening complications., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

Purpose of Review: This article intends to briefly discuss AIDS, summarize the current literature on immune recovery uveitis, describe its ocular manifestations and complications, and tackle its complex management., Recent Findings: The clinical picture of immune recovery uveitis is still evolving. Up to today, there are still no definite criteria for immune recovery uveitis, and although closely associated with cytomegalovirus retinitis and HIV/AIDS, there are several cases of similar intraocular response in non-HIV patients. The exact pathology for this paradoxical inflammatory reaction remains unclear; however, there is an interest in identifying biomarkers to determine underlying mechanisms and identify patients at risk. The management of this disease also remains a challenge and no standardized treatment approach exists currently., Summary: Immune recovery uveitis is an important cause of visual morbidity particularly in HIV/AIDS patients receiving highly active antiretroviral. It is a paradoxical reaction that is frequently associated with a prior cytomegalovirus retinitis infection. Although it can be a transient and self-limiting process, there is a complex decision on the timing of antiviral treatment and the initiation of antiretroviral treatment to prevent immune recovery uveitis. Furthermore, a substantial challenge arises in balancingtreatment decisions for complications in refractory cases., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Purpose: To describe our experience with the use of a sustained-release dexamethasone implant in three patients with recalcitrant macular edema that developed after necrotizing retinitis in the context of the previously treated virus., Case Description: Two immunocompetent patients presented with unilateral acute retinal necrosis (ARN) due to Varicella-Zoster (VZV). The other, an immunocompromised patient, presented with unilateral cytomegalovirus (CMV) necrotizing retinitis. The diagnoses were confirmed by anterior chamber polymerase chain reaction (PCR) and all were treated with oral valganciclovir and intravitreal ganciclovir (2 mg/0.1 ml). Infection was controlled but two of them required pars plana vitrectomy. Between 2 and 4 months after the resolution of signs of infection, resistant macular edema (RME) developed, and an intravitreal dexamethasone device was implanted after anterior chamber PCR had been negative. Functional and anatomical improvement was achieved, with the resolution of the edema accompanied by improvement in visual acuity in all patients. There was no evidence of reactivation at two years. No cataract or ocular hypertension was observed. One patient required two additional dexamethasone implants., Conclusion: Dexamethasone intravitreal implant could be considered as an option for the treatment of macular edema developed after ARN. Care should be taken to avoid reactivation and patients need to be properly informed., Competing Interests: Declaration of conflicting interestsThe authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Cytomegalovirus (CMV) retinitis is the most common eye disease associated with CMV infection in immunocompromised individuals. The CMVR may initially be asymptomatic; however, relatively mild vitreous inflammation at the onset may be an important differential point from other diseases in HIV patients. Fundus photography, CD4 T-cell count, and telemedicine could be used to screen and monitor the high-risk population, particularly in resource-limited regions. Retinitis generally starts in the peripheral retina and advances toward the posterior pole, which could develop to the characteristic "pizza pie" appearance marked by central retinal necrosis and intraretinal hemorrhage. CMVR causes vision loss if left untreated, and early antiviral therapy significantly reduces the risk of vision loss. Alongside traditional antiviral treatments, immunotherapies including CMV-specific adoptive T-cell therapy and CMV immunoglobulin (CMVIG) are emerging as promising treatment options due to their favorable tolerability and reduced mortality. This review comprehensively examines CMV retinitis, encompassing the clinical features, differential diagnosis, laboratory tests, and updated treatment strategies to inform clinical management.

Background: Acquired human cytomegalovirus (CMV) is a noteworthy disease in infants. This case study will highlight the influence of early diagnosis of CMV retinitis (CMVR) on avoid visual impairment., Clinical Findings: We describe a preterm female infant with a birth weight of 2060 gr that was admitted for tracheostomy placement due to hypoxic-ischemic encephalopathy. There were no signs of CMV infection or sepsis in laboratory results upon admission such as serology (IgG, IgM antibodies), Toxoplasma gondii , Rubella virus, Herpes simplex virus, CMVR and urine polymerase chain reaction (PCR)., Primary Diagnosis: Incidentally, upon screening for retinopathy of prematurity, diffuse occlusive vasculitis was detected in the retinal image on the 112th day of life., Intervention: Intravenous and intraocular ganciclovir were administered for 4 weeks., Outcomes: In the follow-up visit 6 weeks after discharge from the hospital, visual impairment was detected on both sides., Practice Recommendations: This is a report of a case of acquired CMVR, a silent finding, as an uncommon complication in preterm neonates during the hospital stay. This diagnosis should be taken into consideration in preterm infants, since early diagnosis and treatment are crucial to avoid visual impairment., Competing Interests: Competing interest : The authors declare no conflict of interest., (Copyright © 2024 by The National Association of Neonatal Nurses.)

Cytomegalovirus retinitis (CMVR) is a significant cause of vision loss. Regular screening is crucial but challenging in resource-limited settings. A convolutional neural network is a state-of-the-art deep learning technique to generate automatic diagnoses from retinal images. However, there are limited numbers of CMVR images to train the model properly. Transfer learning (TL) is a strategy to train a model with a scarce dataset. This study explores the efficacy of TL with different pre-trained weights for automated CMVR classification using retinal images. We utilised a dataset of 955 retinal images (524 CMVR and 431 normal) from Siriraj Hospital, Mahidol University, collected between 2005 and 2015. Images were processed using Kowa VX-10i or VX-20 fundus cameras and augmented for training. We employed DenseNet121 as a backbone model, comparing the performance of TL with weights pre-trained on ImageNet, APTOS2019, and CheXNet datasets. The models were evaluated based on accuracy, loss, and other performance metrics, with the depth of fine-tuning varied across different pre-trained weights. The study found that TL significantly enhances model performance in CMVR classification. The best results were achieved with weights sequentially transferred from ImageNet to APTOS2019 dataset before application to our CMVR dataset. This approach yielded the highest mean accuracy (0.99) and lowest mean loss (0.04), outperforming other methods. The class activation heatmaps provided insights into the model's decision-making process. The model with APTOS2019 pre-trained weights offered the best explanation and highlighted the pathologic lesions resembling human interpretation. Our findings demonstrate the potential of sequential TL in improving the accuracy and efficiency of CMVR diagnosis, particularly in settings with limited data availability. They highlight the importance of domain-specific pre-training in medical image classification. This approach streamlines the diagnostic process and paves the way for broader applications in automated medical image analysis, offering a scalable solution for early disease detection., (© 2024. The Author(s).)

Purpose: To report the clinical features of cytomegalovirus (CMV) retinitis with panretinal occlusive vasculitis., Methods: Retrospective case series., Results: Four eyes in 3 non-HIV patients (male: female = 3:0) were included. Previous medical history included diabetes mellitus (n = 2), age-related macular degeneration (n = 1), and Multiple myeloma under chemotherapy (n = 1). All patients were treated with oral valganciclovir and intravitreal ganciclovir. Slow resolution of retinitis related retinal opacification was noted in all 4 eyes. Two eyes had anti-viral agents discontinued despite the persistent retinitis related opacification and the lesions slowly resolved in the following months. The final decimal visual acuity was equal to or worse than 0.02 in 3 of the 4 eyes., Conclusion: In eyes of CMV retinitis with panretinal occlusive vasculitis, rapid resolution of retinitis lesions is an unreliable sign evaluating the therapeutic efficacy of anti-viral agents. Besides, despite treatment of anti-viral agents, deteriorating vascular occlusion may further endanger macular function.

Purpose: Intravitreal Ganciclovir has been one of the treatments of choice for cytomegalovirus (CMV) retinitis and has been used extensively for its treatment since 1987. It has not been shown to have any major adverse effects. There are no reports on any retinal toxicity even after multiple, repeated injections. Herein, we report a rare case of retinal toxicity after multiple intravitreal injections in a patient of CMV retinitis., Case Report: A 69-year-old one eyed male, who was on oral corticosteroids and systemic immunosuppression for Granulomatosis with Polyangiitis, presented with CMV retinitis in both eyes. His visual acuity was 20/60 in his right eye and no perception of light in his left eye. He was treated with multiple injections of intravitreal Ganciclovir in his right eye. The left eye was not treated since it had no vision potential. The right eye of the patient which had received multiple injections went on to developed a progressive diffuse atrophy of Retinal Pigment Epithelium (RPE). No such changes were noted in the left eye of the patient., Conclusion and Importance: We present a case of progressive diffuse RPE atrophy as a result of toxicity of intravitreal ganciclovir injections. It is important to be aware of this rare potential toxicity of intravitreal Ganciclovir.

Purpose: To investigate predisposing immunosuppressive conditions causing cytomegalovirus retinitis (CMVr) and risk factors for delayed diagnosis in patients diagnosed between 2008 and 2018 in the Swedish population of 10 million., Results: Sixty-three consecutive patients (100 eyes) were diagnosed with CMVr. The most common immunosuppressive state predisposing for CMVr was hematopoietic stem cell transplant recipients (27%) and hematological malignancy (24%). Two patients (3.2%) had no other predisposing factor than diabetes mellitus (DM). Patients with delayed diagnosis (≤30 days) were older than those with earlier diagnosis, mean age 68.7 (±9.8) and 48.8 (±17.6), respectively, p = .001. Signs of intraocular inflammation (IOI) were seen in 42 (70%) of cases and more common in delayed than early diagnosis, 16 (89%) and 21 (60%) respectively ( p = .03)., Conclusion: Delayed diagnosis was more common in older individuals with signs of IOI. DM may be a risk factor for CMVr, and clinical signs can be mistaken for diabetic retinopathy.

Purpose: To investigate the incidence of and predictive factors for recurrent cytomegalovirus retinitis (CMVR) in human immunodeficiency virus (HIV)-negative patients., Methods: A retrospective review of HIV-negative patients who were newly diagnosed with CMVR between January 2005 and February 2019., Results: Of 28 patients (44 eyes), 35.9% of eyes had a recurrence of CMVR after discontinuation of anti-CMV therapy. The incidence of CMVR recurrence was 17 per 100 eye-years. The factors significantly associated with CMVR recurrence were eyes with retinitis area of more than 25% ( P = .013), absence of vitreous haze ( P = .003), neutropenia at presentation ( P = .001), and absence of systemic immunosuppression therapy prior to presentation ( P = .002)., Conclusion: Eyes with a large area of retinitis, absence of vitreous haze, and neutropenia at presentation are predictive of CMVR recurrence while receiving systemic immunosuppression prior to CMVR presentation has a lower risk of CMVR recurrence.

Purpose: Cytomegalovirus (CMV) retinitis can have debilitating impacts on quality of life (QOL), but few contemporary studies have characterized these ramifications. This study assessed the impact of CMV retinitis on vision-related QOL for those living with HIV/AIDS in Thailand., Methods: QOL was assessed as part of a prospective interventional cohort study of patients referred to a tertiary hospital in Thailand for CMV retinitis screening. A validated vision-related QOL questionnaire was administered at the baseline screening visit and at the 6-month study visit. Multivariable linear regression models were performed to determine the effect of CMV retinitis diagnosis on QOL score., Results: A total of 152 participants completed the QOL questionnaire at their initial clinic visit. At baseline, a diagnosis of CMV retinitis diagnosis was significantly associated with decreased QOL score: unilateral retinitis was associated with a 0.11 (95% CI: -0.26-0.03) decrement in QOL, and bilateral retinitis was associated with a 0.33 (95% CI: -0.51-0.16) decrement (joint P-value = 0.0009). For the 78 participants with a 6-month visit, changes in QOL from baseline were small and not significant. A diagnosis of CMV retinitis was still associated with decreased QOL score at 6 months (joint P -value = 0.03)., Conclusions: This study found that vision-related QOL was lower in those with CMV retinitis, especially with bilateral involvement, and did not improve after treatment among those with follow-up. These findings reinforce the debilitating clinical manifestations of this disease, and support efforts for earlier screening to detect CMV retinitis before impacts on QOL have occurred.

Purpose: To report a case series of human immunodeficiency virus (HIV)-negative patients with healed cytomegalovirus retinitis presenting with proliferative retinopathy (in the form of neovascularisation elsewhere)., Methods: Retrospective case series. Multimodal imaging was performed at each follow-up visit., Results: Three patients with non-HIV immune dysfunction were followed up after healing of CMV retinitis. All three developed neovascularisation. Patient 1 after 4 months presented with vitreous haemorrhage for which pars plana vitrectomy was performed. Patient 2 developed neovascularization at disc and neovascularisation elsewhere 4 months after resolution, and patient 3 despite being affected by bilateral CMV retinitis, presented with unilateral neovascularization at 14 month after resolution of retinitis., Conclusion: Increased incidence of this rare entity could be attributed to partial immune dysfunction in non-HIV patients, limited area of retinitis with a more aggressive occlusive vasculitis. Extensive occlusion with more area of viable retina for angiogenic factor production explains this phenomena. It emphasizes the need for continued follow-up even after healing and to differentiate it from reactivation of retinitis and immune recovery uveitis. Abbreviation: CMV: cytomegalovirus; HIV: human immunodeficiency virus; BCVA: best corrected visual acuity.

Purpose: To report a rare case of cytomegalovirus (CMV)-associated non-necrotizing viral retinopathy, occlusive retinal vasculitis, papillitis, and retinal neovascularization in a young 41-year-old woman., Methods: Case report., Results: The patient presented with features of papillitis, peripapillary cotton-wool spots, pre-retinal hemorrhages, and occlusive vasculitis. Her visual acuity was 20/100 in the left eye. She developed a worsening of the disease upon initiation of systemic corticosteroids. Her serum immunoglobulins (Ig) (both IgG and IgM) were highly positive for CMV. Anterior chamber paracentesis was positive for CMV DNA using real-time polymerase chain reaction. After stopping systemic corticosteroids, she was initiated on oral valganciclovir, with rapid resolution of the vasculitis and cotton-wool spots. After three months, the patient developed retinal neovascularization and underwent pan-retinal photocoagulation. However, her uveitis was inactive, and her visual acuity improved to 20/25., Conclusions: Non-necrotizing viral retinopathy has been associated with either varicella zoster virus (VZV) or herpes simplex virus (HSV). Our case highlights that CMV can also lead to non-necrotizing retinopathy and must be suspected in patients who may be negative for VZV and HSV. Appropriate anti-viral treatment can prevent severe vision loss in these patients.

Purpose: To report a case of unilateral cytomegalovirus (CMV) panuveitis with occlusive vasculitis after injection of intravitreal dexamethasone implant in a patient with type 2 diabetes mellitus., Methods: Observational case report., Results: A 60-year-old immunocompetent man with well-controlled type 2 diabetes mellitus was treated with intravitreal dexamethasone implant for recurrent uveitis that was responsive to steroids. Three months after implantation, the patient develops panuveitis with occlusive vasculitis. Anterior chamber tap confirms diagnosis of cytomegalovirus retinitis. Intravitreal foscarnet and oral valganciclovir led to quiescent disease., Conclusions: Patients treated with local immunosuppressants should be monitored carefully to assess treatment response and complications, even in the absence of frank immunodeficiency. Quantitative viral PCR can be an effective way to monitor treatment response to antiviral therapy.

Purpose: To report a case of unilateral, sectoral retinal metastasis of small cell lung cancer (SCLC) that mimicked cytomegalovirus retinitis., Method: Case report., Results: A 48-year-old woman presented with a 4-week history of a visual field loss in her right eye. She had a medical history of extensive-stage SCLC with brain metastasis, stable on maintenance atezolizumab for 2 years. On initial presentation, she was diagnosed with cytomegalovirus retinitis. No improvement was observed with 4 weeks of oral valganciclovir. Upon referral for a second opinion, her fundus examination appeared compatible with cytomegalovirus retinitis, and anterior chamber tap for polymerase chain reaction for viral etiologies was performed followed by intravitreal and IV ganciclovir without improvement. She was referred for a third opinion, where diagnostic vitrectomy with vitreous and retinal biopsies were consistent with SCLC metastatic to the retina. The patient underwent enucleation of the right eye for definitive pathologic analysis and subsequently was started on additional systemic chemotherapy., Conclusion: Retinal metastases are exceedingly rare, particularly retinal metastasis of SCLC. Retinal metastasis should be considered in patients initially diagnosed with viral retinitis who fail to improve despite antiviral therapy, particularly if they have a known history of malignancy. Furthermore, retinal metastasis of SCLC potentially could be misdiagnosed histopathologically as retinoblastoma if the patient's history is unknown and appropriate immunohistochemical stains are not performed., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the Opthalmic Communications Society, Inc.)

Purpose: The purpose of this study was to describe a case of a 64-year-old man presenting with cytomegalovirus and herpes simplex virus retinitis coinfection in the setting of Burkitt lymphoma., Methods: A case report including multimodal imaging and anterior chamber polymerase chain reaction results was used., Results: This case highlights the importance of the clinical examination and maintaining high diagnostic suspicion for viral retinitis in immunocompromised patients., Conclusion: Aqueous fluid PCR can be a useful adjunctive test to distinguish and confirm a diagnosis of viral retinitis. Given the limited sample volume of aqueous biopsy, it is important to prioritize the order of PCR testing based on clinical suspicion of the causative agent.

Good syndrome (GS) is a rare primary immunodeficiency in adults consisting of hypogammaglobulinemia and thymoma that affects both cellular and humoral immunity. It usually appears in patients between the 4th and 6th decade of life and affects both genders equally. Ophthalmological clinical presentation is highly variable; associations with herpetic keratitis, toxoplasmosis, and cytomegalovirus retinitis (CMVR) have been described. GS associated with CMVR is uncommon. Ophthalmologists may be the first to diagnose systemic disease and change the outcome. Only18 cases of CMVR have been described, most of them unilateral with poor visual outcomes. We discuss the clinical features of CMVR in patients with reported GS, pathogenesis, and outline a work-up for diagnosis. CMVR in an apparently healthy patient should encourage the clinician to search for human immunodeficiency virus (HIV) and non-HIV-associated immunosuppression., Competing Interests: Declaration of Competing Interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Inc. All rights reserved.)

Aim: To report a case of cytomegalovirus (CMV) neuroretinitis observed in an immunocompetent patient. Materials and methods: The patient presented with a complaint of diminution of vision in both eyes (BE) and had a traumatic cataract in the right eye (RE). Fundus examination of the left eye (LE) revealed an active white, fluffy lesion with an overlying retinal hemorrhage patch with a macular star. The diagnosis of CMV neuroretinitis was established, and the patient commenced treatment with valganciclovir. Results: The patient exhibited no underlying risk factors. Subsequently, a positive response to oral valganciclovir treatment was observed. Discussion: Cytomegalovirus (CMV) neuroretinitis is typically associated with immunocompromised individuals, such as those with HIV/AIDS. The patient's presentation with a traumatic cataract in the right eye and a distinctive fundus appearance in the left eye posed a diagnostic challenge. The absence of common risk factors for CMV infection necessitated a thorough examination and consideration of rare infectious etiologies. The positive response to valganciclovir reinforces its efficacy in managing CMV-related ocular conditions. This case emphasized the necessity for ophthalmologists to maintain a high index of suspicion for CMV and other unusual pathogens when faced with neuroretinitis in patients who do not present with typical systemic immunosuppressive conditions. Early diagnosis and appropriate antiviral therapy prevent potential complications and preserve vision in such atypical presentations. Conclusion: This case underscores the importance of considering rare infectious agents in immunocompetent patients when encountering neuroretinitis, particularly in the absence of typical symptoms or signs of the disease. Abbreviations: CMV = Cytomegalovirus, BE = Both eyes, RE = Right eye, LE = Left eye, CBC = Complete Blood Count, ESR = Erythrocyte Sedimentation Rate, VDRL = Venereal Disease Research Laboratory, FTA-ABS = Fluorescent Treponemal Antibody Absorption, PPD = Purified Protein Derivative, ANA = Anti-Nuclear Antibodies, RF = Rheumatoid Factor, ACE = Anti Converting Enzyme, Ig G = Immunoglobulin G, HSV = Herpes simplex virus., (#x00A9; The Authors.Romanian Society of Ophthalmology.)

Background: Syphilis has historically been referred to as "the great imitator", for the extent of disease manifestations secondary to infection. Ocular manifestations include a wide range of intra-ocular inflammation., Methods: In this study, we report the case of a 52 years-old male patient with syphilitic hemorrhagic necrotizing retinitis., Results: The patient presented to the emergency room for rapid and progressive vision loss and ocular redness lasting three weeks and was under immunosuppressive treatment. The diagnosis was syphilitic hemorrhagic necrotizing retinitis mimicking the typical clinical picture of retinitis caused by Cytomegalovirus infection in immunocompromised patients., Conclusions: The presented case highlights the need to consider ocular syphilis as a great masquerader even in the presence of atypical presentations such as hemorrhagic retinitis. Syphilis should be tested for treponemal and non-treponemal tests, and it should be ruled out as an etiological agent in every case of new-onset intra-ocular inflammation., Competing Interests: Declaration of conflicting interestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Purpose: Necrotizing viral retinitis is a serious eye infection that requires immediate treatment to prevent permanent vision loss. Uncertain clinical suspicion can result in delayed diagnosis, inappropriate administration of corticosteroids, or repeated intraocular sampling. To quickly and accurately distinguish between viral and noninfectious retinitis, we aimed to develop deep learning (DL) models solely using noninvasive blood test data., Methods: This cross-sectional study trained DL models using common blood and serology test data from 3080 patients (noninfectious uveitis of the posterior segment [NIU-PS] = 2858, acute retinal necrosis [ARN] = 66, cytomegalovirus [CMV], retinitis = 156). Following the development of separate base DL models for ARN and CMV retinitis, multitask learning (MTL) was employed to enable simultaneous discrimination. Advanced MTL models incorporating adversarial training were used to enhance DL feature extraction from the small, imbalanced data. We evaluated model performance, disease-specific important features, and the causal relationship between DL features and detection results., Results: The presented models all achieved excellent detection performances, with the adversarial MTL model achieving the highest receiver operating characteristic curves (0.932 for ARN and 0.982 for CMV retinitis). Significant features for ARN detection included varicella-zoster virus (VZV) immunoglobulin M (IgM), herpes simplex virus immunoglobulin G, and neutrophil count, while for CMV retinitis, they encompassed VZV IgM, CMV IgM, and lymphocyte count. The adversarial MTL model exhibited substantial changes in detection outcomes when the key features were contaminated, indicating stronger causality between DL features and detection results., Conclusions: The adversarial MTL model, using blood test data, may serve as a reliable adjunct for the expedited diagnosis of ARN, CMV retinitis, and NIU-PS simultaneously in real clinical settings.

Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Purpose: To describe cytomegalovirus retinitis in a patient with Good syndrome (hypogammaglobulinemia and thymoma), ocular progression despite treatment and fatal outcome., Methods: A 71-year-old woman with unilateral panuveitis of unknown cause and a history of thymoma resection was referred to the clinic. Visual acuity was 20/100 in her right eye and light perception in her left eye. In slit-lamp examination, the right eye had inferior, fine, pigmented keratic precipitates, 2+ anterior chamber cells, cataract, and 2+ vitreous cells, with no fundus detail and normal ocular ultrasound results. Left eye presented a white cataract, chronic hypotony, and increased choroidal thickness with calcifications. Laboratory evaluations showed normal or negative results for common causes of infection and inflammation. Prednisolone acetate eye drops were started, with improvement of AC inflammation. Cataract surgery was performed, reaching visual acuity of 20/30. Two years later, visual acuity had decreased and 2+ vitritis and retinitis were found. On clinical suspicion of masquerade syndrome, a vitrectomy biopsy was performed; pathologic assessments reported no data on ocular lymphoma. Leukopenia and lymphopenia were found: immunoglobulin levels, CD4 count, and viral load revealed systemic immunosuppression. The aqueous tap was positive for cytomegalovirus. Oral valganciclovir and intravitreal ganciclovir were initiated., Results: In a patient with previous resection of thymoma and hypogammaglobulinemia, final diagnosis was Good syndrome, with cytomegalovirus retinitis being secondary to immunosuppression. Despite treatment, cytomegalovirus retinitis progressed and systemic deterioration resulted in mortal outcome., Conclusion: Good syndrome is an extremely rare disease, and association with cytomegalovirus retinitis is uncommon. To the authors' knowledge, only 14 cases exist in the literature.

We present a case of CMV retinitis with retinal toxicity secondary to inadvertent overdose of intravitreal ganciclovir. To our knowledge, this is the first case published with good visual outcome from timely intervention.

Purpose: To report continuing diffuse retinal dysfunction following resolution of immune reconstitution uveitis (IRU) in patients with cytomegalovirus retinitis (CMVR)., Methods: Retrospective case series describing two patients with IRU following CMVR who underwent serial fundus photography and macular optical coherence tomography. One patient had serial electrophysiology., Results: Both patients had CMVR successfully treated with antiviral medication. The affected eyes later developed IRU that resolved with steroids. However, following resolution, chronic retinal damage was evidenced by ellipsoid line loss in one case and gradual optic disc cupping in the other. Electrophysiology in both cases revealed generalized retinal dysfunction worse in the eye with more severe IRU and demonstrated objectively the efficacy of treatment intervention in the patient with serial recordings., Conclusions: Patients with IRU following CMV retinitis may have continuing diffuse retinal dysfunction despite apparent recovery and normal visual acuity. An aggressive approach to inflammation control may be warranted in such patients., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: To report a case of cytomegalovirus (CMV) retinitis improved by treatment with ganciclovir in a patient with ganciclovir-resistant CMV infection associated with Good syndrome., Study Design: Case report., Result: A 52-year-old gentleman with Good syndrome presented with visual disturbance in his right eye. He had a history of receiving intravitreal ganciclovir treatment with CMV retinitis a year ago. During treatment for CMV colitis three months ago, in systemic blood, UL97 mutation was identified and improved after changing treatment from ganciclovir to foscarnet. CMV retinitis recurred, and intravitreal ganciclovir injection was performed but there was no improvement. Therefore, the treatment was changed to foscarnet, but retinal infiltration progressed. Accordingly, it was changed to ganciclovir again and as a result, the progression of retinitis could be stopped., Conclusions: Even in the case of CMV retinitis, which has been genetically confirmed to be ganciclovir resistance in systemic blood, ganciclovir treatment can be considered if other anti-CMV agents are not effective.

Purpose: To report a case of cytomegalovirus (CMV) retinitis complicated with ganciclovir-related myelosuppression, which was successfully managed with intravenous (IV) ganciclovir and CMV immunoglobulin (CMVIG) therapy., Methods: Observational case report., Results: A 51-year-old male with follicular type non-Hodgkin lymphoma post hematopoietic stem cell transplantation (HSCT) developed vision-threatening retinitis. polymerase chain reaction (PCR) of the aqueous humour showed positive for CMV. Despite myelosuppression occurred during IV ganciclovir therapy, the retinitis resolved and intraocular CMV viral load significantly improved after CMVIG therapy., Conclusion: Combined IV ganciclovir treatment and CMVIG therapy can significantly improve visual outcome and reduce intraocular CMV viral load in vision-threatening CMV retinitis.

Objectives: To report patients with systemic lymphoma and cytomegalovirus (CMV) retinitis, treated with a combination of oral and intravitreal antiviral agents on an outpatient basis., Methods: Retrospective cases series. Information was gathered from the database of the Uveitis clinics at Moorfields Eye Hospital, United Kingdom from December 2014 to December 2018. The inclusion criteria comprised the diagnosis of systemic lymphoma, associated with a diagnosis of CMV retinitis. Exclusion criteria were alternative ocular diagnosis, human immunodeficiency virus (HIV), primary intraocular lymphoma, or other causes of immunosuppression., Results: All seven subjects had been under oncologist care for systemic lymphoma. CMV retinitis presented with a median of 61 months after the systemic lymphoma diagnosis. Five patients underwent a vitreous biopsy, and four of them returned PCR positive for CMV and the fifth patient had PCR positive in a blood sample. All patients were treated with oral Valganciclovir, with an induction dose of 900 mg every 12 h for up to 3 weeks until disease resolution and a maintenance dose thereafter. All but one received additional intravitreal Foscarnet injections, with a dose of 2.4 mg /0.1 ml., Conclusions: The management of patients with systemic lymphoma and CMV retinitis with oral and intravitreal antiviral agents, resulted in effective disease control., (© 2022. Crown.)

Background: Cytomegalovirus retinitis (CMVR) is the most common and sight-threatening opportunistic retinal infection in patients with acquired immunodeficiency syndrome (AIDS) and several controversies remain to be settled. We aimed to summarize the current evidence and clarify the clinical features and prognosis of CMVR in AIDS patients., Methods: The databases PubMed, EMBASE, and Ovid from inception to April 2022 were searched to identify the relevant studies. R software version 3.6.3 was used to perform the statistical analyses. Results in proportion with 95% confidence interval (CI) were calculated using the Freeman-Tukey variant of arcsine square transformation., Results: We finally included 236 studies comprising 20,214 patients. CMVR in AIDS was male-dominated (88%, 95%CI 86%-89%), with 57% (95%CI 55%-60%) aged <41 years and 44% (95%CI 41%-47%) being bilaterally involved. CMVR was preponderant in AIDS patients with the following characteristics: white and non-Hispanic, homosexual, HIV RNA load ≥ 400 copies/mL, and CD4+ T-cells <50 cells/μL. The positivity of CMV-DNA in blood, aqueous humor, and vitreous humor was 66% (95%CI 52%-79%), 87% (95%CI 76%-96%), and 95% (95%CI 85%-100%), respectively. The most common symptoms were blurred vision (55%, 95%CI 46%-65%), followed by asymptomatic, visual field defect, and floaters. CMVR was first diagnosed and regarded as the clue to AIDS diagnosis in 9% (95%CI 6%-13%) of CMVR patients. Approximately 85% (95%CI 76%-93%) of the CMVR patients have received cART. CMVR remission was observed in 72%-92% of patients depending on the specific category of anti-CMV therapy. The general incidence of CMVR-related RD in the entire course was 24% (95%CI 18%-29%), of which most patients received PPV with SO or gas tamponade and the rate of anatomic success was 89% (95%CI 85%-93%)., Conclusion: CMVR is a common opportunistic infection with diverse clinical features in AIDS patients, preponderant in those who are male, homosexual, or with CD4+ T-cells <50 cells/μL. Current therapies for CMVR and CMVR-related RD were shown to be effective. Early detection and routine ophthalmic screening should be promoted in AIDS patients., Systematic Review Registration: PROSPERO, identifier CRD42022363105., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Zhao, Li, Chen and Zhao.)

Purpose: To describe a young male with bilateral sequential Cytomegalovirus retinitis (CMVR) as the presenting feature of Dyskeratosis Congenita., Case Report: A 25-year-old human immunodeficiency virus (HIV) negative male developed CMVR in his left eye, while on a three week course of oral valganciclovir therapy for CMV retinitis in his right eye. Systemic examination revealed reticular hypopigmentation of the forearms, dystrophic nails, oral leukoplakia and complete blood counts showed pancytopenia. A diagnosis of Dyskeratosis Congenita was confirmed with genetic testing., Conclusion: CMVR in non-HIV individuals should be considered as a harbinger of systemic immunosuppressive conditions. Ophthalmologists may be the first ones to suspect and diagnose congenital immunosuppressive disorders like Dyskeratosis Congenita in these patients.

A man in his early 50s on regular follow-up for a stable non-proliferative diabetic retinopathy (NPDR) presented with decreased vision, worsening of retinal pathology and macular oedema in both eyes. His corrected distance visual acuity (CDVA) was 6/9 in the right eye and 6/15 in the left eye and fundus examination showed multiple intraretinal haemorrhages in all quadrants. His systemic workup revealed a severe thrombocytopaenia, which prompted a further detailed systemic evaluation revealing him to be positive for HIV with retinopathy complicating the pre-existing NPDR. Given the significant inflammation and macular oedema, a cocktail of intravitreal bevacizumab, ganciclovir and dexamethasone was administered. The retinopathy and macular oedema resolved and the CDVA improved to 6/6 in both eyes over a 6-month follow-up period. Any sudden worsening of fundus findings in a patient with diabetes necessitates immediate and detailed ocular and systemic evaluation, especially when the immune status is unknown., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Purpose: To explore the clinical features and outcomes of cytomegalovirus retinitis (CMVR) in patients with HIV and non-HIV., Methods: This retrospective cohort study included all patients with CMVR in National Taiwan University Hospital from 2013 to 2018. Demographic data, clinical characteristics, CMVR recurrence, and overall survival were compared between the HIV and non-HIV groups. Generalized estimating equation models were implemented to analyze the risk factors of poor visual prognosis. The Kaplan-Meier survival analysis was performed to investigate recurrence and survival., Results: A total of 66 patients (95 eyes) with CMVR were enrolled, with no significant differences between the HIV (41 patients; 61 eyes) and non-HIV (25 patients; 34 eyes) groups in initial/final visual acuity, lesion area, or viral loads. Poor visual outcome was associated with poor initial visual acuity, retinal detachment, and a higher plasma cytomegalovirus titer. The HIV group had significantly longer survival rate ( P = 0.033) and lower recurrence rate ( P = 0.01) than the non-HIV group, and it also presented with better prognosis in recurrence-free survival analysis ( P = 0.01)., Conclusion: Patients with CMVR without HIV had higher mortality and recurrence rates than the HIV group. Risk factors of poor visual outcome included poor initial visual acuity, retinal detachment, and a high plasma cytomegalovirus titer.

Background: There is scarce information on AIDS-related cytomegalovirus (CMV) retinitis in middle-income countries. The objectives of this study were to identify the prevalence of active CMV retinitis in severely immunosuppressed people living with HIV (PLWHIV) and to describe its main features., Methods: This retrospective cohort study was carried out at a tertiary center in São Paulo, Brazil. We included hospitalized adults PLWHIV with CD4 count ≤100 cells/μL, ≥ one quantitation of CMV DNA in plasma, and indirect ophthalmoscopy evaluation., Results: Thirty-eight (21.6%) of 176 participants had at least an ophthalmoscopy diagnosis and only 3 (1.7%) individuals presented active CMV retinitis. All these participants were male, and retinitis was asymptomatic in 2 cases. Two participants had extraocular end-organ CMV disease and detectable CMV DNA in plasma., Conclusions: These results show a low prevalence of active CMV retinitis in the evaluated population. However, 2 of 3 participants had asymptomatic active CMV retinitis and a fifth of participants had at least one ophthalmoscopy diagnosis, suggesting the need for routine ophthalmologic evaluation in hospitalized severely immunosuppressed PLWHIV. The profile of participants with active CMV retinitis was similar to that described in the pre-ART era and quantitation of CMV DNA in plasma was variable.

Purpose: Wiskott-Aldrich syndrome (WAS) is an X-linked recessive disease whose optimal curative treatment is hematopoietic stem cell transplantation (HSCT). Patients with WAS may suffer from cytomegalovirus retinitis (CMVR) which can cause vision loss. This study is to report the progression and prognosis of patients with WAS and CMVR., Methods: A retrospective case series of ten patients with WAS and CMVR before and after HSCT who were referred to the Ophthalmology Department of Xinhua Hospital from June 2018 to February 2021. Progression and prognosis were recorded., Results: Five patients were diagnosed with CMVR before receiving HSCT at a median age of 10.5 months (range: 4-23 months). Eight patients developed CMVR post-transplantation with a median interval from HSCT of 3.5 months (range: 1-9 months)., Conclusion: Regular fundus examinations and prompt treatments in patients with WAS are therefore crucial before they receiving HSCT or approximately 3.5 months after HSCT until complete reconstitution of immune function.

Purpose: The purposes of this study were to investigate cytokine changes in the aqueous humor after treatment of acquired immune deficiency syndrome (AIDS) complicated with cytomegalovirus retinitis (CMVR) and to determine whether these changes are useful prognostic indicators. Methods: This study included 12 patients (15 eyes) undergoing treatment for AIDS and CMVR. The patients received intravitreal injections and systemic intravenous treatment with ganciclovir and foscarnet sodium. The aqueous humor of each eye was sampled before treatment and before the third and fifth injections. The samples were tested to determine the concentrations of each of 27 cytokines using the Luminex 200™ liquid phase chip. Results: The concentrations of cytokines interleukin (IL)-1rα ( P  = 0.002), IL-1b ( P  = 0.001), IL-8 ( P  = 0.001), basic fibroblast growth factor (bFGF) ( P  < 0.001), interferon γ-induced protein 10 (IP-10) ( P  = 0.001), and tumor necrosis factor (TNF)-α ( P  = 0.004) in the aqueous humor before the third and fifth injections were significantly lower after than before treatment. The reductions in TNF-α ( P  = 0.028) and IL-1b ( P  = 0.028) concentrations after treatment were statistically significant compared with the postoperative visual acuity improvement (≥3 lines and <3 lines). The difference in TNF-α ( P  = 0.018) level before and after treatment (the difference between before treatment and before the fifth intravitreal injection) was also statistically significant compared with the number of injections (≥6 times and <6 times). Conclusion: The cytokines IL-1rα, IL-1b, IL-8, bFGF, IP-10, and TNF-α may offer new avenues for evaluation of therapeutic effect, and TNF-α and IL-1b may be important cytokines for prognostic evaluation (based on visual acuity and the number of injections) in patients suffering from AIDS and CMVR. Clinical Trial Registration: Number: ChiCTR2200056955.

Purpose: To report a case of cytomegalovirus retinitis (CMVR) with hypopyon and intense ocular inflammation., Case Report: An 81-year-old female was referred to our hospital with a suspicion of postoperative endophthalmitis in the left eye. She had been treated with etoposide and prednisolone for non-Hodgkin's lymphoma. Examination revealed mutton-fat keratic precipitates and numerous infiltrating cells in the anterior chamber with hypopyon. The fundus was invisible due to intense vitreous opacity. Systemic and topical administration of antibiotics was started, and vitrectomy was performed. However, the ocular symptoms did not respond to treatment. Vitrectomy was repeated twice, but severe endophthalmitis findings recurred soon after surgery. Finally, a comprehensive viral PCR test using the intraocular fluid detected CMV with 3.34 × 10 9 copies/ml, leading to a diagnosis of CMV retinitis., Conclusions: If the causative agent is not identified in endophthalmitis that develops in immunosuppressive patients, CMV may also be considered as the possible cause.

Purpose: To highlight characteristics in the misdiagnosis of cytomegalovirus retinitis (CMVR)., Methods: Misdiagnosed cases related to CMVR were analyzed retrospectively at the Department of Ophthalmology, Beijing Youan Hospital, from July 2017 to October 2019. The medical records were reviewed by two independent senior ophthalmologists and the patients' clinical characteristics were analyzed., Results: Eight patients (16 eyes) were identified with misdiagnoses related to CMVR. Six of the patients with CMVR were previously unaware of their human immunodeficiency virus (HIV) infection; one patient with CMVR concealed their history of HIV infection. The cases were initially misdiagnosed as diabetic retinopathy (1/7, 14.3%), branch retinal vein occlusion (1/7, 14.3%), ischemic optic neuropathy (1/7, 14.3%), Behçet's disease (1/7, 14.3%), iridocyclitis (2/7, 28.6%), and progressive outer retinal necrosis (1/7, 14.3%). One patient with binocular renal retinopathy and chronic renal insufficiency was misdiagnosed with CMVR. Four eyes (4/16, 25%) presented with pan-retinal involvement. Fourteen eyes (14/16, 87.5%) had optic disc or macular area involvement. At the final diagnosis, one patient was blind, and two patients had low vision. Seven AIDS patients showed an extremely low level of CD4 + T lymphocytes (median of 5 cells/μl; range 1-9 cells/μl)., Conclusion: CMVR may be misdiagnosed in the absence of known immune suppression. CMVR and HIV screening cannot be overlooked if a young male patient presents with yellowish-white retinal lesions. These misdiagnosed patients had severe retinitis associated with poor vision., Competing Interests: None

Purpose: To clarify the cut off value of blood CMV load to indicate CMV retinitis and its relationships with ocular features., Methods: Patients were divided into non-CMV and CMV retinitis groups. A logistic regression model was applied to estimate the association of each variable with CMV retinitis. Spearman correlation was used to estimate the correlation between the blood and aqueous CMV load., Results: Blood CMV load higher than 4log 10 (OR, 6.897; CI: 2.813-16.910; P < .001) was the major predictor of CMV retinitis. Blood CMV load wasn't different between the initial and early stage ( P = .066). No correlation was observed between the blood and aqueous CMV load ( P = .083, r = 0.228)., Conclusions: Blood CMV load higher than 4log 10 is an important predictor for CMV retinitis in HIV/AIDS patients, but it couldn't indicate the ocular features. Ophthalmologic screening is still necessary. Abbreviations : CMV: Cytomegalovirus; CMVR: Cytomegalovirus retinitis; HIV: Human Immunodeficiency Virus; AIDS: Acquired Immune Deficiency Syndrome; ART: Antiretroviral therapy; EOD: End-organ diseases; PCR: Polymerase Chain Reaction; OR: Odds Ratio; CI: 95% Confidence Interval; IQR: Interquartile range.

Propose: A screening protocol for cytomegalovirus retinitis (CMVR) by fundus photography was generated, and the diagnostic accuracy of machine learning technology for CMVR screening in HIV patients was investigated., Methods: One hundred sixty-five eyes of 90 HIV-positive patients were enrolled and evaluated for CMVR with binocular indirect ophthalmoscopy. Then, a single central field of the fundus image was recorded from each eye. All images were then interpreted by both machine learning models, generated by using the Keras application, and by a third-year ophthalmology resident. Diagnostic performance of CMVR screening using a machine learning model and the third-year ophthalmology resident were analyzed and compared., Results: Machine learning model, Keras application (VGG16), provided 68.8% (95% confidence interval [CI] = 50%-83.9%) sensitivity and 100% (95% CI = 97.2%-100%) specificity. The program provided accuracy of 93.94%. However, the sensitivity and specificity for the third-year ophthalmology grading were 67.7% (95% CI = 48.6%-83.3%) and 98.4% (95% CI = 94.5%-99.8%). The accuracy for CMVR classification was 89.70%. When considering for sight-threatening retinitis in Zone 1 and excluded Zones 2 and 3, the machine learning model provided high sensitivity of 88.2% (95% CI = 63.6%-98.5%) and high specificity of 100% (95% CI = 97.2%-100%)., Conclusion: This study demonstrated the benefit of the machine learning model VGG16, which provided high sensitivity and specificity for detecting sight-threatening CMVR in HIV-positive patients. This model is a useful tool for ophthalmologists in clinical practice for preventing blindness from CMVR, especially during the Coronavrus Disease 2019 pandemic.

Purpose: To report a case of immune recovery uveitis (IRU) secondary to cytomegalovirus (CMV) retinitis in a patient with Good syndrome treated with granulocyte colony stimulating factor (GCSF)., Methods: A case report., Case: A 54-year-old woman with a history of Good syndrome for 2 years presented with chronic panuveitis in her right eye for 6 months. She had received multiple doses of GCSF for a pulmonary infection. Her visual acuity was hand movement in the right eye. Few anterior chamber cells, dense vitreous haze, and chorioretinal lesions were noted. Granular retinal atrophic lesions without obvious infiltration were observed during diagnostic vitrectomy. Polymerase chain reaction of the vitreous sample was positive for CMV DNA. A diagnosis of IRU secondary to CMV retinitis was made. The inflammation was controlled with topical steroids after surgery., Summary: In this report, we present a patient with Good syndrome who developed IRU secondary to CMV retinitis.

Aim: Infectious uveitis is one of the most visually devastating causes of uveitis worldwide and accounts for 19.6% of all cases of uveitis in New Zealand. With the burgeoning use of intravitreal injections, there has been a commensurate increase in the number of injection-related complications and reports of infectious uveitis following their administration in recent years. We present a case series of four patients with infectious uveitis after local injections., Method: We retrospectively reviewed the data of four patients (mean age, 67.25 ± 7.58 years) who presented to the department of ophthalmology at Auckland District Health Board with infectious uveitis which occurred or worsened after local triamcinolone acetonide (TA) and/or methotrexate (MTX) injections., Results: Three patients received local TA and one patient received intravitreal MTX. All patients were immunosuppressed prior to treatment. Two patients had toxoplasma chorioretinitis which worsened with local TA and one patient developed cytomegalovirus (CMV) retinitis after intravitreal TA. The last patient had syphilis retinopathy which worsened with intravitreal MTX. There were atypical presentations, as demonstrated by a case of presumed birdshot chorioretinopathy flare which tested positive for toxoplasma chorioretinitis with polymerase chain reaction (PCR)., Conclusion: Uveitis due to infectious etiologies needs to be carefully excluded prior to the use of local steroid and/or methotrexate injections. Disease presentations may be atypical in the presence of marked immunosuppression following local therapy. Polymerase chain reaction (PCR) can play an important role in the diagnosis in this setting.

Purpose: To study clinical efficacy of valganciclovir in cytomegalovirus retinitis (CMVR) in human immunodeficiency virus (HIV)-positive-positive patients in a tertiary care clinic in a developing nation., Methods: In a retrospective study, systemic and ocular records of HIV patients suffering from CMVR and treated with valganciclovir, were analyzed. Primary outcome measures were involvement of the other eye, incidence of retinal detachment, systemic involvement, and mortality encountered. Secondary outcome measures included change in BCVA., Results: Out of nine patients who were included, two patients developed CMVR in the other eye and only one patient (11.11%) developed retinal detachment during the course of the study. No patient developed any systemic manifestations or had mortality during the course of the study. The change in BCVA was not statistically significant., Conclusion: Use of oral valganciclovir showed good outcome and was found to be a better alternative compared to the use of intravitreal ganciclovir in the literature. Introduction of valganciclovir at an affordable price in developing nations can decrease disease burden., Competing Interests: None

Background: To report a very rare acute cystoid macular oedema following ganciclovir injection in patients receiving allogeneic haematopoietic stem cell transplantation., Case Presentation: A 44-year-old male patient experienced vision loss in his left eye eight months after allogeneic stem cell transplantation. Ophthalmologic examination showed posterior retinopathy with retinal haemorrhage, a yellow necrotic border, and a vascular white sheath involved in the superior temporal retina but not the posterior pole. Cytomegalovirus DNA results in both plasma and ocular fluid were positive. All tests combined with the patient's medical history suggested that his ocular disease was cytomegalovirus retinitis. Consequently, he received a weekly ganciclovir vitreous injection. The disease was visibly controlled, and the fundus condition improved after the first three treatments. However, the patient had severe vision loss in his left eye and acute cystic oedema in the macula, while the original lesion was stable two hours after the fourth treatment. The macular oedema subsided significantly on the first day. Over the next week, daily OCT findings indicated that the patient's macular oedema gradually subsided and resolved completely by the second week, and his left eye vision partially improved., Conclusion: Macular oedema may occur in patients with cytomegalovirus retinitis, but it rarely occurs during treatment. In this case, the patient's macular oedema appeared and resolved quickly. Macular oedema in patients with cytomegalovirus retinitis receiving vitreous cavity injections of ganciclovir needs to be further studied and discussed., (© 2022. The Author(s).)

Purpose: To evaluate the correlation of quantitative real-time polymerase chain reaction (qRT-PCR) to the clinical characteristics of patients with viral retinitis., Methods: Retrospective case series., Results: Aqueous or vitreous samples of 20 out of 35 eyes showed qRT-PCR positivity for virus etiology (57.14%). Cytomegalovirus (CMV) was most commonly identified in nine eyes (45%). The mean DNA copy number was 2,68,339.65 copies/mL (range: 90-3205397). DNA copy number significantly correlated with the extent of clinical involvement (P = 0.013); however, there was no correlation between DNA copy number and presenting visual acuity (P = 0.31), macular involvement (P = 0.675), optic nerve involvement (P = 0.14), and development of retinal detachment (P = 0.73). There was a significant correlation between the number of DNA copies and the timing of sampling (P = 0.0005). Samples taken earlier in the course of the disease had higher viral copies than later ones., Conclusion: qRT-PCR is useful in confirming a viral etiology in over 50% of cases of suspected viral retinitis. It correlates well with the extent of clinical involvement and timing of sampling., Competing Interests: None