Multimodal imaging in infectious and noninfectious intermediate, posterior and panuveitis.

PURPOSE OF REVIEW: Given the heterogeneity of uveitis, markers of inflammation vary from patient to patient. Multimodal imaging has proven itself to be critical for accurate evaluation for disease activity and treatment response in uveitis. RECENT FINDINGS: Ultra-widefield (UWF) fluorescein angiography and autofluorescence (AF) as well as optical coherence tomography angiography (OCTA) have provided insights into disease pathogenesis and monitoring not previously appreciated. In addition to structural retinal imaging, OCT can be used to assess the choroid, the posterior cortical vitreous and the retinal vasculature in eyes with uveitis. SUMMARY: Multimodal ocular imaging in eyes with uveitis is critical for disease diagnosis and assessing response to treatment. UWF fluorescein angiography can detect retinal vasculitis even in the absence of overt vascular sheathing. UWF AF can help detect more chorioretinal lesions than clinically visible. OCT can be used to assess the posterior cortical vitreous, retina, large retinal vessels and choroid in uveitis. The use of multimodal imaging will likely be needed to determine clinical trial endpoints in studies evaluating therapeutics for uveitis.

[Treatment of noninfectious intermediate uveitis, posterior uveitis, or panuveitis]. / Traitement des uvéites intermédiaires, postérieures et panuvéites non infectieuses.

Controlling long-term inflammation during non-infectious intermediate, posterior or panuveitis while limiting side effects remains challenging. There is no standardized pre-therapeutic evaluation providing diagnostic certainty, but some simple tests allow us to identifiy the main etiologies. The ophthalmologist identifies the type of uveitis, and the internist completes the investigations according to the ophthalmologist's findings. Fundus photographs, optical coherence tomography, and fluorescein and indocyanine green angiography should be considered during diagnosis and follow-up. Ocular complications of uveitis are numerous. They require close monitoring and specific medical and sometimes surgical management. The growing number of available drugs makes it possible to optimize the management of these conditions with varied etiologies and presentations. Currently, systemic corticosteroids remain the mainstay of therapy, and other alternatives are considered in the case of poor tolerance, steroid resistance or dependence. The choice of a systemic, periocular or intravitreal treatment depends on several factors: chronicity or recurrence of uveitis, duration, bilaterality, association with a systemic inflammatory disease, the presence of contraindications to certain treatments, and also socioeconomic constraints. It is of the utmost importance to find the best compromise allowing tight control of ocular inflammation by means of adapted systemic and/or local treatment while avoiding the main complications.

[Diagnostic Strategy and Therapeutic Dilemma in Acute Posterior and Panuveitis]. / Diagnostische Strategie und therapeutisches Dilemma bei akuter posteriorer und Panuveitis.

Acute posterior and panuveitis mostly affect younger patients and affect both eyes in more than half of cases. Because of the severe consequences in the clinical course, rapid and broad differential diagnosis are critical steps. Permanent loss of vision after a delay in starting therapy and the initiation of ineffective treatment are both serious risks. The initial diagnostic classification is based on clinical presentation (anatomical localisation and type of inflammation) and clinical course and, secondarily, on the response to acute therapy. The aetiology is acute in as many as one third of cases. The most frequent acute posterior uveitis in immunocompetent persons is acute viral retinal necrosis. It is difficult to distinguish this clinically from Behçet uveitis, as long as there are no systemic manifestations. In patients with disease threatening the macula, high dose steroid therapy must be started no later than 24 hours after the start of antiviral and anti-parasitic acute therapy. Thus, misdiagnosis has therapeutic consequences. Moreover, the prognosis is favourably affected by aggressive treatment of acute posterior uveitis. Any delay in starting therapy increases infectious and inflammatory tissue damage, and increases the risk of involvement of the other eye and of other organs. On the other hand, the use of high doses of steroids, immunosuppressives and biological agents can lead to uncontrolled proliferation of the pathogen and relapses.

Clinical Characteristics and Treatment of 308 Panuveitis Patients over 10 Years: Results from the KKESH Uveitis Survey Study Group.

Purpose: To describe the clinical characteristics and surgical outcomes of panuveitis over a 10-year period.Methods: Data were collected on panuveitis etiology, treatment, surgery and change in vision.Results: Overall, 308 patients (594 eyes) were evaluated, 54.9% with non-granulomatous and 45.1% with granulomatous uveitis. Fifty-four patients had infectious and 254 had non-infectious uveitis. In infectious panuveitis, vision remained unchanged at last visit. The most frequent diagnosis was presumed intraocular tuberculosis (PIOTB) uveitis (48.1%). In the non-infectious subgroup vision increased significantly by 2 lines at last visit (p = 0.020). The most common diagnose was Vogt-Koyanagi-Harada (VKH) disease (38.6%). Initial therapy was oral prednisone in 86.6% with non-infectious etiology followed by immunosuppressive agents. Surgery secondary to complications (mainly cataracts) was performed in 46.7% patients.Conclusion: In Saudi Arabia, panuveitis was mainly due to VHK. PIOTB was the most common etiology for infectious panuveitis. Immunosuppressive therapy and surgery maintained BCVA in non-infectious panuveitis.

Epidemiological profile of non-infectious uveitis from the rheumatologist's perspective: a survey from two tertiary referral centres in Italy.

OBJECTIVES: To describe the epidemiology of non-infectious uveitis (NIU) in two tertiary referral rheumatology units in Central and Southern Italy. METHODS: Two hundred and seventy-eight consecutive NIU patients (417 eyes) evaluated between January 2016 and January 2017 were enrolled. Collected data were analysed in accordance with the primary anatomic site of inflammation, clinical course, and laterality. RESULTS: The mean age at NIU onset was 36.92±18.30 years with a female-to-male ratio of 1.34:1. Anterior uveitis (AU) was identified in 151 (54.32%), posterior uveitis (PU) in 67 (24.10%), intermediate uveitis (IU) in 5.40% and panuveitis (PanU) in 16.19% patients. Bilateral involvement was identified in 50% of our cohort. Uveitis was acute in 33.81% of patients, while 24.46% and 41.73% had a chronic and recurrent course, respectively. Gender and laterality did not influence the anatomical pattern, while disease course was significantly more acute or chronic in AU (p<0.05) and chronic in IU (p<0.05). An associated systemic disease was identified in 116 patients (41.73%). Twenty-seven patients (9.7%) had a specific isolated eye disease, 135 patients (48.56%) had idiopathic NIU. Uveitis associated with a systemic disease was significantly bilateral (p=0.01) and acute or chronic (p<0.0001), while the isolated form showed an association with chronic course (p<0.0001) and unilaterality (p=0.01). CONCLUSIONS: The most common anatomic pattern of NIU has been AU, followed by PU, PanU and IU. A systemic disease (mainly Behçet's disease, ankylosing spondylitis and juvenile idiopathic arthritis) has been recognised in a fair proportion of the entire cohort. The rheumatologist should remain a central professional figure in the multidisciplinary team dealing with intraocular inflammation on a daily basis.

A Glimpse into Uveitis in the Aging Eye: Pathophysiology, Clinical Presentation and Treatment Considerations.

Uveitis describes a group of inflammatory conditions of the eye that have various underlying causes and clinical presentations. Susceptibilities to uveitis in the elderly may be attributed to age-related risk factors such as immunosenescence, increased immunological inflammatory mediators, and autoimmunity. Overall, anterior uveitis is more common than posterior and panuveitis in the general population and also in the elderly. Some causes of uveitis in the elderly are herpes simplex virus, ocular ischemic syndrome, sarcoidosis, and central nervous system lymphoma, and these will be discussed in detail herein. Eye care professionals need to consider the wide differential for uveitis, obtain the appropriate history, conduct a detailed clinical examination, and tailor management to the clinical presentation and underlying cause of disease. The challenges of polypharmacy and nonadherence in the elderly impact patient outcomes and must be taken into consideration when considering treatment.

Successful multi-modality approach in management of human leukocyte antigen-B27 associated fulminant panuveitis - rare presentation.

Objective: To report a multimodality approach in the management of human leukocyte antigen B27 (HLA-B27) associated fulminant posterior uveitis, an uncommon presentation, with good visual and anatomical recovery. Methods: A 33-year-old young male presented with HLA-B27 associated severe posterior uveitis, which is a relatively uncommon presentation. The patient had severe vitritis with papillitis, which was sequentially and stepwise managed with oral steroids, pars plana vitrectomy, immunosuppressive agents and sustained release intravitreal steroid implant. Results: The patient had a good recovery of vision with complete resolution of inflammation and without any long-term complication. Conclusion: HLAB27 positivity can be associated with an uncommon presentation of fulminant posterior uveitis that requires a judicious and stepwise multimodality approach in its management, and can have a good visual and anatomical outcome as demonstrated in our case.

Subretinal echinococcosis: a case report.

BACKGROUND: Echinococcosis is a dangerous zoonotic parasitic disease. Ocular echinococcosis is very rare, especially the hydatid cysts in subretinal space. We present a case of subretinal echinococcosis and management. CASE PRESENTATION: A 37-year-old man with subretinal echinococcosis who developed panuveitis and visual impairment. The patient lives on agriculture and animal husbandry, which made him susceptible to parasitic infection. He had severe panuveitis and blurred vision on arrival at hospital. According to his ocular examination and systemic review, the subretinal echinococcosis diagnosis was made. The patient received pars plana lensectomy and pars plana vitrectomy. The lesion underneath his retina was removed, and histopathology examination confirmed the subretinal echinococcosis diagnosis. CONCLUSIONS: Echinococcosis is a dangerous zoonotic parasitic disease in pastoral areas. Ocular echinococcosis is usually secondary to systemic infection. Although the incidence is rare, the disease could lead to destructive visual function impairment.

[Vogt-Koyanagi-Harada disease]. / La maladie de Vogt-Koyanagi-Harada.

Vogt-Koyanagi-Harada (VKH) disease is defined as a severe bilateral, chronic granulomatous panuveitis associated with serous retinal detachments, disk edema, and vitritis, with central nervous system, auditory, and integumentary manifestations. It is an autoimmune inflammatory condition mediated by T cells that target melanocytes in individuals genetically susceptible to the disease. Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal, acute inflammatory, chronic, and recurrent, with extraocular manifestations including headache, meningitis, hearing loss, poliosis, and vitiligo. Optical coherence tomography (OCT) allows earlier diagnosis of VKH disease by revealing heterogeneous exudative detachments of the retina in the acute stage and choroidal thickening, and by demonstrating choroidal thinning in the chronic stage. Treatment of this disease is initially with intravenous corticosteroids, with, if needed, a transition to immunosuppressant drugs for long-term control. Patients with VKH disease can have good final visual outcomes if treated promptly and aggressively.

Management of a rare presentation of Vogt-Koyanagi-Harada disease in human immunodeficiency virus/acquired immunodeficiency disease syndrome patient.

Vogt-Koyanagi-Harada (VKH), a multisystem autoimmune bilateral panuveitis with systemic manifestations, is uncommon in immunocompromised patients such as human immunodeficiency virus (HIV)/acquired immunodeficiency disease syndrome (AIDS). We report a rare presentation of VKH in a 45-year-old HIV-positive female on highly active antiretroviral therapy (HAART) who presented with a history of recurrent panuveitis. A diagnosis of probable VKH was made based on ocular and systemic signs and symptoms. She was treated with topical and systemic steroids with close monitoring of CD4 counts and viral loads. After inflammation control, complicated cataract was managed surgically under perioperative steroid cover. VKH in HIV/AIDS has not been reported earlier. This case shows that significant inflammation can be seen even in HIV/AIDS patients on HAART with VKH in spite of moderate CD4 counts. Management is a challenge considering the systemic risks with long-term use of steroids.

Canine panuveitis: a retrospective evaluation of 55 cases (2000-2015).

OBJECTIVE: To determine the most common etiologies of panuveitis in a group of dogs, patient response to therapy, and visual outcome. DESIGN: Retrospective study. MATERIALS AND METHODS: Medical records of 55 dogs diagnosed with panuveitis at the Purdue University Veterinary Teaching Hospital between December 2000 and December 2015 were reviewed. Data collected included signalment, season of diagnosis, descriptions of ocular lesions, diagnostic testing, treatment protocols, etiologic diagnoses (when determined), and visual outcome. RESULTS: The most common clinical sign in the anterior segment was aqueous flare and in the posterior segment was serous retinal detachment. The most common diagnosis was idiopathic/immune-mediated disease followed by systemic blastomycosis, lymphoma, and other causes (leptospirosis, aspergillosis, and histoplasmosis). Of the 43 dogs for which follow-up was available, 40% of dogs had resolution of visible inflammation and retinal reattachment, 32% of dogs had slight to moderate improvement, and 28% of dogs had no improvement or deterioration of lesions. Mean follow-up time was 11 months, and mean time to resolution of visible inflammation and retinal reattachment was 32 days. Sixty-five percent of dogs either retained or regained vision in one or both diseased eyes with initial medical therapy. CONCLUSIONS: Idiopathic/immune-mediated panuveitis was the most common diagnosis. Dogs with idiopathic/immune-mediated panuveitis tended to respond more favorably to medical therapy than dogs with infectious panuveitis. Visual outcome was favorable in those dogs that responded to initial medical therapy, but long-term therapy was required in many dogs to maintain vision and prevent recurrence of disease.

Multifocal Choroiditis and Panuveitis - difficulties in diagnosis and treatment.

We present the case of a 49-year-old patient who was treated with Aripriprazole in context of Paranoid Schizophrenia. The patient had a history of numerous Panuveitis recurrences for the left eye, which led to a marked decrease of the vision VA-NLP and was diagnosed with Multifocal Choroiditis and Panuveitis for the right eye. The examination revealed VA of 20/ 200 for right eye, keratic precipitates, and vitritis. Fundus aspect of the right eye showed multiple discrete, ovoid, yellowish-grey lesions at the posterior pole and periphery, optic disc oedema was present. The Human leukocyte antigen typing of group A, ancillary investigation (OCT, Angiofluorography, B-mode ultrasonography) and fundus examination confirmed the diagnosis of Multifocal Choroiditis and Panuveitis but we did not exclude antipsychotic-related chorioretinopathy or a Birdshot-like Syndrome.

Coriorretinitis placoide sifilítica. Caso clínico / Syphilitic placoid chorioretinitis. A case-report

CASO CLÍNICO: Presentamos el caso de una mujer de 45 años sin antecedentes de interés y con una pérdida súbita de visión en su ojo izquierdo secundaria a una uveítis posterior bilateral. Tras despistaje, se diagnosticó de coriorretinitis placoide posterior aguda sifilítica, y recibió tratamiento con penicilina intravenosa. Discusión: Existen múltiples manifestaciones oculares de la sífilis que pueden simular cuadros y etiologías muy diversas. El tratamiento anti-treponémico normalmente produce una rápida y positiva respuesta en pacientes afectos. El diagnóstico precoz y certero de estos pacientes es por tanto crucial aunque, en ocasiones, los daños anatómicos y funcionales son irreversibles

CLINICAL CASE: We report the case of a 45-year-old woman, with unremarkable past medical history, who presented with acute visual loss in her left eye due to bilateral posterior uveitis. After the screening, she was diagnosed with acute syphilitic placoid chorioretinitis and was treated with intravenous penicillin. DISCUSSION: Clinical manifestations of ocular syphilis are extremely heterogeneous and may mimic several aetiologies. Anti-treponema treatment usually induces a quick and positive response in affected patients. Prompt and proper diagnosis of these patients is crucial, although anatomical and functional damage may persist

Choroidal Vascularity Index (CVI)--A Novel Optical Coherence Tomography Parameter for Monitoring Patients with Panuveitis?

PURPOSE: To compute choroidal vascularity index (CVI) using an image binarization tool on enhanced depth imaging (EDI)-optical coherence tomography (OCT) scans as a non-invasive optical tool to monitor progression in panuveitis and to investigate the utility of volumetric data from EDI-OCT scans using custom image analysis software. MATERIALS AND METHODS: In this retrospective cohort study, segmented EDI-OCT scans of both eyes in 19 patients with panuveitis were taken at baseline and at 3-month follow-up and were compared with EDI-OCT scans of normal eyes. Subfoveal choroidal area was segmented into luminal (LA) and stromal interstitial area (SA). Choroidal vascularity index (CVI) was defined as the proportion of LA to the total circumscribed subfoveal choroidal area (TCA). RESULTS: The mean choroidal thickness was 265.5±100.1µm at baseline and 278.4±102.6µm at 3 months follow up (p = 0.06). There was no statistically significant difference in TCA between study and control eyes (p = 0.08). CVI in the control group was 66.9±1.5% at baseline and 66.4±1.5% at follow up. CVI was 74.1±4.7% at baseline and 69.4±4.8% at 3 months follow up for uveitic eyes (p<0.001). The % change in CVI was 6.2 ±3.8 (4.3 to 8.0) for uveitic eyes, which was significantly higher from % change in CVI for control eyes (0.7±1.1, 0.2 to 1.3, p<0.001). CONCLUSION: The study reports composite OCT-derived parameters and CVI as a possible novel tool in monitoring progression in panuveitis. CVI may be further validated in larger studies as a novel optical tool to quantify choroidal vascular status.

Uveítis sifilítica asociada al virus de inmunodeficiencia humana / Syphilitic uveitis associated with the human immunodeficiency virus

La sífilis es una infección crónica y multisistémica, producida por la espiroqueta Treponema pallidum. Las manifestaciones oculares de la sífilis son varias y pueden afectar todas las estructuras oculares. La uveítis es el tipo de afectación ocular más frecuente. Puede presentarse en la fase secundaria, latente o terciaria y cursa de forma bilateral en el 50 por ciento de los casos. También puede verse asociada al virus de inmunodeficiencia humana. Desde la introducción de la terapia antirretroviral de gran actividad, la mortalidad asociada al SIDA disminuyó y la incidencia de sífilis y otras enfermedades de trasmisi¾n sexual aumentó como consecuencia de la expansión de la población homosexual. Teniendo en cuenta la gran variedad de formas de presentación, se le conoce como la gran simuladora y forma parte del diagnóstico diferencial de cualquier uveítis. Presentamos el caso de un paciente masculino de 64 años de edad que acude al Cuerpo de Guardia, donde refiere visión borrosa del ojo derecho de aproximadamente 45 días de evolución, asociado a enrojecimiento ocular, dolor y molestias a la luz, a quien posteriormente se le diagnóstico una uveítis sifilítica asociada al virus de inmunodeficincia humana(AU)

Syphilis is a chronic and multisystemic infection, caused by Treponema Pallidum spirochetes. The ocular manifestations of syphilis are varied and may affect all the ocular structures, being uveÝtis the most common type of ocular effect. It can appear in the secondary, latent or tertiary phase and in a bilateral form in 50 percent of the cases, it may be associated with the human immunodeficiency virus. Since the introduction of the highly active antiretroviral therapy, AIDS-associated mortality lowered whereas the incidence of syphilis and other sexually transmitted diseases increased as a result of the rise of homosexual population. Due to their great variety in the ways of presentation, syphilis is known as the great simulator and is part of the differential diagnosis of any kind of uveitis. Here is the case of a 64 year-old male patient who went to the Emergency Service, because he felt blurred vision in his right eye for 45 days approximately. Additionally, he presented with eye reddening, pain and discomfort when looking into the light; finally he was diagnosed as human immunodeficiency virus -associated syphilitic uveitis case(AU)

Seasonal hyperacute panuveitis: an update.

PURPOSE OF REVIEW: Seasonal hyperacute panuveitis (SHAPU), a mysterious disease of Nepal, has increased the burden of childhood blindness ever since it was described in 1975. Although moths are suspected as the cause, this source is not yet proven. The management of this disease is still not well outlined. This article reviews recent developments in the understanding of SHAPU pathogenesis, clinical features, cause, and preventive measures as well as curative management. RECENT FINDINGS: Smaller summer outbreaks occur every even year together with larger autumn outbreaks every odd year. Vitreous fluid from some cases grew bacteria on culture. Viruses were isolated in one case. Tiny hair follicles have been identified in ocular structures. Several cases had decreased corneal sensation and few cases had iris atrophy. Early vitrectomy is beneficial. Intravitreal vancomycin, amikacin, and dexamethasone injection have given good results. Unlike previous reports, the use of repeated subconjunctival dexamethasone injection was also claimed to reverse the eye disease. However, treatment helped to save the vision in one eye or both only if cases presented early enough (within 7 days) for treatment. SUMMARY: Recent findings have shown a correlation between SHAPU and moths (probably Gazalina); therefore, more research on this moth is urged. Various new findings also hint towards the involvement of microbes; thus, further advanced studies are required to confirm this involvement.

Current approach in the diagnosis and management of panuveitis.

Panuveitis is a generalized inflammation of not only the whole of the uveal tract but also involves the retina and vitreous humor. It differs from other anatomical sites of inflammation in terms of causes as well as distribution. The common causes of panuveitis in our population are tuberculosis, Vogt-Koyanagi-Harada syndrome, sympathetic ophthalmia, Behcet's disease and sarcoidosis. A large number of cases still remain idiopathic. A stepwise approach is essential while evaluating these patients to be able to identify and treat the disease timely and correctly. Ancillary tests can be appropriately applied once the anatomic site of inflammation is identified. An exhaustive approach comprising a full battery of tests is obsolete. Only specific tailored investigations are ordered as suggested by the preliminary clinical and ocular examination. The mainstay of the treatment of uveitis is corticosteroids. Immunosuppressive agents are administered if the inflammation is not adequately controlled with corticosteroids. One of the recent breakthroughs in the treatment of refractory uveitis includes the introduction of immunomodulating drugs: Tumor necrosis factor-alpha antagonist and Interferon-alpha. Vitrectomy has been used in uveitis for over a few decades for diagnostic and therapeutic purposes. When compared to other anatomical sites of inflammation, panuveitis has poor visual outcome due to more widespread inflammation. The side-effects of the chronic treatment that these patients receive cannot be overlooked and should be specifically monitored under the supervision of an internist with special interest in inflammatory diseases.

Panuveítis bilateral sifilítica: a propósito de un caso / Syphilitic bilateral panuveitis: a case report

Caso clínico: Mujer de 57 años inmunocompetentees diagnosticada de panuveítis bilateral sifilíticatras presentar un cuadro dramático de déficit visualsevero y bilateral con inflamación del segmentoanterior, importante vitritis y coriorretinitis que seresuelven favorablemente tras el tratamiento.Discusión: La sífilis es una enfermedad cuya frecuenciaestá en aumento. A nivel ocular puede provocarpérdida severa de visión si no se diagnosticaa tiempo ya que puede originar neuritis y coriorretinitiscon panuveítis, siendo el 50% de ellas bilaterales.Sin embargo, se considera una de las pocascausas de uveítis que con tratamiento específico sepueden curar(AU)

Case report: A 57-year-old immunocompetentwoman was diagnosed with syphilitic bilateralpanuveitis after the onset of dramatic bilateralvisual loss with inflammation of the anterior segmentof the eye, severe vitritis and chorioretinitiswhich improved after treatment.Discussion: Syphilis is a complex disease of increasingprevalence. If diagnosis is delayed ocularsyphilis can produce severe visual loss due to neuritis,chorioretinitis with panuveitis, affecting botheyes in 50% of cases. Nevertheless, syphilis is consideredone of the few causes of uveitis in which acure can be obtained with proper treatment(AU)