[Intermediate and Posterior Uveitis - Disease entities]. / Intermediäre und posteriore Uveitis ­ Teil 2.

Intermediate and posterior uveitis can have multiple infectious and noninfectious causes, and posterior uveitis in particular is clinically multifaceted. Some entities require prompt initiation of therapy to ensure visual prognosis. This article presents typical characteristics of intermediate and posterior uveitides and explains special features of their treatment.

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.

Immune modulator therapy compared with vitrectomy for management of complicated intermediate uveitis: a prospective, randomized clinical study.

PURPOSE: To compare the benefits and side effects of pars plana vitrectomy with those of systemic immune modulator therapy for patients with complicated intermediate uveitis. METHODS: This prospective clinical trial enrolled patients with recurrent intermediate uveitis who exhibited minimal improvement of visual acuity, despite injections of periocular steroids. Twenty patients were randomized to the pars plana vitrectomy group or oral steroid and cyclosporine-A group (10 eyes of 10 patients per group). Follow-up was performed for 24 months to study changes in visual acuity, binocular indirect ophthalmoscopy score, fluorescein angiography, and optical coherence tomography findings. RESULTS: Visual acuity (logarithm of the minimal angle of resolution) significantly improved from 0.71 to 0.42 (p=0.001) in the surgical group, whereas it improved from 0.68 to 0.43 (p=0.001) in the immune modulator therapy group. Seven patients (70%) in the surgical group gained ≥2 lines, and six patients (60%) in the immune modulator therapy group gained ≥2 lines (p=0.970). Fluorescein angiography and optical coherence tomography studies showed that six of seven pars plana vitrectomy patients who had cystoid macular edema experienced improvement, whereas two patients with diffuse macular edema did not experience improvement. In the immune modulator therapy group, three of six patients with cystoid macular edema did not experience improvement, whereas two patients with diffuse macular edema experienced improvement. CONCLUSIONS: Pars plana vitrectomy and immune modulator therapy resulted in significant improvement in visual function in patients with persistent inflammation secondary to chronic intermediate uveitis. Despite this success, there remains a need for the determination of optimal indications for the use of each modality. Immune modulator therapy was successful for the treatment of diffuse macular edema associated with chronic intermediate uveitis, whereas pars plana vitrectomy was not.

Immune modulator therapy compared with vitrectomy for management of complicated intermediate uveitis: a prospective, randomized clinical study / Terapia com imunomodulador ou vitrectomia para manejo de uveíte intermediária complicada: estudo clínico prospectivo e randomizado

ABSTRACT Purpose: To compare the benefits and side effects of pars plana vitrectomy with those of systemic immune modulator therapy for patients with complicated intermediate uveitis. Methods: This prospective clinical trial enrolled patients with recurrent intermediate uveitis who exhibited minimal improvement of visual acuity, despite injections of periocular steroids. Twenty patients were randomized to the pars plana vitrectomy group or oral steroid and cyclosporine-A group (10 eyes of 10 patients per group). Follow-up was performed for 24 months to study changes in visual acuity, binocular indirect ophthalmoscopy score, fluorescein angiography, and optical coherence tomography findings. Results: Visual acuity (logarithm of the minimal angle of resolution) significantly improved from 0.71 to 0.42 (p=0.001) in the surgical group, whereas it improved from 0.68 to 0.43 (p=0.001) in the immune modulator therapy group. Seven patients (70%) in the surgical group gained ≥2 lines, and six patients (60%) in the immune modulator therapy group gained ≥2 lines (p=0.970). Fluorescein angiography and optical coherence tomography studies showed that six of seven pars plana vitrectomy patients who had cystoid macular edema experienced improvement, whereas two patients with diffuse macular edema did not experience improvement. In the immune modulator therapy group, three of six patients with cystoid macular edema did not experience improvement, whereas two patients with diffuse macular edema experienced improvement. Conclusions: Pars plana vitrectomy and immune modulator therapy resulted in significant improvement in visual function in patients with persistent inflammation secondary to chronic intermediate uveitis. Despite this success, there remains a need for the determination of optimal indications for the use of each modality. Immune modulator therapy was successful for the treatment of diffuse macular edema associated with chronic intermediate uveitis, whereas pars plana vitrectomy was not.

RESUMO Objetivo: Comparar os benefícios e efeitos co laterais da vitrectomia via pars plana com os da terapia imunomo duladora sistêmica em pacientes com uveíte intermediária complicada. Métodos: Estudo clínico prospectivo incluiu pacientes com uveíte intermediária recorrente que apresentaram melhora minima da acuidade visual, apesar das injeções perioculares de esteroides. Vinte pacientes foram randomizados para o grupo de vitrectomia via pars plana ou esteróide oral e ciclosporina A (10 olhos de 10 pacientes por grupo). O acompanhamento foi de 24 meses para estudar al te rações na acuidade visual, o escore da oftalmoscopia binocular indireta, a angiofluoresceinografia e achados na to mográfica de coerência óptica. Resultados: A acuidade visual (logaritmo do ângulo mínimo de resolução) melhorou significativamente de 0,71 para 0,42 (p=0,001) no grupo cirúrgico, enquanto melhorou de 0,68 para 0,43 (p=0,001) no grupo da terapia imunomoduladora. Sete pacientes (70%) no grupo cirúrgico ganharam ≥2 linhas e seis pacientes (60%) no grupo da terapia imunomoduladora ganharam ≥2 linhas (p=0,970). Os estudos de angiofluoresceinografia e tomografia de coerência óptica mostraram que seis dos sete pacientes da vitrectomia via pars plana que apresentaram edema macular cistóide melhoraram, enquanto dois pacientes com edema macular difuso não apresentaram melhora. No grupo da terapia imunomoduladora, três dos seis pacientes com edema macular cistoide não apresentaram melhora, enquanto dois pacientes com edema macular difuso melhoraram. Conclusões: A vitrectomia via pars plana e a terapia imunomoduladora resultaram em melhora significative da função visual dos pacientes com inflamação persistente secundária a uveíte intermediária crônica. Apesar desse sucesso, continua sendo necessário determinar as melhores indicações para o uso de cada modalidade. A terapia imunomoduladora foi bem sucedida no tratamento do edema macular difuso associado à uveíte intermediária crônica, enquanto a vitrectomia via pars plana não foi.

[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.

Chronic candida endophthalmitis as a cause of intermediate uveitis.

Intermediate uveitis is a subset of intraocular inflammation where vitritis is the most consistent sign, with or without snowball opacities or snow banks over the pars plana. Some patients will have an associated underlying systemic disease such as sarcoidosis, multiple sclerosis, ocular tuberculosis, inflammatory bowel disease, possibly Behçet's disease and intraocular lymphoma, whereas some will be classified as pars planitis in case of the lack of an identifiable systemic disease association. Our patient, a 47-year-old woman, developed intermediate uveitis after cataract surgery in her right eye, was misdiagnosed as pars planitis, and received steroid monotherapy for 8 months. Her inflammation only fully resolved after vitrectomy with removal of the intraocular lens (IOL) and capsular bag. Oral fluconazole and intravitreal amphotericin B injection had failed to resolve her inflammation when Candida albicans was identified as the cause of her persistent intermediate uveitis.

Factors affecting therapeutic decisions in intermediate and posterior uveitis.

PURPOSE: To evaluate whether cost, insurance prior authorization, pregnancy considerations, and subspecialty practice lead to changes in therapeutic decisions among uveitis and retina specialists in the treatment of intermediate and posterior uveitis. DESIGN: Prospective cross-sectional study. METHODS: A total of 934 uveitis and retina specialists across the United States were surveyed via e-mail regarding their choice in long-term therapy for 3 hypothetical patients with uveitic conditions (Behçet disease, birdshot retinochoroiditis, and intermediate uveitis). Respondents were asked to select first- and second-choice therapies and then reselect first and second choices assuming cost of all options was equal to the patient and there would be no issues with insurance prior authorization. In 1 case, they were additionally asked for their treatment preferences if the patient desired a pregnancy. Outcomes of interest were differences in therapy choice based on cost/prior authorization, pregnancy, and subspecialty practice. RESULTS: One hundred and six respondents (11.3%) completed the survey; 44 were uveitis specialists and 62 retina specialists. Cost and prior authorization affected the therapy choices of uveitis specialists treating ocular Behçet disease (P = .008). Uveitis specialists and retina specialists differed in their first choice in therapy for each vignette when cost and prior authorization were equalized (P = .0018, P = .0049, and P = .0156). Both uveitis specialists and retina specialists changed their therapeutic choices for intermediate uveitis when pregnancy was a consideration (P = .00001 for uveitis specialists, P = .0044 for retina specialists). CONCLUSIONS: Physician decision making in intermediate and posterior uveitis is affected by cost and prior authorization concerns, pregnancy considerations, and subspecialty practice.

Pacientes con pars planitis tratados con fotocoagulación selectiva: curso clínico / Clinical course of pars planitis in patients treated with selective photocoagulation

Introducción: La pars planitis es una uveítis intermedia, con una presentación bilateral y asimétrica. La etiología es desconocida y la patogenia no está clara. El tratamiento sigue el algoritmo de Foster, donde se incluye la fotocoagulación selectiva. El mecanismo de acción de la fotocoagulación es desconocido. Materiales y métodos: Estudio observacional, longitudinal, cohorte ambilectivo. El objetivo fue evaluar el curso de la inflamación en pacientes con pars planitis tratados con láser argón selectivo. Resultados: Se incluyó a 29 pacientes con diagnóstico de pars planitis tratados con láser selectivo; 10 pacientes eran del género femenino y 19 del masculino. La edad promedio de inicio de la enfermedad fue 11,37 años. De los pacientes, en el momento de recibir el láser selectivo, 18 (62,1%) no se encontraban con inmunosupresión y 11 (37,9%) estaban con inmunosupresor. Las indicaciones de láser fueron por: seguimiento del algoritmo 19 (65,55%), hemorragia vítrea siete (24,1%), vitrectomía dos (6,98%) y neovasos uno (3,4%). El tiempo promedio de disminución de la inflamación fue de 5,9 meses y 17 pacientes (58,6%) no presentaron recaída. Las agudezas visuales presentaron mejoría posláser (OD p = 0,025 y OI p = 0,022). Se observó mejoría clínica de la celularidad vítrea. Conclusión: El láser selectivo demostró ser efectivo en el 58,6%% de los pacientes (AU)

Introduction: Pars planitis is an intermediate uveitis with bilateral and asymmetric presentation. The etiology is unknown and pathogenesis is unclear. Treatment follows the algorithm of Foster, which includes selective photocoagulation. The mechanism of action of photocoagulation is still unknown. Material and methods: An observational, longitudinal, ambispective cohort study was performed with the objective of evaluating the course of inflammation in patients with pars planitis treated with a selective argon laser. Results: The study included 29 patients (10 female and 19 male) diagnosed with pars planitis and were treated with selective laser. The mean age of onset was 11.77 years. Eighteen (62.1%) patients were not immunosuppressed at the time of receiving the selective laser, and 11 (37.9%) were taking immunosuppressants. Indications for selective laser were; following the algorithm, 19 (65.55%), vitreous hemorrhage 7 (24.1%), vitrectomy 2 (6.98%), and neovascularization 1 (3.4%). The mean time for inflammation reduction was 5.9 months, and 17 patients (58.6%) had no relapse. Visual acuity showed improvement post-laser (OD P = 0.025 and OI P = 0.022). There was also an improvement in vitreous cells. Conclusion: Selective laser was effective in 58.6%% of patients (AU)

Pars plana vitrectomy versus immunomodulatory therapy for intermediate uveitis: a prospective, randomized pilot study.

PURPOSE: Comparison of pars plana vitrectomy (PPV) with immunomodulatory therapy (IMT) for patients with intermediate uveitis (IU). METHODS: A prospective, randomized pilot study was performed on patients with recalcitrant IU associated with degradation of visual acuity (VA) despite standard treatment. Outcome measures (VA, intraocular pressure, anterior chamber and vitreous cellular infiltrate) were collected. RESULTS: Sixteen patients (18 eyes) were randomized to the PPV IMT group. Nine of 11 eyes (82%) treated with PPV showed resolution of inflammation at follow-up, at 5.93 years. Four of 7 eyes (57%) given IMT had persistent inflammation requiring subsequent PPV. PPV patients showed greater improvement in Snellen line, IOP, and vitreous cell reduction. Three PPV patients had cystoid macular edema (CME) initially; all resolved postoperatively. CME improved in 2 of 3 eyes using IMT. CONCLUSIONS: A higher percentage of patients treated with PPV had improvement of uveitis compared to those given IMT. A multicentered clinical trial is needed to confirm and statistically validate these conclusions.

Intermediate uveitis.

Intermediate uveitis (IU) is described as inflammation in the anterior vitreous, ciliary body and the peripheral retina. In the Standardization of Uveitis Nomenclature (SUN) working group's international workshop for reporting clinical data the consensus reached was that the term IU should be used for that subset of uveitis where the vitreous is the major site of the inflammation and if there is an associated infection (for example, Lyme disease) or systemic disease (for example, sarcoidosis). The diagnostic term pars planitis should be used only for that subset of IU where there is snow bank or snowball formation occurring in the absence of an associated infection or systemic disease (that is, "idiopathic"). This article discusses the clinical features, etiology, pathogenesis, investigations and treatment of IU.

[Use of plasmapheresis-based extracorporeal hemocorrection in the treatment of endogenic uveitis and autoimmune eye diseases].

The paper gives the methods of plasmapheresis-based extracorporeal exposure to blood and its components to correct hemostatic disorders. Afferent methods, as well as quantum hemotherapy methods are outlined. The immunomodulating mechanism of their action, which favors a prompter elimination of inflammation, increases ocular functions, and reduces recurrences, is revealed. The mechanisms of action of plasmapheresis as a technique used alone and in combination with laser and ultraviolet blood irradiation and indications for and contraindications to the use of various hemocorrection methods are described.

Endoteliopatía corneal autoinmune como manifestación de uveitis intermedia / Corneal autoimmune endotheliopathy as manifestation of intermediate uveitis in children

Background: Intermediate Uveitis (IU) is an important cause of uveitis in children. It is considered a chronic intraocular inflammation that mainly affects the anterior vitreous and peripheral retina. Pars Planitis is a subtype of IU, consisting of a white opacity that covers the pars plana and ora serrata plus vitreous condensations in the eye. Corneal endothelium disease is a rare clinical finding associated with pars planitis. Objective: Describe the corneal manifestations in IU and its associated treatment between ophthalmology and pediatric rheumatology. Case-report: A 5 years-old boy with autoimmune endotheliopathy and unilateral pars planitis in the right eye. Initially, he was treated topically, but evolved with intraocular complications that required systemic medication. Long-term follow-up was performed, searching for associated systemic diseases, until one of these entities appeared. Conclusion: It is very important to search for inflammation in the anterior vitreous and pars plana in all children with corneal endotheliopathy, considering that an early and integral management of IU could diminish the risk of visual impairment as a complication.

Introducción: La Uveitis Intermedia (UI) es una causa importante de uveitis infantil. Se considera como una inflamación intraocular crónica que afecta principalmente a la retina periférica y vitreo anterior. La Pars Planitis es un subtipo de UI caracterizada por opacidades blanquecinas sobre la pars plana y ora serrata más condensaciones vitreas. La enfermedad del endotelio corneal es un hallazgo clínico infrecuente asociado con la Pars Planitis. Objetivo: Destacar la manifestación corneal de una UI y el tratamiento de la enfermedad entre oftalmólogo y reumatólogo infantil. Caso clínico: Se presenta caso clínico de niño de 5 años de edad con endoteliopatía autoinmune asociada a Pars Planitis unilateral del ojo derecho, que en principio se trató localmente, pero evolucionó con complicaciones intraoculares que requirieron medicación sistémica y se efectuó un seguimiento clínico por varios años buscando enfermedades sistémicas asociadas a UI hasta que ella apareció. Conclusión: Es necesario buscar la presencia de inflamación de pars plana y vitreo en todo niño que se presente con endoteliopatía corneal, considerando que el tratamiento oportuno y manejo integral del paciente con UI puede disminuir el riesgo de pérdida visual asociado a las complicaciones de esta enfermedad.

[Uveitis intermedia in Whipple's disease]. / Uveitis intermedia bei Morbus Whipple.

We report a case of a 45-year-old man who complained of progressive vision loss in his right eye. Visual acuity was 20/300 in the right eye and 20/25 in the left eye. Bilateral uveitis intermedia R>L was diagnosed and treated with systemic and local steroids. An internal checkup was also done, and duodenal biopsy identified Whipple's disease. Despite specific antibiotic therapy, the patient's follow-up examination showed increased inflammatory activity R>L and bilateral cataracta complicata. Cataract surgery and pars plana vitrectomy with removal of epiretinal membranes were done. Histologic analysis of the vitreous and epiretinal membranes showed periodic acid-Schiff-positive macrophages, pathognomonic for Whipple's disease. Whipple's disease is a rare but severe disease with multiple manifestations and should be considered a differential diagnosis in uveitis.

Intermediate and posterior uveitis.

Sight-threatening intraocular inflammation affecting the posterior segment of the eye may be predominantly located in the peripheral retina and vitreous (intermediate uveitis) or postequatorially where it manifests as inflammation of the retina, retinal vessels and/or optic nerve with cellular infiltration of the choroid and retina and edema particularly at the macula. Involvement of the macula is the main cause for visual loss. Experimental models of posterior uveitis have revealed much concerning the mechanisms of inflammatory cell damage to the retina, implicating CD4 T cells, effector macrophages and pro-inflammatory cytokines. In particular, transgenic and gene deletion models of inflammation have allowed an understanding of how immune privilege in the posterior segment of the eye is disrupted. Importantly, this has led to the development of new treatments with novel immunosuppressants and 'biologics' and the promise of cell-based therapies which may allow customized therapies tailored to the individual's inflammatory profile.

[Estimation of immunological markers in children with intermediate uveitis symptoms]. / Ocena wybranych wykladników odpowiedzi immunologicznej u dzieci z objawami zapalenia czesci posredniej blony naczyniowej.

PURPOSE: The purpose of this study was to estimate markers of immunological response in the blood of children with intermediate uveitis. MATERIAL AND METHODS: The records of 13 children (26 eyes), aged 10-17 years, with idiopathic intermediate uveitis were reviewed. In all cases indicators of inflammation and immunological response parameters were analyzed during the active stage of the disease. RESULTS: In almost all patients ((90%) with intermediate uveitis disturbances in the level and activity of lymphocyte system, were detected. There were also nonspecific changes in immunoglobulins level in all patients, in 31% in more then one class of immunoglobulins. Chemiluminescence of phagocytic cells were reduced in more than 50% of children, and increase in their phagocytic activity was observed in 62% of patients. Circulating immune complexes were present in 50% of cases. CONCLUSIONS: The study presents data supporting the theory of autoimmunological background of the intermediate uveitis.

Intermediate uveitis.

Intermediate uveitis is an intraocular inflammation involving the anterior vitreous, peripheral retina and pars plana. It usually affects patients from 5 to 30 years old, without gender or racial preferences. The etiology is unknown but there are several associated diseases: multiple sclerosis, idiopathic optic neuritis, autoimmune corneal endotheliopathy, sarcoidosis, thyroid diseases and inflammatory bowel diseases. Symptoms are blurry vision, floaters and distortion of central vision. The syndrome is bilateral in 80% of the patients and chronic with periods of exacerbation and remission. Clinical presentation includes: mild to moderate anterior chamber inflammation, thin keratic precipitates in the inferior portion of the cornea, autoimmune endotheliopathy, vitreitis, vasculitis in the peripheral retina, intravitreal "snowballs," retinal "snowbanking," optic neuritis and cystoid macular edema. Cataract and glaucoma are frequent complications. Treatment of intermediate uveitis is based on periocular and oral corticosteroids. Cryotherapy or laser photocoagulation of the peripheral retina are options in patients with snowbanking when there is an insufficient response to periocular or systemic corticosteroids. Imunosuppression may also be used when other therapies fail, and Cyclosporin A is the first drug of choice. Pars plana vitrectomy is indicated in patients with chronic significant inflammation, non-responsive cystoid macular edema, non-clearing vitreous hemorrhage, tractional retinal detachment and epiretinal membranes. The long-term prognosis of intermediate uveitis is usually good, particularly with strict control of inflammation and with proper management of complications. Patients can often maintain a vision of 20/50 or better.

Intermediate uveitis: a hospital based study.

UNLABELLED: Intermediate uveitis affecting pars plana, peripheral retina and underlying choroid affects primarily healthy children and young adults. OBJECTIVE: To study the components of intermediate uveitis at Nepal Eye Hospital in the year 2003 August to 2004 March. MATERIALS AND METHODS: A prospective study was done in patients with intermediate uveitis visiting Nepal Eye Hospital. Sample size was 50. Specially designed proforma of intermediate uveitis was used to collect particulars of the patients, history, clinical examination findings, investigation reports, management protocols used and the final visual outcome. RESULTS: Intermediate uveitis was seen more in 20 to 29 years age group (38%) and more prevalent in males (52%). Among the study population farmers constituted maximum proportion (36%) and Mongoloid origin was mostly affected (30%). High number of patients were from the central development region with highest proportion from Dhading district (34%). Anterior segment affection with keratic precipitates and anterior chamber cells were noted in 72% of involved eyes. Vitreous cells were noted in all affected eyes while 4% had vitreous snow banking and 3% had vitreous snow balls. Most of the patients (52%) received between topical steroid and posterior subtenon injection of Triamcinolone. Visual Acuity was improved in 52% of cases and remained static in 46%.while deteriorated in 2%. CONCLUSION: Significantly high number of patients with intermediate uveitis has been reported from the central development region mainly from Dhading Kathmandu and Nuwakot districts People in their third decade were found to be commonly involved and Mongloid origin were more commonly affected Anterior chamber spill over was seen in higher percentage of patients than described in literature As the patients presented in milder forms visual acuity improved is of cases with the treatment protocol followed.