Update of the evidence based, interdisciplinary guideline for anti-inflammatory treatment of uveitis associated with juvenile idiopathic arthritis.

BACKGROUND: Uveitis in juvenile idiopathic arthritis (JIAU) is frequently associated with the development of complications and visual loss. Topical corticosteroids are the first line therapy, and disease modifying anti-rheumatic drugs (DMARDs) are commonly used. However, treatment has not been standardized. METHODS: Interdisciplinary guideline were developed with representatives from the German Ophthalmological Society, Society for Paediatric Rheumatology, Professional Association of Ophthalmologists, German Society for Rheumatology, parents' group, moderated by the Association of the Scientific Medical Societies in Germany. A systematic literature analysis in MEDLINE was performed, evidence and recommendations were graded, an algorithm for anti-inflammatory treatment and final statements were discussed in a consensus meeting (Nominal Group Technique), a preliminary draft was fine-tuned and discussed thereafter by all participants (Delphi procedure). RESULTS: Consensus was reached on recommendations, including a standardized treatment strategy according to uveitis severity in the individual patient. Thus, methotrexate shall be introduced for uveitis not responding to low-dose (≤ 2 applications/day) topical corticosteroids, and a TNFalpha antibody (preferably adalimumab) used, if uveitis inactivity is not achieved. In very severe active uveitis with uveitis-related deterioration of vision, systemic corticosteroids should be considered for bridging until DMARDs take effect. If TNFalpha antibodies fail to take effect or lose effect, another biological should be selected (tocilizumab, abatacept or rituximab). De-escalation of DMARDs should be preceded by a period of  ≥ 2 years of uveitis inactivity. CONCLUSIONS: An interdisciplinary, evidence-based treatment guideline for JIAU is presented.

[Management of anticoagulants in ophthalmic surgery-a survey among ophthalmic surgeons in Germany]. / Umgang mit gerinnungshemmenden Substanzen in der Ophthalmochirurgie ­ eine Umfrage unter Ophthalmochirurgen in Deutschland.

INTRODUCTION: As our population ages and comorbidities rise, ophthalmic surgeons are increasingly faced with patients on anticoagulant therapy or with clotting disorders. The ophthalmic surgeon has to weigh the perioperative risk of haemorrhage when anticoagulation continues against the risk of thromboembolism caused by discontinuation or changing the patient's medication (bridging, switching, cessation). There are currently no guidelines or recommendations. METHODS: A survey was sent to the DOG (German Ophthalmologic Society) divisions and associated surgical organizations to determine the status quo. A questionnaire was sent out and filled out by the different groups of specialists. RESULTS: All four divisions of the DOG and four associated organizations returned completed questionnaires. Surgical interventions were listed that are carried out during anticoagulant therapy without exceptions, as well as interventions that were classified to require medical adjustment. Although the assessments varied, general consensus was achieved regarding interventions not requiring adjustments due to anticoagulants (i. e., intravitreal injection, cataract surgery, laser and corneal operations, simple muscle surgery), and those interventions requiring adjustments in medications (glaucoma operations, complex retina surgery, eye socket surgery, complex surgery of the lid). CONCLUSION: Main result of this survey was the specification of serious bleeding complications which are permanent vision loss and re-operation. They could serve as endpoint parameters for essential future investigations. Nevertheless, this survey makes clear that the decision about an adjustment of anticoagulant medication in ophthalmic surgery is currently made individually and not based on established standards.

[Differential diagnosis of anterior uveitis]. / Differenzialdiagnose der anterioren intraokularen Entzündung.

Anterior uveitis is a potentially sight-threatening disease, which affects either one or both eyes. Although the etiology is not fully understood, important associations with systemic diseases and infections have been established. A thorough clinical investigation with the slit lamp may reveal important findings leading to diagnosis and therapy. Additional diagnostic procedures are usually required only with the occurrence of relapses. Most anterior uveitis cases with an underlying autoimmune etiology respond very well to topical steroids and mydriasis while systemic treatment is rarely needed. In contrast, infectious causes typically require systemic medication. Anterior uveitis has a good prognosis if treated adequately despite some complications like cataract, macular edema, or increase of intraocular pressure, which cannot be prevented in all cases.

[Wide-field fundus autofluorescence in non-infectious posterior uveitis]. / Weitwinkelfundusautofluoreszenz bei nichtinfektiöser Uveitis posterior.

BACKGROUND: Posterior uveitis comprises a heterogeneous group of diseases with inflammatory alterations of the posterior fundus and is a common cause of visual impairment and blindness. The goal of this study was to evaluate the diagnostic value of wide-field fundus autofluorescence (FAF) in patients with non-infectious posterior uveitis and chorioretinal alterations. MATERIAL AND METHODS: In this study 73 eyes from 51 patients were included. Best-corrected visual acuity, wide-field color and FAF images achieved by a wide-field scanning laser opththalmoscope (SLO, Optomap P200Tx, Optos PLC, Dunfermline UK) and a full ophthalmological examination were obtained from each patient. A systematic analysis of chorioretinal alterations detected with FAF and color images was conducted followed by the evaluation of the diagnostic information of wide-field FAF compared to the clinical finding and wide-field color images. RESULTS: Of the 73 eyes included in the study 52 showed peripheral alterations. In 32 cases wide-field FAF images revealed a greater number and more extensive chorioretinal alterations than the corresponding wide-field color images of the posterior fundus. CONCLUSIONS: In this study wide-field FAF images showed more chorioretinal alterations than seen in funduscopy or in color SLO images. Therefore, wide-field FAF images offer important additional information for detection and documentation of peripheral and central chorioretinal alterations.

[Unilateral retinal pseudoarteritis after eyeball contusion]. / Unilaterale retinale pseudoarteritis nach contusio bulbi.

This article reports an unusual case of unilateral frosted branch angiitis. Three major groups of this disease are known: secondary frosted branch angiitis due to viral infection or autoimmune diseases, frosted branch-like angiitis related to malignant diseases and the rare entity of idiopathic frosted branch angiitis. A 58-year-old patient presented with strictly unilateral sheathing and partly occluded retinal arteries, vitritis and macular edema and 4 months prior to presentation he suffered a contusion of the eye with traumatic cataract and vitreous hemorrhage followed by cataract extraction and vitrectomy. The general medical history revealed that the contralateral eye and biochemical analyses were unremarkable despite slightly elevated antinuclear antibody (ANA) levels. Under high dose steroid therapy the patient showed slow improvement in all clinical findings. This case does not fit into any of the known groups and can therefore be defined as pseudoarteritis.

[Recurrent uveitis of unknown origin in childhood]. / Kindliche rezidivierende Uveitis unklarer Genese.

This report describes a 12-year-old girl with diffuse infiltrating retinoblastoma. This inflammatory condition belongs to the uveitis masquerade syndromes, which comprise a group of various ocular diseases such as chronic intraocular inflammation and ocular tumors.

[Acute retinal necrosis]. / Akute Retinanekrose.

Acute retinal necrosis syndrome (ARN) is a rare retinitis caused by the herpes virus family, including herpes simplex virus and varicella zoster virus. ARN most commonly occurs in otherwise healthy patients of either sex at any age. It is characterized by an initial onset of episcleritis or scleritis, periorbital pain, and a frequently granulomatous anterior uveitis. The key criterion is a necrotizing retinitis starting in the periphery and spreading towards the posterior pole, associated with vitreous opacification. Optic neuropathy may also occur. A total of 75% of untreated eyes develop retinal detachment within the first two months after onset of the disease. Two out of three ARN cases show involvement of the fellow eye. Early intravenous antiviral therapy is mandatory to stop ARN progression. Peripheral retinal breaks can be treated by laser photocoagulation, thereby reducing the risk of retinal detachment. Vitreoretinal surgery is often required, and silicon oil is the tamponade of choice in ARN, resulting in good reattachment rates (90%). Visual prognosis, however, is guarded.

[Ocular toxoplasmosis and toxocariasis in childhood]. / Okuläre Toxoplasmose und Toxocariasis im Kindesalter.

Toxoplasmosis and toxocariasis are parasitic infections that are transmitted by cats and dogs, respectively, to humans, and which may induce posterior uveitis already in childhood. Toxoplasmosis presents as a congenitally or postnatally contracted infection whereas toxocariasis is always an acquired disease. The typical ocular sign of toxoplasmosis is retinochoroiditis, occurring as an active lesion, in most instances, associated with an inactive pigmented scar. In contrast, toxocariasis leads to a choroidal granuloma secondarily involving the retina or an endophthalmitis-like picture. Although toxoplasmosis represents the most common cause of posterior uveitis, there are uncertainties regarding the timing and specificity of the diagnosis, namely in atypical cases and those at risk of permanent severe loss of function. Antiparasitic treatment should be tailored to the severity of the inflammation and the risk of visual function loss. Concomitant steroids may be used to control the sequelae of unspecific inflammation, but should be used with caution and must be combined with an antimicrobial regimen. Because it is a rare disorder, one may not be familiar with the clinical presentation and suggested therapy for ocular toxocariasis. With this survey we, therefore, wish to provide a current, practice-oriented overview on the infection, ocular manifestations, diagnosis and treatment of ocular toxoplasmosis and toxocariasis in childhood.

[Topical and systemic corticosteroid therapy for uveitis in childhood]. / Topische und systemische Kortikosteroidtherapie bei Uveitis im Kindesalter.

Systemic and topical corticosteroids constitute an important part in the treatment of children with uveitis, because of their rapid therapeutic onset. Patients with anterior uveitis receive eye drops initially every 30 minutes or every hour. Children will experience the same side effects as adults but, in addition, there will be a growth retardation. Therefore, if treatment is required for extended periods of time it is important to avoid steroid quantities above the Cushing level and to initiate an additional systemic immunosuppressive treatment regimen early.

[Susac syndrome: case reports and review of the literature]. / Susac-Syndrom: Fallberichte und Literaturübersicht.

BACKGROUND: Susac syndrome is a rare disease of unknown pathogenesis. It consists of retinal arterial occlusion, hearing loss and encephalopathy (triad). PATIENTS: The features of two cases of this syndrome are presented. CONCLUSION: A high percentage of patients do not have the clinical triad at the time of onset of symptoms and, therefore, this disease may be under-diagnosed. Any patient with unexplained encephalopathy should thus be examined by an ophthalmologist and have an audiogram performed.

[Bilateral lid swelling during infectious mononucleosis (Hoagland-sign)]. / Beidseitige Oberlidschwellung bei Mononucleosis Infectiosa (Hoagland-Zeichen).

BACKGROUND: Infectious mononucleosis ("kissing disease") is an infection caused by Epstein-Barr-Virus (EBV, human herpes virus 4). METHODS: We present the case of an 18-year old female patient with dacryoadenitis and sub-febrile temperatures followed by a distinct feeling of sickness. RESULTS: Laboratory results revealed a Leukocytosis of 16.3 G/I with explicit elevation of hepatic parameters. The ultrasound scan of the orbital region showed a bilateral, echo-reduced, compressible lesion in the area of the lachrymal gland. The abdomen-ultrasound scan revealed a splenomegaly and the size of the liver was within normal limits. Serology was positive for EBV-VCA-IgG, EBV-EA-IgG and EBV-VCA-IgM. CONCLUSIONS: A dacryoadenitis can be the presenting symptom of an infectious mononucleosis.

[Cystoid macular edema in uveitis]. / Zystoides Makulaödem bei Uveitis.

Cystoid macular edema (CME) is the most frequent cause of visual deterioration in uveitis patients. Intraocular inflammation disturbs the blood-retina barrier and leads to retinal edema. The basis of successful treatment is the anti-inflammatory and immunosuppressive therapy of uveitis. Restoration of the blood-retina barrier is mediated by corticosteroids and nonsteroidal anti-inflammatory agents. Resorption of extracellular fluid is improved by systemic carboanhydrase inhibitors. Despite aggressive therapy loss of visual acuity is frequent. Therefore, early diagnosis of CME and initiation of treatment, even if visual acuity is not yet impeded, is mandatory.

[Allergic conditions of the eye]. / Allergische Erkrankungen des Auges. Wann ist die Sehkraft in Gefahr?

In the doctor's office, seasonal and non-seasonal conjunctivitis must be differentiated from more serious conditions. These include vernal conjunctivitis which, when chronic, represents a risk for corneal complications. In atopic keratoconjunctivitis, too, which manifests in every fourth patient with atopic dermatitis, the patient's vision is in danger. Furthermore, wearers of contact lenses may develop characteristic conjunctival changes or a contact allergy triggered by lens cleansing fluid. When the diagnosis has been established, treatment with local or systemic antihistaminics, H1 blockers or mast cell stabilizers can be initiated. In contrast, the indication for glucocorticoids should be established only by an ophthalmogist.

[Practical advice for recovery and successful processing of anterior chamber puncture and vitrectomy samples]. / Praktische Hinweise zur Gewinnung und erfolgreichen Aufbereitung von Vorderkammerpunktat und Vitrektomiematerial.

For the diagnosis of unclear intraocular inflammation which threatens visual acuity and does not respond to therapy, the collection of intraocular fluid from the anterior chamber or the vitreous body is sometimes required. After surgery the correct and speedy processing of the rapidly degrading fluid determines the quality and reliability of the laboratory results. This article describes the different steps which are important after surgery to ensure optimal results for the different diagnostic laboratory procedures. If specimens are to be sent to cytology or microbiology laboratories, processing directly after surgery is necessary, while material which has to undergo PCR analysis, should remain untreated.