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Die multiple Sklerose (MS) ist eine chronisch entzundliche Erkrankung des Gehirns und Ruckenmarks, die uberwiegend im jungeren Erwachsenenalter auftritt und mit vorubergehenden oder dauerhaften neurologischen Ausfallerscheinungen einhergeht. Eine Assoziation zwischen Uveitis und MS ist seit Langem bekannt. Aktuelle Angaben legen eine ca. 10-mal hohere Uveitispravalenz bei Patienten mit MS gegenuber der allgemeinen Bevolkerung nahe. Immer deutlicher stellt sich dabei die dominierende Verbindung von MS zur intermediaren Uveitis mit einer typischen retinalen Begleitvaskulitis heraus. Die Behandlung der Uveitis sollte sich nicht nur am Schwergrad der intraokularen Entzundung orientieren, eine Abstimmung der Wirkstoffe muss auch die neurologische Manifestation in Betracht ziehen. Da sowohl die Uveitis als auch die MS pathogenetisch auf eine immunvermittelte Genese zuruckgefuhrt werden, dominieren immunmodulatorische Therapieansatze, wobei berucksichtigt werden muss, dass Tumor-Nekrose-Faktor(TNF)-α-Blocker eine MS verschlechtern konnen.

There are many interfaces between ophthalmologists and rheumatologists. On the one hand ophthalmologists face the question if an inflammation of the eye is caused by systemic inflammatory rheumatic diseases and on the other hand rheumatologists have to consider that ocular manifestations are relatively common in some inflammatory rheumatic diseases. Furthermore, these ocular manifestations may influence therapeutic decisions of the rheumatologist. This article summarizes which ocular inflammations can be associated with rheumatoid arthritis, connective tissue diseases and vasculitides. The description of acute anterior uveitis in spondyloarthritis and in juvenile idiopathic arthritis is omitted in this article but will be dealt with elsewhere in this issue.

Der Morbus (M.) Behcet (auch Behcet Erkrankung oder Adamantiades-Behcet-Syndrom) ist ein systemisches entzundliches Krankheitsbild unbekannter Ursache. In der aktuellen Chapel-Hill-Nomenklatur fur die Vaskulitiden wird der M. Behcet als Vaskulitis variabler Gefase klassifiziert, da Arterien und Venen aller Kaliber in unterschiedlichem Ausmas betroffen sind. Sowohl das angeborene als auch das adaptive Immunsystem sind bei der Pathogenese der Erkrankung beteiligt. Es liegen uberwiegend Merkmale einer autoinflammatorischen Erkrankung vor, weswegen aktuell diskutiert wird, dass es sich bei der Erkrankung um ein Mischbild zwischen Autoimmunitat und Autoinflammation handelt. Die Krankheit ist durch Exazerbationen und schubartigen Verlauf unterschiedlicher Symptome und Organmanifestationen gekennzeichnet. Sie kann durch geringe Haut- und Schleimhautlasionen in Erscheinung treten oder zu schwerwiegenden Augenbeteiligungen mit Erblindungsfolge fuhren. Zusatzliche Beteiligungen des Magen-Darm-Traktes, des Nervensystems und der grosen Gefase sind manchmal lebensbedrohlich. Die Behandlung des M. Behcet umfasst ein breites Spektrum von immunmodulatorischen Wirkstoffen mit zunehmender Bedeutung von Biologika. So konnte die IFN-α-Therapie fur die intraokulare Entzundung eine signifikante Wirksamkeit belegen. Monoklonale Antikorper gegen Tumornekrosefaktor (TNF)-α und Interleukin-1 wurden in klinischen Studien erfolgreich gepruft und erstere inzwischen in einigen Landern zugelassen. In dieser Ubersichtsdarstellung stellen wir den aktuellen Stand des Wissens zusammen und betonen insbesondere die Rolle des Augenarztes bei der Diagnostik und Behandlung des M. Behcet.

Die Behandlung von Autoimmunerkrankungen hat in den letzten Jahren erhebliche Weiterentwicklungen erfahren. Neue Wirkstoffklassen, v. a. Biologika und „small molecules“, haben bei vielen Erkrankungen zu bis dato nicht bekannten Behandlungserfolgen gefuhrt. Bisher profitierten davon Patienten mit rheumatologischen bzw. dermatologischen Autoimmunerkrankungen. Auch fur die Autoimmunuveitis liegen zahlreiche Belege sehr guter therapeutischer und praventiver Wirkung vor. Allerdings stehen die erforderlichen gesetzlichen Zulassungen fur die Therapie im Erwachsenenalter noch aus. Mit diesem Beitrag sollen aktuelle Fortschritte und kunftige therapeutische Moglichkeiten zur Behandlung der nichtinfektiosen Entzundung am hinteren Augensegment dargestellt werden.

Multiple sclerosis (MS) is a chronic inflammatory disorder of the brain and the spinal cord occurring mostly in young adults and is associated with temporary or permanent neurological deficits. An association between uveitis and MS has been recognized for a long time. Current data indicate an approximately 10 times higher prevalence of uveitis in patients with MS compared to the general population. In particular, MS is associated with intermediate uveitis and typically with concomitant retinal vasculitis. The treatment of uveitis should not only take the severity of intraocular inflammation into account but a coordination of the active agents must also consider the neurological manifestations. Since uveitis and MS are pathogenetically based on an immune-mediated genesis, immunomodulatory treatment approaches are dominant but it is important to bear in mind that tumor necrosis factor(TNF)-alpha blocking agents may worsen MS.

Hintergrund Beim primaren vitreoretinalen Lymphom (PVRL), einem seltenen hamatopoetischen okularen Tumor, handelt es sich meist um ein groszelliges diffuses B-Zell-Lymphom. Das PVRL, das fruher auch als primares intraokulares Lymphom (PIOL) bezeichnet wurde, ist eine Unterart des primaren ZNS-Lymphoms (PZNSL).

Hintergrund Lymphome der okularen Adnexe stellen ein heterogenes onkologisches Krankheitsbild dar.

There are many interfaces between ophthalmologists and rheumatologists. On the one hand ophthalmologists face the question if an inflammation of the eye is caused by systemic inflammatory rheumatic diseases and on the other hand rheumatologists have to consider that ocular manifestations are relatively common in some inflammatory rheumatic diseases. Furthermore, these ocular manifestations may influence therapeutic decisions of the rheumatologist. This article summarizes which ocular inflammations can be associated with rheumatoid arthritis, connective tissue diseases and vasculitides. The description of acute anterior uveitis in spondyloarthritis and in juvenile idiopathic arthritis is omitted in this article but will be dealt with elsewhere in this issue.

To evaluate the efficacy of mineralocorticoid-receptor antagonists in comparison to acetazolamide and observation in the treatment of central serous chorioretinopathy.Retrospective, interventional cohort study on 93 patients with acute or chronic central serous chorioretinopathy (37 patients: acetazolamide group, 20 patients: mineralocorticoid-receptor antagonist group, 8 patients: observation group, and 27 patients with a therapy switch between both medications). Main outcome measures were the change in best-corrected visual acuity, subretinal fluid (SRF) volume, central retinal prominence, and highest retinal prominence (HRP) at 12 weeks.HRP and SRF volume improved with statistical significance (P ≤ 0.05) after mineralocorticoid-receptor antagonist (P = 0.0000003 for the prominence, P = 0.008 for the volume) and acetazolamide (P 0.0000001 for the prominence, P = 0.0000007 for the volume) treatment. HRP and SRF volume also improved after observation, but without statistical significance (P = 0.08 for the prominence, P = 0.72 for the volume). Corresponding visual acuity improved significantly in acetazolamide (P = 0.002) and mineralocorticoid-receptor antagonist (P = 0.03) treated patients. Interestingly, HRP and SRF volume in acetazolamide nonresponsive patients improved after switch to mineralocorticoid-receptor antagonists, whereas no benefit was seen in patients switching vice versa.Both medical treatments are effective first-line treatment options for central serous chorioretinopathy. In patients who are nonresponsive to acetazolamide, therapy switch to mineralocorticoid-receptor antagonists could be beneficial.

Noninfectious posterior uveitis is a leading cause of visual impairment. Although conventional immunosuppressive agents have been successfully used, these are nonspecific and their long-term use may induce significant adverse effects. The purpose of this article is to identify recent advances and future therapeutic options in noninfectious posterior uveitis.A MEDLINE database search was conducted through May 2014 using the terms: uveitis, treatment, intravitreal and corticosteroid, biological. To provide ongoing and future perspectives in treatment options, also clinical trials as registered at ClinicalTrials.gov were included.For individuals who do not respond to conventional immunotherapy, two major lines of treatments can be identified as focus in recent years: i) the intraocular application of anti-inflammatory drugs and ii) the introduction of new agents, for example, biologicals and small-molecule inhibitors. Whereas intravitreal treatments have the beauty of avoiding systemic side effects, new agents are gaining increased importance because of their highly targeted molecular effects. Even when current treatment strategies are still hampered by the paucity of randomized controlled trials, promising progress and continuous efforts are undertaken to close this gap. Still, a critical evaluation of new agents has to be made because 'new' agents are almost exclusively based on experience in other autoimmune disorders.

Unter einer posterioren Uveitis werden fokale, multifokale und diffuse Lasionen einer Choroiditis, Chorioretinitis, Retinochoroiditis, Retinitis und Neuroretinitis subsumiert. Posteriore Uveitiden umfassen ein breites Spektrum von infektiosen und nichtinfektiosen autoimmunologischen Entzundungen. Die posteriore Uveitis manifestiert sich am haufigsten jedoch endogen. Im 1. Teil dieser Publikation wurden Epidemiologie, Atiologie, Symptomatik, diagnostisches Vorgehen und Grundkonzepte der Therapie dargestellt ( Heiligenhaus et al. 2014 ). In diesem 2. Teil werden die in unserer Bevolkerung typischen infektiosen und nichtinfektiosen Formen der posterioren Uveitis beschrieben.

Das Behcet-Syndrom (auch Morbus Behcet oder Admantiades-Behcet-Erkrankung) ist eine chronische Vaskulitis. Die Erkrankung ist durch Exazerbation und Remission einer Vielzahl von Symptomen und Organmanifestationen charakterisiert. Die Befunde variieren von milden mukokutanen Lasionen bis zu schweren, visusbedrohenden intraokularen Entzundungen. Es kann daruber hinaus Gelenke, alle Gefasbahnen und das zentrale Nervensystem betreffen. Aufgrund hoher Morbiditat und erheblicher Mortalitat ist eine adaquate Therapie von groser Bedeutung. Cyclosporin A ist das bisher in Deutschland einzig zugelassene Therapeutikum zur Behandlung der Augenbeteiligung. Neuro- und Nephrotoxizitat schranken den Einsatz allerdings ein. Bei Patienten mit schweren Uveitisverlaufen haben sich „Biologika“ als therapeutischer „Durchbruch“ erwiesen. Interferon-α hat sich als sehr wirksam bei intraokularer Entzundung gezeigt. Monoklonale Antikorper gegen TNF-α und Interleukin-1 sind ebenfalls in klinischen Studien effektiv gewesen und sind in einigen Landern fur diese Indikation bereits zugelassen. Dieser Beitrag fasst den aktuellen Wissenstand zusammen und unterstreicht die bedeutende Rolle des Augenarztes in der Therapie des Behcet-Syndroms.

Treatment of autoimmune diseases has undergone significant changes and developments in recent years. New classes of active substances, in particular biologics and small molecules have resulted in previously unknown success in the treatment of many diseases. In particular patients suffering from autoimmune rheumatic or dermatological diseases have benefited. For autoimmune uveitis there are numerous reports indicating excellent therapeutic and preventive effects; however, statutory approval for therapy in adults is still pending. This article outlines recent advances and future therapeutic options for the treatment of posterior segment noninfectious uveitis.

In 1951, the pathologists Churg and Strauss reviewed the records of 13 patients suffering of a periarteritis nodosa-like disease, which they called allergic granulomatosis and angiitis, which was later changed to Churg-Strauss syndrome. Actually, this disease is renamed to eosinophilic granulomatosis with polyangiitis (EGPA).

Das Behcet-Syndrom (auch Morbus Behcet oder Adamantiades-Behcet-Erkrankung) ist eine chronische Vaskulitis, die hauptsachlich durch rekurrierende mukokutane Lasionen und visusbedrohende intraokulare Entzundungen charakterisiert ist. Es kann daruber hinaus Gelenke, alle Gefasbahnen und das zentrale Nervensystem betreffen. Aufgrund hoher Morbiditat und erheblicher Mortalitat sind eine fruhe Diagnostik und adaquate Therapie von groser Bedeutung. Die Behandlung und Prognose der Erkrankung konnten in den letzten Jahren deutlich durch die Einfuhrung der „Biologika“ profitieren. Dieser Beitrag fasst den aktuellen Wissensstand zusammen und unterstreicht die bedeutende Rolle des Augenarztes bei der Diagnostik des Behcet-Syndroms.

Da die Entzundung eine wichtige Rolle bei der Induktion von choroidalen Neovaskularisationsmembranen (CNV) spielt, besteht bei einer Uveitis posterior das Risiko einer CNV-Bildung. Erkrankungen wie das Presumed ocular Histoplasmosis Syndrom (POHS), die multifokale Choroiditis (MFC) oder die Punctate inner Choroidopathy (PIC) tragen dabei ein sehr hohes CNV-Risiko. Meistens werden durch inflammatorische Prozesse klassische Membranen ausgelost. Dieses hat neben der haufig kleinen CNV-Grose und dem jungeren Alter der Patienten den Vorteil der besseren Behandelbarkeit. Analog der CNV-Membranen bei der AMD wurden inflammatorische Membranen vor der Anti-VEGF-Ara mittels Argonlaserkoagulation und spater dann mittels PDT behandelt. Deutlich bessere Visusergebnisse lassen sich heutzutage mit VEGF-Inhibitoren, gegebenenfalls in Verbindung mit einer PDT erreichen. Wenige Publikationen mit groseren Patientenzahlen aufgrund der Seltenheit des Erkrankungsbilds sowie zahlreiche Falldarstellungen konnen dieses belegen. Neben der symptomatischen Behandlung der CNV-Membran sollte bei diesen Erkrankungsbildern jedoch nie die Entzundungskontrolle vergessen werden, da sie den Trigger darstellt.

Die Atiologie und Pathogenese des Behcet-Syndroms (Morbus Behcet) sind bisher nicht geklart. Postuliert wird eine multifaktorielle Genese, die – ausgelost durch ein moglicherweise infektioses Agens – bei Vorliegen einer genetischen Disposition (HLA-B51) zu einer generalisierten Vaskulitis fuhrt. Eine uberschiesende Reaktion des angeborenen, spater auch erworbenen Immunsystems u. a. gegen „Heat-Shock-Proteine“ schliest sich vermutlich sekundar an. In diesem Beitrag wird eine kurze Zusammenfassung jungster Ergebnisse gegeben. Sie sollen v. a. im Hinblick auf neue therapeutische Moglichkeiten und klinisch relevante Perspektiven dargestellt werden.

Die okulare Symptomatik des Morbus Behcet (MB) beginnt bei 50–87% der Patienten in Form einer unilateralen sowie schubweise verlaufenden Uveitis anterior. Die ursprunglich von Adamantiades und Behcet als charakteristisch beschriebene Hypopyoniritis kommt zwar vor, ist jedoch heute – vermutlich aufgrund der fruhen Diagnose und Therapieinitiierung – nur noch selten zu finden. Im weiteren Verlauf folgt schlieslich bei 75% der Betroffenen der Ubergang in eine bilaterale Uveitis posterior bzw. in eine Panuveitis mit einem chronisch rezidivierenden Verlauf. Haufig finden sich Zeichen einer begleitenden retinalen Vaskulitis, die regelmasig okklusiv verlauft. Bei dieser konnen sowohl die retinalen Arterien als auch die Venen betroffen sein. Als Komplikation tritt haufig ein zystoides Makulaodem auf, das neben der retinalen Vaskulitis verantwortlich fur die meist limitierte Visusprognose ist.

Interferon alpha (IFN-α) and interferon beta (IFN-β) are naturally occurring cytokines, which belong to the type I interferons and share the same receptor leading to very similar therapeutic effects. The immunomodulatory effect of type I interferons includes modulation of antibody production, inhibition of lymphocyte proliferation, inhibition of delayed-type hypersensitivity and enhancement of T-cell and NK-cell cytotoxicity. An increasing number of open clinical studies and case reports have demonstrated the efficacy of IFN-α for severe ocular inflammation in patients with Behcet's disease and of interferon-β, which has been used mainly for the treatment of multiple sclerosis.

Unter einer Arthrogryposis multiplex congenita (AMC) versteht man ein heterogenes Krankheitsbild, unter welchem klinisch unterschiedliche Typen bekannt sind. Die „Arthrogryposis“ selbst stellt eine nichtprogressive Erkrankung dar, die durch kongenitale Kontrakturen verschiedener Gelenke in unterschiedlichen Korperregionen charakterisiert ist und als Manifestation bei einigen Syndromen auftritt. Innerhalb dieser Syndromenkomplexe wurde in der Literatur bisher eine ophthalmologische Beteiligung in Form einer Retinopathie bei lediglich 2 Patienten beschrieben. Ein 12 Jahre altes Madchen mit einer AMC stellte sich in unserer Ambulanz vor, da sie seit 1 Jahr eine Sehverschlechterung beidseits bemerkt hatte. Der ermittelte Visus lag bei R/L 0,5/0,8, das Gesichtsfeld zeigte eine konzentrische Einengung fur kleine Gesichtsfeldmarken. Am Fundus war ein ubiquitar atrophes Pigmentepithel auffallig. Bei den elektrophysiologischen Untersuchungen fanden wir stark reduzierte Antworten des skotopischen Elektroretinogramms (ERG) sowie verzogerte Zapfenantworten. Das multifokale Elektroretinogramm (mfERG) zeigte sich mit zentral wie auch peripher verringerten Amplituden ebenfalls pathologisch. Funf und 8 Jahre spater konnten wir die Patientin erneut untersuchen. Der Visus war auf R/L 0,05/0,1 abgefallen, ebenso hatten sich der Gesichtsfeldbefund und das ERG massiv verschlechtert; das mfERG war zwischenzeitlich nicht mehr messbar. Die Patientin wies auser der Arthrogryposis multiplex congenita und der Retinitis pigmentosa keine weiteren Erkrankungszeichen, insbesondere keine zerebrale Retardierung auf. Wir beschreiben hier einen ungewohnlichen Fall, bei welchem es sich moglicherweise um ein neues Arthrogryposissyndrom handelt.

Objectives: To develop evidence-based European League Against Rheumatism (EULAR) recommendations for the management of Behcet disease (BD) supplemented where necessary by expert opinion. Methods: The multidisciplinary expert committee, a task force of the EULAR Standing Committee for Clinical Affairs (ESCCA), consisted of nine rheumatologists (one who was also a clinical epidemiologist and one also a Rehabilitation Medicine doctor), three ophthalmologists, one internist, one dermatologist and one neurologist, representing six European countries plus Tunisia and Korea. A patient representative was also present. Problem areas and related keywords for systematic literature research were identified. Systematic literature research was performed using Medline and the Cochrane Library databases from 1966 through to December 2006. A total of 40 initial statements were generated based on the systematic literature research. These yielded the final recommendations devel- oped from two blind Delphi rounds of voting. Results: Nine recommendations were developed for the management of different aspects of BD. The strength of each recommendation was determined by the level of evidence and the experts' opinions. The level of agreement for each recommendation was determined using a visual analogue scale for the whole committee and for each individual aspect by the subgroups, who consider themselves experts in that field of BD. There was excellent concordance between the level of agreement of the whole group and the ''experts in the field''. Conclusion: Recommendations related to the eye, skin- mucosa disease and arthritis are mainly evidence based, but recommendations on vascular disease, neurological and gastrointestinal involvement are based largely on expert opinion and uncontrolled evidence from open trials and observational studies. The need for further properly designed controlled clinical trials is apparent.

Objectives: To present and analyse the literature sources regarding the management of Behcet disease (BD) identified during the systematic literature research, which formed the basis for the European League Against Rheumatism (EULAR) evidence-based recommendations for the management of BD. Methods: Problem areas and related keywords regarding the management of BD were determined by the multidisciplinary expert committee commissioned by EULAR for developing the recommendations. A systematic literature research was performed using MedLine and Cochrane Library resources through to December 2006. Meta-analyses, systematic reviews, randomised controlled trials (RCTs), open studies, observational studies, case control studies and case series’ involving ⩾5 patients were included. For each intervention the effect size and number needed to treat were calculated for efficacy. Odds ratios and numbers needed to harm were calculated for safety issues of different treatment modalities where possible. Results: The literature research yielded 137 articles that met the inclusion criteria; 20 of these were RCTs. There was good evidence supporting the use of azathioprine and ciclosporin A in eye involvement and interferon (IFN)α in mucocutaneous involvement. There were no RCTs with IFNα or tumour necrosis factor (TNF)α antagonists in eye involvement. Similarly controlled data for the management of vascular, gastrointestinal and neurological involvement is lacking. Conclusion: Properly designed, controlled studies (new and confirmatory) are still needed to guide us in managing BD.

Lymphomas of the ocular adnexa are heterogeneous and demonstrate a wide range of clinical, histological, immunohistochemical and molecular genetic characteristics.The aim of this article is to give an overview of the interdisciplinary diagnostics and individually adapted lymphoma subtype-based therapy.Depending on the lymphoma localisation, i.e. whether in the eyelid, the conjunctiva or in the orbit, a photograph or a radiological scan is required to record the tumor extent. Visual function is more likely to be impacted when the lymphoma arises in the posterior orbit, close to the optic nerve and imaging diagnostics are therefore necessary. Histological investigations are essential for confirming the lymphoma diagnosis and give information about the particular subtype, which in turn will determine subsequent patient management, Clinical staging investigations for determining the systemic extent of the lymphoma manifestation (e.g. imaging, blood analyses as well as bone marrow biopsy) are mandatory.External beam radiation, local and systemic chemotherapy or in some cases antibiotics are treatment options after surgical excision in isolated ocular adnexal lymphoma. The TNM classification of the American Joint Committee on Cancer or the Ann Arbor staging system, as well as the guidelines of the German Society of Hematology and Medical Oncology are all tools to aid the choice of the appropriate individually adapted therapy for systemic disease, which includes psycho-oncological care.

Purpose To describe the clinical finding of subretinal fluid (SRF) in the posterior pole by spectral domain optical coherence tomography (SD-OCT) in eyes with active ocular toxoplasmosis (OT). Design Retrospective case series. Participants Thirty-eight eyes from 39 patients with active OT. Methods Eyes with active OT which underwent SD-OCT were reviewed. SRFs in the posterior pole were further analyzed. Main Outcome Measures Presence of SRF; its accompanying features, e.g. retinal necrosis, cystoid macular edema (CME), choroidal neovascularization (CNV); and longitudinal changes of SRF, including maximum height and total volume before and after treatment. Results SRF presented in 45.5% (or 15/33) of eyes with typical active OT and in 51.3% (or 20/39) of eyes with active OT. The mean maximum height and total volume of SRF were 161.0 (range: 23–478) µm and 0.47 (range: 0.005–4.12) mm3, respectively. For 12 eyes with SRF related to active retinal necrosis, SRF was observed with complete absorption after conventional anti-toxoplasmosis treatment. The mean duration for observation of SRF clearance was 33.8 (range: 7–84) days. The mean rate of SRF clearance was 0.0128 (range: 0.0002–0.0665) mm3/day. Conclusions SRF (i.e., serous retinal detachment) is a common feature in patients with active OT when SD-OCT is performed. The majority of SRF was associated with retinal necrosis and reacted well to conventional therapy, regardless of total fluid volume. However, SRF accompanying with CME or CNV responded less favorably or remained refractory to conventional or combined intravitreal treatment, even when the SRF was small in size.

Immunosuppressive treatment has shown to be effective in various ocular inflammatory disorders. Factors limiting their use are the individual response and the rate of side effects. This report summarizes our knowledge about the use of mycophenolate mofetil (MMF) in the treatment of ocular cicatricial pemphigoid (OCP), uveitis, atopic keratoconjunctivitis (AKC), prevention of graft rejection after penetrating keratoplasty (PK) and scleritis. Controlled studies have been performed for prevention of graft rejection after PK, showing MMF as effective in the prevention of graft rejection as cyclosporine A. In experimental uveitis, MMF has been demonstrated to be highly effective in prevention of retinal destruction. A number of studies have now shown that MMF also seems effective in uveitis. There are also studies with smaller patient groups which point out the effectiveness of MMF in OCP, AKC, and scleritis. In most of the studies, the spectrum of side effects was small, compared to other immunosuppressives.

Beim Morbus Behcet (MB) handelt es sich um eine systemische Vaskulitis bislang unbekannter Genese. Trotz immunsuppressiver Therapie ist die Visusprognose auserst schlecht. Neuere Studien konnten nun zeigen, dass Interferon alfa-2a (IFN alfa-2a) bei okularem MB sehr effektiv wirkt. Zielsetzung dieser Studie war es, die langfristige Visusentwicklung bei Patienten mit schwerem okularem MB, welche mit IFN alfa-2a therapiert wurden, zu untersuchen. Wir untersuchten 15 Augen von 9 Patienten mit aktiver, unter Immunsupression therapierefraktarer Panuveitis und/oder retinaler Vaskulitis bei MB und verglichen die Sehscharfe vor Beginn der IFN-Therapie und am Ende des Beobachtungszeitraumes. Die Dauer der IFN-Behandlung betrug im Mittel 40,6±17,0 Monate. Mit Ausnahme eines Patienten konnte IFN alfa-2a in kompletter Remission der okularen Symptomatik abgesetzt werden. Wahrend des mittleren Beobachtungszeitraumes von 68,8±10,6 Monaten zeigten 10 Augen eine Visusverbesserung von mindestens 2 Zeilen, bei 5 Augen blieb die Sehscharfe stabil. Bei keinem Patienten kam es zu einer Verschlechterung des Visus. Beim Vorliegen eines Makulaodems kam es zu einer raschen Resorption unter IFN alfa-2a, kein Auge entwickelte eine Optikusatrophie. Verglichen mit herkommlichen Immunsuppressiva scheint Interferon alfa-2a erheblich besser einen langfristigen Visusverlust bei schwerem okularem Morbus Behcet verhindern zu konnen.

Objective. A familial accumulation in some forms of uveitis has rarely been described. The objective of this study was to identify such cases and to examine both clinical course and similarities in the HLA-pattern. Methods. From 1993 to 2000 all new uveitis patients, who were examined in the uveitis clinic of the University Eye Hospital Tuebingen, were asked about a positive family history. If possible, all affected family members underwent an ophthalmological examination and HLA-typing was performed. Results. In 7 families we found a familial accumulation of uveitis. The underlying etiologies were anterior uveitis in ankylosing spondylitis with HLA-B27 association,anterior uveitis in Blau syndrome with no HLA association, anterior and intermediate uveitis in sarcoidosis with no HLA association, and panuveitis in Behcet's disease with HLA-B51 association. For familial uveitis we calculated an incidence of 0.03 cases per 100,000 persons and year. Conclusion. Our data confirm that familial forms of uveitis are very rare. We suggest that these may be subgroups of known uveitis syndromes (e.g. sarcoidosis, ankylosing spondylitis). The factors causing the inheritance are still unknown. By genetic examination of families with uveitis it may be possible to identify single uveitis genes or possible antigens.

The distribution of the different HLA-B*51 suballeles among patients with Behcet’s disease (BD) of German (n=33) and Turkish (n=92) origin in comparison to their presence in the respective ethnically matched healthy control groups (German: n=325, Turkish: n=93) was studied. HLA-B*51x was significantly increased in both patient groups in comparison to the controls (Germans: 58% vs. 12%, OR 9.76, P

Behcet’s disease is a multisystem vasculitis with oral and genital aphthous ulcers, cutaneous vasculitis, uveitis, and arthritis as its main features. 1 Ocular manifestations, especially panuveitis, have a poor prognosis, resulting in blindness in most patients after five years, irrespective of treatment. Recent reports have shown significant improvement of visual prognosis by early and aggressive immunosuppressive treatment. 2 However, immunosuppressive treatment bears a risk of opportunistic infections, especially herpesvirus reactivation and increased incidence of malignancies. 3 Kaposi’s sarcoma is a rare malignant tumour (incidence 0.01%) of endothelia and vascular smooth muscle cells which has a markedly increased incidence in patients with AIDS (0.9%) 4 and iatrogenic immunosuppression (0.52%).

The aim of the study was to obtain normal values for the colour-arrangement test, Roth 28-hue desaturated. In 146 healthy non-smokers colour vision was tested monocularly. The subjects were divided into four age groups: 0–19,20–39,40–59, and 60–79 years. The overall error score for all groups was 54±24 (median ± mean absolute deviation). The values for the 20–39 year group were significantly lower than those for the other groups (Kruskal-Wallis: P

Background Multifocal electroretinography allows physiological mapping of the central retina. The purpose of this study was to describe the spatial distribution of ERG-activity in patients with impairment of macular function which usually do not exhibit a pathologic Ganzfeld-ERG. Subjects and methods 6 patients with macular lesions due to uveitis (4), retinitis centralis serosa (1), and contusio bulbi (1) were examined using the multifocal ERG technique. Results In normal volunteers the response density of the multifocal ERG decreased with eccentricity according to cone density distribution. In eyes with impaired central vision the foveal and macular responses were markedly diminished while surrounding signals were of normal or moderately decreased amplitudes. Conclusions With the multifocal ERG disturbances of macular function due to oedema and secondary structural changes were detected in the presented cases and the extension of the central lesions was estimated.

Background To evaluate the extent of morphological changes of the perilimbal vessels of the conjunctiva in patients with general vascular disorders. Materials and Methods With a questionaire systemic vessel diseases were investigated in 285 patients (m:f= 145:140; mean age = 54 ± 19 years) with pericorneal vessel alterations. Persons without any morphological alteration of the perilimbal vessels served as control group (n = 58; m:w = 44:14; mean age 26 ± 13 years). Results In the patient group we found the following pericorneal vessel alterations: vessel ectasia in 79%, vessel aneurysma in 41%, vessel interruptions in 37% and avascular regions in 12%. The distribution of the three most frequent systemic vascular processes in the patient/control group was: peripheral vascular spasm (cold hands and/or feet) in 57%/19%, migraine in 39%/14% and arterial hypertension in 33%/3%. Conclusion Alterations in perilimbal vessels seem to be correlated with systemic vascular disorders. In case of perilimbal vascular anomalies an internal medical examination should be considered.

Behcet's disease (also called Admantiades-Behcet syndrome) is a chronic vasculitis. The disease is characterized by exacerbations and remissions of symptoms and organ manifestations and may produce only mild mucocutaneous lesions, whereas ocular lesions can cause blindness. In addition, involvement of the gastrointestinal tract, central nervous system (CNS) and large blood vessels is sometimes life-threatening. Cyclosporin A is the only agent for treatment of ocular lesions registered in Germany; however, the neurotoxicity and nephrotoxicity restrict usage of the drug. In patients suffering from severe uveitis, biologics have been a breakthrough. Interferon (IFN) alpha therapy has shown significant efficacy for intraocular inflammation. Monoclonal antibodies to TNF-alpha and interleukin-1 have been successful in clinical trials and are approved in some countries. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the therapy of Behcet's disease.

Background Behcet`s disease (BD) is a variable vessel vasculitis. To date, there are only retrospective case series showing efficacy of interferon alpha for ocular BD. This trial was started in 2007. Objectives To prove that interferon alpha2a is at least as effective as cyclosporin A. Methods Maintenance dosage of IFN was 3 to 9 Mill iU 3x/week, CSA dosage 3 to 5 mg/kg, adapted to response every two weeks. In case of nonresponse, the patients were switched to the alternative treatment arm after week six. Efficacy was measured by Posterior Uveitis scoring system (PUS), Behcet`s disease current activity form (BDCAF), Health assessment questionnaire (HAQ) and Beck depression Inventory (BDI) were also used. Primary endpoints were time to remission by PUS and number of cross over from one arm to the other. Results The study was stopped in 2013 after 37 patients due to slow recruitment. 13 patients per treatment arm (n=26, 65% male, 35% female, 38% German, 50% Turkish, 12% other, mean age 30.5 years) were evaluated. All 13 patients with primarily IFN achieved remission of their ocular disease, 9 of 13 in the CSA arm (p=0.096). The median time to complete PUS remission was 84 vs. 72 days, p=0.899. The probability to achieve remission after one year was 0.92 for IFN (95% CI 0.49–0.99) and 0.62 for CSA (95%CI 0.24–0.81). There were significantly more switches from CSA to IFN (n=7) than vice versa (n=1) (p=0.030). The main reasons were inefficacy (n=4 for CSA) and/or side effects (n=3 for CSA). PUS of the affected eyes improved significantly in both arms during the first year (p 0.001), as did HAQ (p=0.047). Safety analysis revealed more hair loss (p=0.004) and cytopenia (p Conclusions Both treatments are effective for ocular and extraocular BD. There is a tendency towards superiority of interferon alpha considering that there were more switches from CSA to IFN due to inefficacy and side effects. Due to the small sample size, this did not reach significance. A prospective international multicenter trial may help to overcome these limitations. Acknowledgement This trial was supported by the German Ministry of Health (BMBF/DLR, No. 01KG0706) and by Roche (supply of interferon alpha2a) Disclosure of Interest None declared

Behçet's disease (also known as morbus Behcet or Admantiades-Behcet syndrome) is a chronic vasculitis mainly characterized by recurrent mucocutaneous lesions and sight threatening uveitis. It may also involve joints, vessels of all sizes and the central nervous system. Because of its severe morbidity and considerable mortality early diagnosis and treatment is important. Treatment and prognosis of this disorder have profited considerably in recent years following the introduction of biologic agents. This article summarizes the current state of knowledge and emphasizes the important role of the ophthalmologist in the diagnosis and therapy of Behçet's disease.

Inflammation plays a key role in the induction of choroidal neovascular membranes (CNV). This explains why each form of posterior uveitis may lead to CNV formation. Diseases like presumed ocular histoplasmosis syndrome (POHS), multifocal choroiditis (MFC) or punctate inner choroidopathy (PIC) carry a high risk of CNV creation. Inflammatory processes mostly cause classical membranes. Because of the classical membrane form, smaller membrane size and the younger age of the patients' inflammatory membranes are often better treatable than membranes in AMD patients. In the times before VEGF inhibition inflammatory membranes were treated with argon laser coagulation and later with PDT. Nowadays better visual acuity results are achievable through VEGF inhibitor injections with or without PDT. This is proven by a few publications with greater numbers of patients because of the rarity of the diseases and several case reports in the literature. In addition to CNV treatment the control of intraocular inflammation should never be forgotten because it forms the leading CNV trigger.

The etiology and pathogenesis of Behçet's syndrome remains unclear. It has been postulated that an infectious agent may induce an immune-mediated, generalized vasculitis in genetically predisposed individuals (HLA-B51 +). There is accumulating evidence that an abnormality of the innate and adaptive immune response, e.g. directed against heat shock proteins plays an important role in Behçet's syndrome. This article is intended to evaluate the more recent knowledge on this disorder and evaluate new therapeutic options.

The first ocular symptom in 50-87% of patients with Behçet's disease (BD) is unilateral anterior uveitis. As a characteristic sign of ocular BD the appearance of hypopyon iritis was originally described by Adamantiades and Behçet, but nowadays, probably due to an earlier and more aggressive treatment, this rarely occurs. In the further course of the disease up to 75% of BD patients develop a bilateral chronic relapsing form of posterior uveitis or panuveitis. In addition occlusive retinal vasculitis, which involves arteries as well as veins, is frequently present. A typical complication consists of a cystoid macular edema, which is, besides the retinal vasculitis, responsible for the limited prognosis of visual acuity.