[Treatment of Behçet's disease yesterday and today]. / Therapie des Morbus Behçet gestern und heute.

Uveitis due to Behçet's disease belongs to those types of intraocular inflammation with a poor visual prognosis. Despite early treatment with corticosteroids and immunosuppressants, patients often become blind. With the so-called biologicals, highly effective drugs are now available which very rapidly lead to quiescence of intraocular inflammation and which successfully avoid further uveitis relapse. Recently, an interdisciplinary group of experts on behalf of the EULAR developed nine recommendations for the treatment of Behçet's disease. Two of these address ocular involvement. Compared with TNF alpha antagonists, interferon alpha offers the advantage that even after cessation of treatment a high percentage of patients remain in remission. This article provides an overview on current medical treatment of ocular Behçet's disease.

[Systemic voclosporin for uveitis treatment]. / Systemisches Voclosporin bei Uveitis.

To date cyclosporine is the only immunosuppressive drug approved for the treatment of uveitis in Germany. However, side effects often limit its use in daily practice. Voclosporin (Luveniq) represents a next generation calcineurin inhibitor which had shown efficacy in an animal model of experimental autoimmune uveitis as well as in clinical trials for the prevention of renal allograft rejection and for plaque psoriasis. The LUMINATE program included three prospective, randomized, double-masked, placebo-controlled multicenter trials which demonstrated the efficacy and tolerability of voclosporin 0.4 mg/kg b.i.d. in noninfectious intermediate and posterior uveitis and panuveitis. In Europe, the Marketing Approval Application (MAA) of voclosporin for the treatment of uveitis was submitted to the European Medicines Agency (EMA) in February 2010 and could be approved in March 2011.

[Secondary glaucoma in uveitis]. / Sekundärglaukom bei Uveitis.

Secondary glaucoma represents a frequent complication especially in chronic forms of uveitis. Different immunological and mechanical alterations can be responsible for the elevation of intraocular pressure (IOP). To enable a tailored treatment strategy the diagnostic process has to include the search for characteristic findings which can be indicative for the underlying uveitis entity. Sometimes it can be difficult to distinguish inflammatory glaucoma from primary non-inflammatory forms of glaucoma as well as from steroid-induced elevation of IOP. Treatment of secondary glaucoma due to uveitis has to include not only medicinal and surgical lowering of IOP but also control of the inflammation, e.g. by immunosuppressive or antiviral drugs. Under normal conditions, beta-receptor antagonists and carbonic anhydrase inhibitors will represent first-line topical treatment. Besides cyclodestructive and filtrating procedures, the use of drainage implants has gained importance in the surgical treatment of secondary glaucoma due to intraocular inflammation.

Efficacy and tolerability of interferon alpha treatment in patients with chronic cystoid macular oedema due to non-infectious uveitis.

AIM: To assess the efficacy and tolerability of interferon (IFN) alpha in chronic cystoid macular oedema (CMO) due to non-infectious uveitis. METHODS: Retrospective analysis of an interventional case series. IFN alpha-2a was administered at an initial dose of 3 or 6 million IU per day subcutaneously and tapered afterwards to the lowest possible dose to maintain the absence of CMO. Treatment efficacy was assessed by optical coherence tomography. RESULTS: Twenty-four patients with chronic CMO (median duration 36.0 months) due to non-infectious anterior (n = 2), intermediate (n = 18) or posterior (n = 4) uveitis have been analysed. Ineffective pretreatment included systemic corticosteroids (all patients), acetazolamide (22 patients) and at least one immunosuppressive drug (18 patients). IFN therapy was shown to be effective (= complete resolution of CMO within 3 months, able to taper IFN) in 15 patients (62.5%), partly effective (= incomplete resolution of CMO, unable to taper IFN) in six patients (25.0%) and not effective (= no response or recurrence of CMO) in three patients (12.5%). IFN treatment was generally well tolerated. Common side effects including flu-like symptoms, fatigue or increased liver enzymes were dose-dependent and led to discontinuation of IFN in only one patient. CONCLUSION: The data demonstrate IFN alpha to be an effective and well-tolerated therapy for chronic refractory uveitic CMO.

[Scleromalacia associated with varicella-zoster virus]. / Varizella-zoster-Virus-assoziierte Skleromalazie.

BACKGROUND: Scleromalacia usually appears following vasculitis in systemic rheumatoid diseases, especially as a late symptom of rheumatoid arthritis. CASE REPORT: A 67-year-old woman was referred to our hospital for further evaluation with the diagnosis of a "fast-growing tumor" of the left eye. Sixteen months ago she had suffered from herpes zoster ophthalmicus-associated keratouveitis and trabeculitis in the same eye. Scleromalacia associated with varicella-zoster virus (VZV) was diagnosed after the biomicroscopic and gonioscopic examination of the eye was completed and a systemic disease had been ruled out. One week after beginning systemic application of acyclovir (5 x 800 mg daily) and prednisolone (30 mg daily), the anterior chamber inflammation regressed and a fibrosis seemed to appear in the atrophic scleral area. CONCLUSION: Although scleral atrophy mostly appears as a late sign of systemic rheumatoid diseases, it might also develop secondary to infectious diseases. Scleromalacia associated with varicella-zoster virus has been previously described only in a few cases. Scleromalacia is a vision-threatening complication of zoster ophthalmicus which responds well to combination therapy with systemic antiviral and anti-inflammatory agents.

[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.

[Epidemic keratoconjunctivitis. Detecting adenoviruses]. / Keratoconjunctivitis epidemica. Nachweis von Adenoviren.

OBJECTIVE: The validity of the 15-min adenovirus assay SAS Adenotest was evaluated compared with virus detection by polymerase chain reaction (PCR) from conjunctival swabs. METHODS: In 75 patients with assumed epidemic keratoconjunctivitis, adenovirus detection from conjunctival swabs was performed by the immunochromatographic assay SAS Adenotest and PCR. RESULTS: In 25 patients adenovirus was detected by PCR, 18 of whom were detected by the SAS Adenotest and 7 of whom were not. No false positive results occurred. Sensitivity was 72% and specificity was 100%. CONCLUSIONS: The results indicate that rapid adenovirus detection with the SAS Adenotest is a useful tool in early epidemic keratoconjunctivitis. An additional PCR should be performed when clinical symptoms persist for 5 days or more.

MMF and eye disease.

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.

[Ocular involvement in Behçet's disease: first 5-year-results for visual development after treatment with interferon alfa-2a]. / Augenbeteiligung bei Morbus Behçet: Erste 5-Jahres-Ergebnisse zur Visusentwicklung nach Therapie mit Interferon alfa-2a.

BACKGROUND: Behçet's disease (BD) is a systemic vasculitis of unknown origin. Up to now ocular involvement has a poor visual prognosis irrespective of immunosuppressive treatment. Now recent open studies have shown that interferon alfa-2a (IFN alfa-2a) is very effective in the treatment of ocular BD. The purpose of this study was to evaluate the long-term development of visual acuity (VA) in patients with severe ocular BD who were treated with IFN alfa-2a. METHODS: We included 15 eyes of 9 patients with an active panuveitis and/or retinal vasculitis due to BD refractory to immunosuppressive treatment. VA before initiation of IFN-therapy was compared to VA at the end of the follow-up time. RESULTS: Mean follow-up time was 68.8+/-10.6 months. Mean duration of IFN-treatment was 40.6+/-17.0 months. With the exception of one patient IFN alfa-2a could be discontinued in complete remission of ocular symptoms. During the follow-up time 10 eyes showed an increase of VA of two lines or more. In 5 eyes VA remained stable. There was no decrease of VA in any eye. If a macular edema was present, a quick response to IFN alfa-2a was seen. No eye developed a pale optic disk during follow-up. CONCLUSION: Compared to conventional immunosuppressants, interferon alfa-2a seems to be much more effective to prevent a loss or decrease of VA over a long period of time in patients with severe ocular BD.

[Familial uveitis. Forms and incidence in patients at the University Eye Hospital Tubingen]. / Familiäre Uveitis. Formen und Häufigkeit im Patientengut der Universitäts-Augenklinik Tübingen.

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 Behçet'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.