Immunology of ocular tumours.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to M Zierhut.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
BACKGROUND: The Churg-Strauss syndrome, also known as allergic granulomatosis with angiitis, is a rare necrotizing vasculitis with unknown pathogenesis. The necrotizing granulomatous vasculitis involves small and medium-sized arteries, capillaries and veins, leading to the characteristic changes of intra- and extravascular, eosiniphilic granulomas, accompanied by clinically symptoms: bronchial asthma, hypereosinophilia and fever. Ocular manifestations are rarely reported. CASE REPORT: A 53-year-old woman suffered from bronchial asthma, relapsing lung infiltrates and sinusites since 1994. In August 1997 she suddenly disclosed vasculitic skin manifestations on both legs and a mononeuritis of the left peroneus nerve. At the same time visual acuity of the right eye decreased, before she had shown some attacks of amaurosis fugax. The funduscopy showed a central retinal artery occlusion. LABORATORY FINDINGS: blood eosinophilia of 20%, elevated IgE value to 396 kU/l (normal value < 120 kU/l), and negative parameters for antineutrophil cytoplasmatic antibodies (p- and c-ANCA). CONCLUSION: The clinical and laboratory findings are characteristic signs for the Churg-Strauss syndrome. Without such typical manifestations the histologic examination leads to the diagnosis and helps to differentiate this disease from other necrotizing vasculitides, e.g. panarteriitis nodosa or Wegener's granulomatosis.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
PATIENT: A 60-year-old female patient presented with recurrent anterior, necrotizing scleritis with inflammation and a newly developed secondary glaucoma in the right eye. Anterior uveitis occurred some years before. Severe scleral thinning was circumferentially present and focal scleral ectasia was found. Physical examination revealed no systemic association of scleritis. Immunosuppressive therapy with metotrexate was initiated and control of scleritis achieved. Intraocular pressure elevation persisted and was refractory to glaucoma medication. Diurnal pressure curve showed IOP-values of 40 mm Hg despite the use of systemic carbonic anhydrase inhibitors. Visual acuity was 20/50 in the right and 20/25 in the left eye. METHOD: Diode laser cyclophotocoagulation (Oculight SLx 810 nm, Iris Medical Instruments Inc. California, USA) was performed under general anaesthesia using reduced parameters for application (12 laser spots, 1 second, 1.25 W). No complications occurred during and after laser application. Postoperatively, intraocular pressure was within normal range between 14 and 18 mm Hg. No reactivation of scleritis or uveitis was seen. CONCLUSION: In our experience, diode laser cyclophotocoagulation is effective and safe in treating secondary glaucoma associated with anterior, necrotizing scleritis with inflammation and uveitis using reduced parameters for application.
Explore the source record for details and available documents.
AIM: To study long term effects of interferon alpha 2a (IFN alpha 2a) on panuveitis in seven patients with Behçet's disease in a prospective, open clinical trial. METHODS: Seven patients were treated with IFN alpha 2a for a mean of 23.6 months (14-37 months). They received an initial dose of IFN alpha 2a of 6 x 10(6) IU/day, followed by 3 x 10(6) IU/day after 1 month and 3 x 10(6) IU every other day after 3 months. Two patients received low dose prednisolone (between 0.2 and 0.4 mg/kg/body weight) additionally at the beginning of the therapy. Complete cessation of IFN alpha 2a was possible in three patients (observation period 22, 6, and 4 months). RESULTS: Marked improvement occurred in six patients who had ocular manifestations of Behçet's disease for the first time or with minor damage during their course of chronic relapsing panuveitis. In one patient with advanced ocular Behçet's disease, new relapses were prevented. Retinal infiltrates resolved within 2 weeks; vasculitis, macular oedema, infiltration of the anterior chamber and vitreous resolved within 4 weeks. Mean posterior uveitis score before treatment (nine affected eyes) was 6.6, 4 weeks after IFN it was reduced to 0.4. The mean observation period is 27.6 months, ranging from 14 to 42 months. CONCLUSION: Treatment of ocular symptoms of Behçet's disease with IFN alpha 2a alone or in combination with low dose steroids led to complete remission of ocular vasculitis in all patients treated in this open, uncontrolled trial. Treatment with IFN alpha 2a may prevent permanent retinal or optic nerve damage due to vascular occlusion. No severe side effects occurred. Controlled randomised studies are warranted in order to prove the efficacy of IFN alpha 2a in ocular Behçet's disease and to compare it with other, established treatments such as azathioprine or cyclosporin A.
BACKGROUND: Episcleritis and scleritis can be caused by various systemic disorders, which can be triggered by infectious diseases. We studied the autoantibody pattern against various organ-specific and non-organ-specific antigens in episcleritis and scleritis patients. MATERIAL AND METHODS: Sera from 46 patients (episcleritis n = 28, scleritis n = 18) were studied for antibodies against nuclei, smooth muscle cells, mitochondria, endothelial cells, sarcolemma, liver cells, heart muscle fibrils, parietal cells and thyroid cells by immunofluorescence testing. Titers of antibodies against thyroglobulin, laminin, keratin and microsomes were evaluated by ELISA. RESULTS: In patients with episcleritis the pattern of autoantibodies found was different from that in scleritis patients. Thus, in episcleritis the levels of antibodies against sarcolemma (32%), parietal cells (25%), laminin (38%), keratin (58%) and microsomes (28%) were elevated, while scleritis patients, besides keratin antibodies (50%), demonstrated anti-nuclear antibodies (ANA) in 28% of cases. These differences were not significant. Approximately 5% of normal control patients show these antibodies. CONCLUSIONS: Previous studies have shown that episcleritis rarely develops into scleritis. Our results suggest that this may be due to different underlying diseases. While 28% of scleritis patients had ANA, which may suggest an autoimmune disposition related to collagenosis, episcleritis patients had a different autoantibody pattern such as has been found in various infectious diseases and diseases for which triggering by infectious organisms seems possible, such as anterior uveitis, ankylosing spondylitis and Behçet disease. Investigations in larger groups of patients are needed to check the statistical significance of these differences.
UNLABELLED: Numerous systemic diseases can cause scleritis or episcleritis. Frequently, symptoms and complications compromising vision can only be managed with systemic immunosuppressants. There are no clear guidelines on the indications for systemic immunosuppressants in patients with episcleritis and scleritis. PATIENTS AND METHODS: The aim of the present retrospective study was to investigate how many patients with episcleritis or scleritis have an associated systemic disease and at what stage it is diagnosed. Secondly, the proportion of patients who present with episcleritis or scleritis in the first instance and then change into the other category during the course of the disease was analyzed. Finally, we checked whether the presence of an associated systemic disease indicates the necessity to treat the patient with nonsteroidal systemic immunosuppressive drugs. RESULTS: Sixty-eight patients with inflammatory scleral diseases were treated at the University Eye Clinic between 1991 and 1995. In 13 patients an associated systemic disease was diagnosed before the appearance of ocular symptoms, and in 8 patients such an illness was diagnosed at a later stage. In 2 cases (3%) the ocular disease category changed during the course of the disease. Neither in the episcleritis nor in the scleritis population was a statistically significant correlation established between the diagnosis of an associated systemic disease and the necessity to treat the patient with nonsteroidal systemic immunosuppressive drugs. CONCLUSION: The small number of patients who changed the ocular disease category may indicate that episcleritis and scleritis are two independent entities, which might even be caused by different mechanisms. The indications for the management of episcleritis and scleritis with immunosuppressive drugs should not only depend on the diagnosis of an associated systemic disease, but also and mainly on the severity of the ocular manifestation.
The physiological mechanisms for the protection of the eye are only partly known. It becomes increasingly evident that the eye is supervised by and included in the immune system of the body. The physiological protection system of the eye consists of a mechanical and an immunological component. The lacrimal gland plays a key role for the immune system of the anterior segment. It is part of the MALT system, and the characterisation of the special ocular components of this system-including possible homing receptors-will give important informations. Research of the ocular immune system has disclosed various special features, like the anterior chamber associated immune deviation (ACAID). The distribution of antigen presenting cells and its localization, but also the distribution of T- and B-lymphocytes is an important factor for limiting ocular inflammation. The phenomenon of 'apoptosis' seems to have an important role in maintenance of the ACAID. The cornea is nearly free of cells, and infiltrating antigen presenting cells may prevent ACAID. Research regarding mechanisms which are used to tuned these various cells will give answers to the questions how the cornea contains its optical transparency, how corneal transplant rejection works, how the eye participates in systemic disorders and may also define the role of a possible dysfunction of antigen presenting cells of the retinal pigment epithelium in senile maculopathy.
The clinical differentiation of the various subtypes of scleral and episcleral inflammation is crucial for its early and adequate management. Particularly important is the recognition of necrotizing scleritis, since it is frequently associated with ocular complications, going along with a bad ocular prognosis. In patients who suffer from associated vasculitic systemic disease, the appearance of scleritis indicates a generalization of the vasculitis, which can result in potentially lethal systemic complications. Only the early diagnosis and an adequate aggressive therapy can preserve ocular functions and the patients life. Conventional steroid therapy generally fails to control the inflammatory activity in necrotizing scleritis. However, application of nonsteroidal immunosuppressive drugs has been shown to control the vasculitic conditions in the majority of cases, to improve ocular prognosis and to reduce mortality.
Explore the source record for details and available documents.
Four children, 4 to 10 years old, with chronic uveitis were treated with cyclosporin A (CsA; starting dosage 2 mg/kg per day) and prednisolone. All children had previously received systemic steroids and/or cytotoxic agents, which resulted either in a poor therapeutic effect or in intolerable side effects. In one child, suffering from uveitis in combination with juvenile rheumatoid arthritis, a complete reduction of intraocular inflammation was achieved. In a second child treatment resulted in a marked reduction of inflammatory activity with stabilization of visual function, although a temporary increase in the steroid dosage was necessary at one stage. In the remaining two cases therapy failed to improve the intraocular inflammation. During the time of treatment, ranging from 11 to 26 months, no serious side effects were observed. All patients developed moderate hypertrichosis and one child gingival hyperplasia. In our experience, cyclosporin A (CsA) combined with low-dose systemic steroids is useful as a therapeutic alternative to steroids alone and to other immunosuppressive agents in severe cases of chronic uveitis in childhood.
Explore the source record for details and available documents.
Bullous dermatoses such as erythema exsudativum multiform major (EEMM) and bullous pemphigoid can lead to severe ocular involvement. In rare cases, both diseases develop as paraneoplastic syndromes. The immunopathologic mechanisms are discussed. A 69-year-old woman with non-Hodgkin's lymphoma (NHL) of grade IIIb developed EEMM while under systemic treatment with Fluconazole, Ofloxacin, and/or a combination of sulfamethoxazole and trimethoprim after polychemotherapy. In the eye, conjunctival necrosis with sicca syndrome led to Staphylococcus aureus-induced corneal superinfection, perforation, and consecutive keratoplasty à chaud. The patient died 6 weeks after the first presentation. A 44-year-old man with NHL of grade IVa after polychemotherapy developed a bullous pemphigoid affecting the skin, mucous membranes, and both eyes while under systemic treatment with sulfamethoxazole and trimethoprim. Although the underlying malignancy responded well to chemotherapy, the ocular manifestations of the paraneoplastic systemic syndrome slowed down only on treatment with cyclosporin A but not following therapy with azathioprine and cyclophosphamide. Therapy could not stop cicatrization and keratinization of the conjunctiva and cornea. An occult malignancy should be excluded in acute and chronic oculomucocutaneous syndromes. The prognosis for the eye seems to be poor because of the rapid course and the ineffectiveness of therapy as demonstrated in the present cases.