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Scleritis associated with systemic vasculitic diseases.

PURPOSE: Scleritis may occur associated with systemic vasculitic diseases. The detection of systemic vasculitic diseases in patients with scleritis is a sign of poor general prognosis because it indicates potentially lethal systemic complications. This study was undertaken to analyze the ocular prognosis of patients with scleritis and the different systemic vasculitic diseases. METHODS: Patient characteristics, scleritis type, and ocular complications were evaluated in 82 patients with scleritis with systemic vasculitic diseases; comparisons were made between patients with scleritis with a specific systemic vasculitic diseases and patients with scleritis with the other systemic vasculitic diseases. RESULTS: Patients with scleritis with Wegener granulomatosis had more necrotizing scleritis (79%, P = 0.0001), decrease in vision (79%, P = 0.014), and peripheral ulcerative keratitis (50%, P = 0.0139) than patients with scleritis with the other systemic vasculitic diseases. Patients with scleritis with spondyloarthropathies had less decrease in vision (8%, P = 0.001) and peripheral ulcerative keratitis (0%, P = 0.0256) than patients with scleritis with the other systemic vasculitic diseases. Patients with scleritis and systemic lupus erythematosus had less necrotizing scleritis (0%, P = 0.0412) than patients with scleritis with the other systemic vasculitic diseases. CONCLUSIONS: Ocular prognosis of scleritis with systemic vasculitic diseases varies depending on the specific systemic vasculitic diseases: scleritis in spondyloarthropathies or in systemic lupus erythematosus is usually a benign and self-limiting condition, whereas scleritis in Wegener granulomatosis is a severe disease that can lead to permanent blindness; scleritis in rheumatoid arthritis or relapsing polychondritis is a disease of intermediate severity, which should be monitored closely for the development of ocular complications.

Adolescent

Scleritis associated with rheumatoid arthritis and with other systemic immune-mediated diseases.

PURPOSE: Rheumatoid arthritis is the most common systemic immune-mediated condition associated with scleritis. The purpose of this study is to determine whether or not scleritis is more severe in patients with rheumatoid arthritis than in those without associated disease ("idiopathic scleritis"), or than in patients with other systemic immune-mediated diseases. METHODS: Patient characteristics, type of scleritis, and ocular complications of 32 patients with scleritis associated with rheumatoid arthritis were compared with those of 74 patients with idiopathic scleritis and with those of 50 patients with scleritis associated with other systemic immune-mediated diseases. RESULTS: Patients with scleritis associated with rheumatoid arthritis were older (mean age, 60.78; P = 0.0011) and more often had necrotizing scleritis (34%; P = 0.0001), decrease in vision (59%; P = 0.0001), and peripheral ulcerative keratitis (31%; P = 0.0001) than patients with idiopathic scleritis; by contrast, there was no statistical association with sex, bilaterality, anterior uveitis, glaucoma, or cataract. Patients with scleritis associated with rheumatoid arthritis were older (P = 0.0261) and more often had bilateral scleritis (53%; P = 0.0221) than patients with scleritis associated with other systemic immune-mediated diseases; however, there was no statistical association with type of scleritis, sex, decrease in vision, anterior uveitis, peripheral ulcerative keratitis, glaucoma, or cataract. CONCLUSIONS: Scleritis associated with rheumatoid arthritis is more severe than idiopathic scleritis but is as severe as scleritis associated with other immune-mediated diseases.

Adolescent

Scleritis-associated uveitis.

PURPOSE: Anterior uveitis may accompany scleritis. This study was undertaken to analyze the incidence, characteristics, and meaning of uveitis in the course of scleritis. METHODS: Patient characteristics, scleritis type, ocular complications, and specific systemic diseases were evaluated in patients with scleritis-associated uveitis; comparisons were made between patients with scleritis-associated uveitis and patients with scleritis without uveitis. RESULTS: Seventy three (42%) of 172 patients with scleritis had anterior uveitis. Scleritis-associated uveitis ranged from mild to moderate intensity and always was related to the presence of active scleritis. Patients with scleritis-associated uveitis had more necrotizing scleritis (37%, P = 0.0001), decrease in vision (49%, P = 0.0046), peripheral ulcerative keratitis (22%, P = 0.0095), and glaucoma (19%, P = 0.0313) than did patients with scleritis without uveitis. Patients with scleritis-associated uveitis did not have any specific associated systemic disease more often than did patients with scleritis without uveitis. CONCLUSION: Extension of scleral inflammation to the anterior uveal tract is a consequence of a more severe disease with possible ocular complications that may cause progressive visual loss. The occurrence of anterior uveitis in the course of scleritis entails a poor ocular prognosis. The authors believe, therefore, that the anterior uveal tract should be evaluated at every follow-up visit of a patient with scleritis, so that emergence of this important prognostic condition (anterior uveitis) may be detected promptly and systemic therapy instituted appropriately.

Adolescent

Ultrasound biomicroscopy in scleritis.

OBJECTIVE: This study aimed to investigate the usefulness of ultrasound biomicroscopy (UBM) for detecting and following up scleritis and episcleritis. DESIGN: The study design was a case series. PARTICIPANTS: Patients with scleral inflammatory diseases (n = 16) were examined. INTERVENTION: Patient-reported problems and slit-lamp and UBM (50-MHz transducer) findings were compared retrospectively for signs of scleral inflammation. MAIN OUTCOME MEASURES: Thickness, reflectivity, and homogeneity of the sclera and episclera were the criteria for discriminating between the different types of scleritis with the UBM technique. RESULTS: Scleral disease was associated with Wegener disease (n = 3), Cogan disease (n = 1), Hashimoto thyroiditis (n = 1), myositis (n = 1), or panuveitis (n = 1). Initial slit-lamp evaluation showed episcleritis (n = 3), diffuse scleritis (n = 9), nodular scleritis (n = 3), or necrotizing scleritis (n = 1). By means of UBM analysis, the diagnosis of episcleritis or scleritis was in agreement with the slit-lamp findings in 2 of 3 and 6 of 13 cases, respectively. In contrast to the slit-lamp diagnosis, UBM studies excluded scleritis in one patient, disclosed necrosis in four patients with scleritis, and detected nodular scleritis patterns in two further patients with diffuse scleritis. The determination of complete remission, improvement, or progression of disease by slit-lamp and UBM evaluation was in agreement in 11 of the 14 patients examined. However, UBM was superior to slit-lamp examination with respect to detecting scleral necrosis, scleral thinning, or the nodular type of scleritis. CONCLUSIONS: The findings indicate that UBM is helpful in rapidly differentiating scleritis from severe episcleritis, detecting the diverse scleritis types with high accuracy, disclosing minimal disease progression, and judging treatment efficacy.

Adult

Severity of scleritis and episcleritis.

PURPOSE: Inflammation of the wall of the eyeball may extend to adjacent ocular tissues with blinding consequences and may be associated with potentially lethal systemic disorders. This study was undertaken to evaluate the ocular complications and systemic disease associations of the different types of scleritis and episcleritis. METHODS: Ocular complications and specific disease association were evaluated in 266 patients (358 eyes) with different types of scleritis (diffuse, nodular, necrotizing, scleromalacia perforans, and posterior) and episcleritis (simple and nodular). RESULTS: In patients with scleritis, decrease in vision occurred in 37%, anterior uveitis was present in 42%, peripheral ulcerative keratitis developed in 14%, glaucoma occurred in 13%, cataract formed in 17%, fundus abnormalities appeared in 6%, and specific disease association was uncovered in 57%. These findings were most commonly associated with necrotizing scleritis. In patients with episcleritis, decreased vision occurred in 2%, anterior uveitis was present in 11%, glaucoma developed in 4%, cataract formed in 2%, and specific disease association was uncovered in 32%. These findings were similar in simple and nodular episcleritis. CONCLUSIONS: In a patient with scleritis, examination of visual acuity, anterior uvea, cornea, lens, intraocular pressure, and fundus must be performed in every follow-up visit, and a meticulous approach for detection of a specific associated disease must be undertaken since the first visit. Scleritis is more severe than episcleritis, and necrotizing scleritis is the most severe type of scleritis. Classification of scleritis and episcleritis provides valuable prognostic information.

Adolescent

An analysis of therapeutic decision for scleritis.

PURPOSE: To compare the long-term efficacy of different systemic therapeutic regimens for patients with noninfectious anterior scleritis to establish guidelines for institution of therapy. METHODS: Therapeutic failure of systemic nonsteroidal anti-inflammatory drugs (NSAIDs), systemic steroidal anti-inflammatory drugs, and systemic nonsteroidal immunosuppressive drugs was evaluated in 132 patients with noninfectious anterior scleritis (diffuse, nodular, or necrotizing types). RESULTS: In patients with diffuse scleritis, therapeutic failure for initial regimens occurred in 7% of patients treated with NSAIDs, in 16% of patients treated with steroids, and in 27% of patients treated with immunosuppressive drugs. In patients with nodular scleritis, therapeutic failure for initial regimens occurred in 9% of patients treated with NSAIDs, in 28% of patients treated with steroids, and in 25% of patients treated with immunosuppressive drugs. Addition or substitution of steroids or immunosuppressive drugs as second- or third-line therapies helped control the scleritis. In patients with necrotizing scleritis, therapeutic failure for initial regimens occurred in 100% of patients treated with NSAIDs, in 91% of patients treated with steroids, and in 26% of patients treated with immunosuppressive drugs. CONCLUSIONS: In patients with diffuse and nodular scleritis, NSAIDs should be the initial choice; in case of therapeutic failure, steroids should be added or substituted as second-line therapy, tapering and discontinuing them as soon as possible while maintaining remission with continued NSAIDs; in case of therapeutic failure, immunosuppressive drugs should be added or substituted as third-line therapy. In patients with necrotizing scleritis, immunosuppressive drugs should be the initial choice.

Adolescent

Scleritis and episcleritis.

The data from 159 patients (217 eyes) with episscleritis and 207 patients (301 eyes) with scleritis have been investigated in detail and the results analysed with the help of a computer. Of these patients, 91 per cent were followed-up during a period of one to eight years. A new classification is presented which is as follows: Episcleritis (217 eyes) Simple episcleritis (170 eyes) Nodular episcleritis (47 eyes) Scleritis (301 eyes) Diffuse anterior scleritis (119 eyes) Nodular anterior scleritis (134 eyes) Necrotizing scleritis (42 eyes). Of these, 13 were regarded as scleromalacia perforans. Posterior scleritis (6 eyes) The diagnosis is based on an exact clinical examination which is fully described. Episcleritis has been shown to be a benign recurring condition, a mild keratitis being the only occasional complication. Episcleritis does not progress to scleritis, except in the case of herpes zoster which sometimes starts as an episcleritis with the vesicular stage of the eruption, to reappear three months later as a scleritis in the same site. No clear conclusions could be drawn as to the aetiology of episcleritis.

Adult

[Autoantibody pattern in scleritis and episcleritis].

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.

Adult

[New clinical and epidemiologic aspects of episcleritis and scleritis].

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.

Adult

Cells perpetuating the inflammatory response in scleritis.

Scleritis can be a destructive disease frequently associated with autoimmune disorders. It is believed that primary vasculitis plays an important role in its pathogenesis, but little is known about the cellular effector mechanisms. The purpose of this study was to analyse the inflammatory cellular infiltrate in scleritis. Six episcleral biopsies and two enucleated eyes were studied. The episcleral biopsies were taken from patients with nodular scleritis. In one patient enucleation was done after perforation in anterior necrotising scleritis and, in the other after misdiagnosis of posterior scleritis as intraocular tumour. Morphological criteria and immunohistochemical methods were used to characterise the inflammatory cellular infiltrate. The inflammatory cells infiltrating the episcleral tissue were mainly T lymphocytes and macrophages. There was a predominance of CD4 positive cells, but only few lymphocytes were activated (expressed IL-2 receptor). The cells infiltrating the scleral fibres in the enucleated eyes consisted in both cases predominantly of T cells. Clusters of B cells were found in perivascular areas. In circumscribed areas neutrophils, macrophages, and plasma cells were part of the scleral infiltrate. Signs of a granulomatous process with activated macrophages (epithelioid and giant cells) were present in necrotising scleritis. Expression of major histocompatibility class II molecules (MHC II) was found on lymphocytes and rarely on macrophages. Signs of primary vasculitis were not found in any of the specimens. The cellular infiltrate in scleritis shows, at least at certain stages, features compatible with a T cell mediated (autoimmune) disorder, which may have major therapeutic implications.

Adult

Infectious posterior scleritis caused by Pseudallescheria boydii. Clinicopathologic findings.

PURPOSE: The purpose of the study is to describe an unusual case of infectious posterior scleritis after excision of a pterygium. METHODS: The case history of a patient in whom anterior and posterior scleritis developed after excision of a pterygium is reviewed. Histopathologic findings are presented. RESULTS: Pathologic examination results of a scleral biopsy site and the eye showed extensive involvement of the posterior sclera with fungi that on culture grew Pseudallescheria boydii. The development of posterior scleral thickening and intrascleral abscesses gave the clinical impression of autoimmune posterior scleritis or possible tumor. Ultrasonography and computed tomography scan of the eye and orbit proved particularly helpful in following progression of scleral involvement. CONCLUSIONS: Pseudallescheria boydii is capable of causing an infectious posterior scleritis, which can mimic autoimmune posterior scleritis. The use of systemic immunosuppressive agents contributed to this unusual clinical presentation. Progression of posterior scleritis in the face of apparent adequate immunosuppressive therapy always should suggest the possibility of tumor or infection.

Aged

Scleritis and Streptococcus pneumoniae.

We retrospectively review our experience with four patients with Streptococcus pneumoniae scleritis. Two of the patients had been exposed to beta irradiation after pterygium removal 4 and 13 years previously. One patient had a 3-year history of chronic anterior nodular scleritis, and one patient had severe rheumatoid arthritis. All were treated with intensive i.v. and topical fortified antibiotics. In two of the cases, the infection was controlled and visual acuity returned to 20/30 and 20/60. In one patient, infectious scleritis progressed to endophthalmitis. This eye ultimately became phthisical and required enucleation because of chronic pain. In the remaining patient, infectious scleritis led to perforation, which required a corneal-scleral patch graft. This patient had a final visual acuity of counting fingers. An infectious etiology should be suspected in cases of necrotizing scleritis associated with a purulent discharge, and appropriate smears and cultures should be obtained. Infectious scleritis can be caused by streptococcal organisms. Appropriate topical and intravenous antibiotic treatment is effective in some cases.

Aged

Prediction of response to treatment in patients with scleritis using a standardised scoring system.

Scleritis is a severe chronic inflammation of the eye wall. High-dose corticosteroids and other immunosuppressive drugs are often required to control the inflammatory process. With the development of new and potentially more effective treatment modalities for scleritis has emerged the need for an accurate and reproducible system for quantifying the severity of scleritis and evaluating the response of individual patients to treatment. We have developed a quantitative scoring system, based on common clinical signs of scleritis, and evaluated it in 24 patients with scleritis. Our results indicate that this system is simple, rapid, reproducible and useful in grading the severity of scleritis and in predicting the response of patients to systemic immunosuppressive therapy.

Adolescent

[Inflammatory cellular infiltration in scleritis].

BACKGROUND: Scleritis can be a destructive disease frequently associated with autoimmune disorders. It is believed that primary vasculitis plays an important role in its pathogenesis, but little is known about the cellular effector mechanisms. The purpose of this study was to analyze the inflammatory cellular infiltrate in scleritis. PATIENTS AND METHODS: Two enucleated eyes were studied. In one patient, enucleation was done after perforation occurred in anterior necrotizing scleritis and, in the other, after the misdiagnosis of posterior scleritis as an intraocular tumor. Morphological criteria and immunohistochemical methods were used to characterize the inflammatory cellular infiltrate. RESULTS: The cells infiltrating the scleral fibers in the enucleated eyes consisted predominantly of T cells in both cases; many of the T cells were CD4-positive. Clusters of B cells were found in perivascular areas. In circumscribed areas neutrophils, macrophages, and plasma cells were part of the scleral infiltrate. Signs of a granulomatous process with activated macrophages (epitheloid and giant cells) were present in necrotizing scleritis. Expression of major histocompatibility class II molecules (MHC II) was found on lymphocytes and rarely on macrophages. Signs of primary vasculitis were not found in any of the specimens. CONCLUSION: The cellular infiltrate in scleritis shows, at least at certain stages, features compatible with a T-cell mediated (autoimmune) disorder, which has major therapeutic implications.

Adult

Computed tomography and magnetic resonance imaging in the diagnosis of posterior scleritis.

The clinical records of 14 patients with posterior scleritis were reviewed to compare the diagnostic potential of computed tomography (CT) and magnetic resonance imaging (MRI) in this condition. None of these patients had evidence of collagen vascular or other systemic diseases. Done 13 times on 11 patients, CT was diagnostic for posterior scleritis in six patients on seven occasions. We used MRI six times in five patients, and it was not diagnostic for posterior scleritis in any patient. Thus, CT appeared to be more sensitive than MRI in the diagnosis of posterior scleritis. The CT results appeared comparable to ultrasonographic findings (obtained eight times in six patients) in the diagnosis of posterior scleritis. Both CT and MRI, however, detected signs of orbital pseudotumor in six and three patients, respectively. These findings support the concept that posterior scleritis and orbital pseudotumor may represent a continuum of the same disease process.

Adolescent

Nodular scleritis: case report involving immunosuppressive therapy.

BACKGROUND: Scleritis is a chronic inflammation of the scleral coat of the eye. Although the clinical manifestations of scleritis can follow a benign clinical course, the more serious forms may lead to vision loss and even enucleation of the eye. METHODS: A case is presented of a 57-year-old woman with a diagnosis of nodular scleritis and attendant ocular manifestations. RESULTS: Scleritis affects females slightly more frequently than males, and is most commonly found in patients who are 40 to 60 years of age. Patients who are symptomatic in only one eye typically have bilateral involvement within 5 years. More importantly, 50% of patients with scleritis have an underlying systemic disorder, which makes a detailed medical history and complete physical examination a crucial part of the treatment regimen. CONCLUSION: Recognizing the disease and determining the possible underlying systemic etiologies as early as possible will elucidate the appropriate treatment options and help suppress the potentially devastating effects of scleritis.

Chronic Disease

Ultrastructural changes in scleral proteoglycans precede destruction of the collagen fibril matrix in necrotizing scleritis.

Proteoglycans were localized using cuprolinic blue and visualized by electron microscopy in sclera from the eye of a 58-year-old woman with necrotizing scleritis. Samples of sclera taken from clinically normal anterior and posterior sclera and from sites involved in anterior necrotizing scleritis with ulceration and posterior scleritis showed markedly different patterns of proteoglycan distribution. Proteoglycans appeared as stain-positive filaments associated regularly with the 'd/e' bands of the collagen fibrils in normal anterior sclera, but were absent from intact collagen fibrils in the zone of anterior ulcerative scleritis. In posterior sclera including a site of posterior scleritis, proteoglycans were reduced in number with more heterogeneous associations with collagen fibrils. Depletion of scleral proteoglycans therefore precedes the degradation of scleral collagen in necrotizing scleritis, which may also involve the synthesis of atypical proteoglycan types in pathological sclera.

Adult

Corneal sensation in scleritis and episcleritis.

A series of 59 patients were examined for loss of corneal sensitivity. Thirty-five of them suffered from scleritis and 24 suffered from episcleritis. There was a difference between the 2 conditions in that significantly more patients with scleritis were affected than patients with episcleritis. It was also found that among the scleritis patients there was a close relationship between the number of quadrants affected and decrease in sensitivity. Five patients had necrotising scleritis, and they all showed marked loss of sensitivity. Sensitivity returned to normal when the scleritis resolved except when a large area of scleral ectasia resulted. The test is of importance because it is one of the easiest methods of detecting the onset of necrotising scleritis in its earliest stages.

Cornea