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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↗

Mycophenolate mofetil for the treatment of scleritis.

PURPOSE: To evaluate the usefulness of mycophenolate mofetil (MMF) (CellCept, Roche, Nutley, NJ), an antimetabolite immunosuppressant with a selective antiproliferative effect on T and B lymphocytes, for the treatment of scleritis. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: Eight patients with scleritis treated with MMF in a tertiary referral center. METHODS: Review of the clinical records of patients evaluated at the National Eye Institute and prescribed MMF for the treatment of scleritis. MAIN OUTCOME MEASURES: Control of scleral inflammation, the ability to taper prednisone or other immunosuppressive medications, and adverse events were recorded for each patient. Mycophenolate mofetil was determined to be an effective steroid-sparing agent if the daily prednisone dosage could be reduced by 50% or more and was determined to be an effective adjunctive immunosuppressive agent if the scleral inflammation was controlled in patients with active scleritis. RESULTS: Four patients with diffuse anterior scleritis, two with necrotizing scleritis with inflammation, one with nodular anterior scleritis, and one with nodular anterior and posterior scleritis, were identified. Mycophenolate mofetil administration was initiated as a steroid-sparing agent in 4 patients with controlled scleritis and as an additional immunosuppressive agent in 4 patients with active scleritis receiving concomitant treatment with prednisone and cyclosporine or methotrexate. In 3 of the 4 patients started on MMF as a steroid-sparing agent, the scleritis remained controlled while the prednisone dosage was tapered by more than 50%. One of the patients started on MMF as a steroid-sparing agent had recurrent scleritis, and each of the patients with active scleritis continued to have persistent scleral inflammation requiring additional immunosuppressive therapy. Adverse effects recorded in 4 of the 8 patients included a rash, gastrointestinal symptoms, paresthesias, and laboratory evidence of hepatotoxicity and renal toxicity. CONCLUSIONS: Although MMF maybe be useful as a steroid-sparing agent, it was not effective as an adjunctive immunosuppressive agent in patients with active scleritis in our small, tertiary referral series. The adverse effects encountered with the use of MMF in this study cannot be attributed conclusively to MMF and are more likely complications of the multiagent systemic immunosuppressive therapy required for the treatment of recalcitrant scleritis.

Adult↗

Episcleritis and scleritis: clinical features and treatment results.

PURPOSE: To evaluate the clinical experience with episcleritis and scleritis at a tertiary care eye center. METHODS: Retrospective chart review. RESULTS: One hundred thirty-four patients with scleral inflammation were seen over a 12-year period. Thirty-seven patients had episcleritis, and 97 patients had scleritis. Ocular complications occurred in only 13.5% of patients with episcleritis but in 58.8% of patients with scleritis (P <.0001). No patient with episcleritis had a decrease in visual acuity, whereas 15.9% of patients with scleritis did. Only 16.7% of patients with episcleritis required more than topical corticosteroids for treatment, and these patients required oral nonsteroidal anti-inflammatory drugs. Conversely, 30.4% of patients with scleritis required nonsteroidal anti-inflammatory drugs, 31.9% oral prednisone, and 26.1% systemic immunosuppressive drugs (P <.0001). Necrotizing scleritis and posterior scleritis more often were associated with ocular complications, occurring in 91.7% and 85.7%, respectively, than were diffuse anterior scleritis and nodular anterior scleritis (P =.020). Patients with necrotizing scleritis and posterior scleritis were more likely to be treated with oral corticosteroids or immunosuppressive drugs (90% and 100%, respectively) than were patients with diffuse anterior scleritis and nodular anterior scleritis (56.4% and 21.4%, respectively, P =.002). CONCLUSIONS: Scleritis is a severe ocular inflammation, often associated with ocular complications, and nearly always treated with systemic medications. Nearly 60% of these patients will need oral corticosteroids or immunosuppressive drugs to control the disease.

Adolescent↗

[Indication and effect of immunosuppression in patients with scleritis].

PURPOSE: The value of immunosuppressive drugs for the therapy of scleritis patients is unclear. The authors investigated the indications and effects of immunosuppression in a group of patients with scleral inflammation. METHOD: Retrospective study of patients treated for scleritis (n = 87) or episcleritis (n = 18). The demographic factors, clinical symptoms, visual outcome, course of inflammation, ocular complications resulting from inflammation, topical and systemic antiinflammatory medication, and associated systemic diseases were analysed. RESULTS: Only one patient with episcleritis, but 37 with scleritis presented with ocular complications (P = 0.003). The vision was impaired in 15 patients with scleritis, but not in episcleritis patients (P = 0.022). In the group of patients with episcleritis, only those with frequent relapses required more than topical antiinflammatory drugs, especially systemic non-steroidals. In contrast, systemic therapy was indicated in all of the scleritis patients. Ocular complications were found more often in patients with necrotising (n = 7/10) or posterior scleritis (n = 10/11) than in those with diffuse (9/39) or nodular (11/27) scleritis. Compared with the other patients, associated systemic autoimmune diseases were more common in patients with necrotising scleritis (P = 0.03). The need for immunosuppression was associated with vision-threatening complications (glaucoma, uveitis, peripheral ulcerative keratitis) (P < 0.01), systemic autoimmune disease, and necrotising and posterior form of scleritis (P < 0.01). Quiescence of scleritis was obtained in 59 of the scleritis patients, and improvement of inflammation was achieved in further 21. In 26 patients, scleritis did not improve with systemic steroid or non-steroidal treatment, but with immunosuppression. CONCLUSIONS: Scleritis is often associated with life-threatening systemic diseases and vision-threatening ocular complications. In patients with severe scleritis, especially with the posterior and necrotising form, improvement can often be achieved with immunosuppression.

Adult↗

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↗

[Clinical features and response to systemic treatment of primary and secondary episcleritis and scleritis resistant to local treatment].

BACKGROUND: Scleritis and episcleritis may extend to adjacent ocular tissues with blinding consequences and may be associated with potentially lethal systemic disorders. AIM: To evaluate the ocular complications and systemic disease associations of the different types of scleritis and episcleritis. PATIENTS AND METHODS: Forty six patients with refractory scleritis and episcleritis were studied and treated during the period 1991 to 1998. RESULTS: Necrotizing type was the most common and severe category in the scleritis group of patients. A decrease in vision occurred in 58.3% of patients with scleritis v/s a 23.5% of patients with epiescleritis (p < 0.05). Uveitis was present in 35.4% of patients with scleritis and scleromalacia was present in 33.3% (p < 0.05). A specific disease association was uncovered in 51% of scleritis and in 38% of episcleritis patients. Rheumatoid arthritis, primary systemic vasculitic disease and Sjögren syndrome with vasculitis were the most common associated systemic diseases. Three patients with scleritis had tuberculosis. CONCLUSIONS: 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. A meticulous approach for the detection of a specific associated disease must be undertaken. Scleritis associated with vasculitis has a worse ocular prognosis than other non infectious diseases. Cyclophosphamide is the most effective immunosuppressive treatment to control severe ocular involvement.

Adolescent↗

Totally necrotic choroidal and ciliary body melanomas: associations with prognosis, episcleritis, and scleritis.

PURPOSE: To report associations between prognosis, total tumor necrosis, scleritis, and episcleritis in choroidal and ciliary body melanomas. METHODS: In this retrospective observational histopathologic study, 157 totally necrotic melanomas and 177 melanomas that were not totally necrotic of choroidal and ciliary body were retrieved from the Registry of Ophthalmic Pathology. The eyes were examined for the histologic evidence of inflammatory cells within the sclera and episcleral tissues. Contingency table, Kaplan-Meier, and Cox proportional hazard regression analyses were performed. In the survival analyses, only deaths with metastatic melanoma were considered as events in the statistical analysis. RESULTS: Among the totally necrotic melanomas, 118 of 157 (75.1%) had both episcleritis and scleritis; two of 157 (1.3%) had scleritis only; 29 of 157 (18.5%) had episcleritis only; and eight of 157 (5.1%) had neither episcleritis nor scleritis. Among the non-necrotic melanomas, 23 of 177 (12.9%) had both episcleritis and scleritis; eight of 177 (4.5%) had scleritis only; 71 of 177 (40.1%) had episcleritis only; and 75 of 177 (42.3%) had neither episcleritis nor scleritis. Cox regression indicated that total necrosis and scleritis were prognostically significant (P <.05) when analyzed univariately but not significant when analyzed multivariately with tumor size. CONCLUSIONS: Scleritis and episcleritis were statistically significantly associated with total tumor necrosis. There was a higher incidence of episcleritis than scleritis in both the necrotic and non-necrotic tumor types. Associations with patient outcome were identified for necrosis and scleritis. However, these associations were statistically insignificant when the prognostic effect of tumor size was also considered in a multivariate model.

Choroid Neoplasms↗

Ocular characteristics and disease associations in scleritis-associated peripheral keratopathy.

OBJECTIVES: To evaluate ocular characteristics and systemic disease associations in patients with scleritis-associated peripheral keratopathy and its different patterns, and to assess any ocular or systemic prognostic significance of the presence of the types of peripheral keratopathy in patients with scleritis. DESIGN: Review of 125 patients with scleritis alone and 47 patients with scleritis-associated peripheral keratopathy; review of patients with scleritis and the different patterns of peripheral keratopathy: peripheral corneal thinning, stromal keratitis, and peripheral ulcerative keratitis (PUK); review of ocular and systemic outcomes comparisons between patients with scleritis with and without peripheral keratopathy. RESULTS: Patients with peripheral keratopathy had more necrotizing scleritis (57%, P<.001), decrease in vision (81%, P<.001), anterior uveitis (62%, P<.002), impending corneal perforation (62%, P<.001), and potentially lethal specific-disease association (87%, P<.001) than did patients with scleritis alone. Patients with PUK had the worst ocular and systemic outcomes. Of the 24 patients with PUK, 16 (67%) had necrotizing scleritis (P =.02), virtually all had a potentially lethal systemic disease (P =.02), and all had impending corneal perforation (P<.001). CONCLUSION: The detection of peripheral keratopathy, and especially PUK, in a patient with scleritis indicates a poor ocular and systemic prognosis.

Adolescent↗

Posterior scleritis: clinical features, systemic associations, and outcome in a large series of patients.

OBJECTIVE: To document the clinical features, systemic associations, and visual outcome in a large number of patients with posterior scleritis. DESIGN: Retrospective, noncomparative case series. PARTICIPANTS: There were 137 patient records showing patients with a diagnosis of posterior scleritis who were attending or had attended the scleritis clinic at Moorfields Eye Hospital between 1974 and 1996. Ninety-nine records were suitable for detailed analysis. METHODS: The medical records and B-mode ultrasound examinations were reviewed. MAIN OUTCOME MEASURES: The clinical features, systemic associations, treatment, and outcome of each patient were determined. RESULTS: Posterior scleritis occurred at all ages. The mean age at onset was 49.3 years. Posterior scleritis began before age 40 in 30% of patients and was twice as common in women as in men. The B-mode ultrasound examination showed diffuse and nodular changes in the posterior sclera. Necrotizing posterior scleritis was not identified. Twenty-nine percent of patients had an associated systemic disease that included systemic vasculidites, autoimmune diseases, and lymphoma. Such patients more commonly had nodular changes on B-mode ultrasound examination. Early treatment controlled posterior scleral inflammation and limited visual loss. Thirty-one percent of patients lost two or more lines of vision. Statistical analysis revealed that patients older than age 50 had an increased risk of having an associated systemic disease and were more likely to experience visual loss. Patients with associated systemic disease required more aggressive immunosuppressive therapy and more frequently had accompanying anterior scleritis. There was no association between unilateral, bilateral, or recurrent disease and the presence of systemic disease or visual loss from posterior scleritis. CONCLUSIONS: The B-mode ultrasound examination reveals that posterior scleritis occurs far more often than previously thought and can lead to rapid and permanent visual loss. All patients with posterior scleritis must be assumed to be at risk of visual loss. Forty percent of patients had no anterior scleral inflammation, and 9% had no detectable physical signs. All patients need to be investigated for an associated systemic disease and all require early treatment to minimize loss of vision.

Adolescent↗

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↗

Scleritis associated with sarcoidosis.

PURPOSE: To review scleritis cases associated with sarcoidosis and to evaluate clinical features, medical treatment, and clinical course. METHODS: Two cases of diffuse anterior scleritis and a nodular scleritis case associated with systemic sarcoidosis were evaluated. Scleritis was the first manifestation of sarcoidosis in two of the patients and the diagnosis of sarcoidosis was confirmed with a conjunctival biopsy. The third patient had the diagnosis of systemic sarcoidosis when she developed scleritis. RESULTS: Based on the clinical and histological appearance, two patients presented with bilateral diffuse anterior scleritis and the third patient with an anterior nodular scleritis. One patient already had the diagnosis of systemic sarcoidosis at presentation, while ocular findings led to the diagnosis in the other two cases. Conjunctiva or skin biopsies revealed features compatible with the diagnosis of sarcoidosis. Scleritis attacks recurred in two patients. All patients responded well to systemic corticosteroid (prednisone) therapy. CONCLUSIONS: Scleritis associated with sarcoidosis has rarely been described and it may be the presenting sign of systemic sarcoidosis.

Aged↗

Orbital myositis in scleritis.

AIMS: To investigate the association between scleritis and myositis. METHODS: Retrospective, non-comparative case series. Records and ultrasonograms were examined of 132 patients, with a diagnosis of episcleritis or scleritis, who attended the ophthalmology department at Leiden University Medical Center between 1997 and 2000. 103 were eligible for comprehensive examination. Medical records were evaluated. Ultrasonography was performed in all patients diagnosed with episcleritis or scleritis. Clinical features, precipitating factors, systemic associations, ocular complications, treatment, and outcome of each patient were assessed. RESULTS: Of the 103 patients, 27 (26.2%) had episcleritis and 76 (73.8%) had scleritis. Myositis was found to be present in 11 patients. It was present in 14.5% of all patients with scleritis and 30.5% of those in whom the posterior sclera was affected. The presence of the associated myositis did not worsen the visual prognosis and the presence of myositis was not associated with other systemic diseases. There were no cases of unilateral scleritis with bilateral orbital myositis. During an attack ocular complications were more common in patients with scleritis and myositis (64%) than in patients with scleritis alone (30.4%), indicating a more diffuse and potentially dangerous inflammation. There was no evidence that the inflammatory changes in the orbit had spread to involve the sclera, so it is assumed that the muscle changes are an extension of a generalised response to intense inflammation of the episclera and sclera. CONCLUSION: This study found a frequent association between myositis and scleritis. Prognosis for vision was not affected by coexistence of myositis.

Adult↗

[Characteristics of scleritis in patients older than 60 years].

PURPOSE: This study aimed to describe the clinical characteristics of scleritis presented de novo in patients older than 60 years of age. DESIGN: Type of scleritis, ocular manifestations, previous ocular surgery, and disease association were evaluated in patients older than 60 years with scleritis. Comparisons were made between patients with scleritis older and younger than 60 years. RESULTS: Patients with scleritis older than 60 years (61 of 195 patients, 31.3%) had more necrotizing scleritis (47.5%, p=0.0001), decrease in vision (57.4%, p=0.0001), peripheral ulcerative keratitis (29.5%, p=0.0001), previous ocular surgery (39.3%, p=0.0001), and disease association (73.8%, p=0.0001) than did patients with scleritis younger than 60 years. Rheumatoid arthritis (p=0.03) and Wegener granulomatosis (p=0.01) were the most common associated diseases. CONCLUSIONS: The detection of scleritis presented for the first time in the elderly, indicates a poor ocular prognosis because it is often associated with necrotizing scleritis, decrease in vision, and peripheral ulcerative keratitis. It has also an ominous systemic prognosis because it is often associated with potentially lethal systemic diseases.

Age Factors↗

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↗