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PubMed1997

[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↗

PubMed2004

Evaluation of patients with scleritis for systemic disease.

OBJECTIVE: To evaluate the relationship between associated medical conditions and scleritis-particularly, the timing of the diagnosis of these diseases. DESIGN: Retrospective case series. PARTICIPANTS: Patients with scleritis presenting to a single center over an 18-year period. METHODS: Medical records were reviewed for the presence of an associated infectious or rheumatic disease and for the timing of the diagnosis of the systemic disease relative to the presentation for evaluation of the scleritis. MAIN OUTCOME MEASURES: Presence of an associated medical condition and timing of diagnosis relative to that of scleritis. RESULTS: In a series of 243 patients with scleritis, 44.0% had an associated medical condition: 7.0%, an infection, and 37.0%, a rheumatic disease. The most frequent infection was herpes zoster, and the most frequent rheumatic disease was rheumatoid arthritis, present in 4.5% and 15.2% of patients, respectively. Of the 107 patients with an underlying disease, 77.6% had a previously diagnosed disease, 14.0% had their conditions diagnosed as a result of the initial evaluation, and 8.4% developed a systemic disease during follow-up. Systemic vasculitis was less likely to have been previously diagnosed than other rheumatic diseases (59.1% vs. 83.8%, P = 0.015) and more likely to be diagnosed by the initial diagnostic evaluation (27.3% vs. 8.8%, P = 0.027). Ten patients (4.1%) had a positive antineutrophil cytoplasmic antibody (ANCA) test result without clinical evidence of a systemic vasculitis. Four of 5 patients with a positive cytoplasmic ANCA test result but no clinical evidence of systemic vasculitis required immunosuppressive drugs for control of the scleritis, whereas 1 of the 5 patients with a positive perinuclear ANCA test result required immunosuppressive drugs. Among patients with no evident systemic disease after the initial diagnostic evaluation, the rate of occurrence of a rheumatic disease was 4% per person-year. CONCLUSIONS: Although associated systemic diseases are frequent among patients with scleritis, the majority are previously diagnosed. Systemic vasculitis is less likely than other rheumatic diseases to have been previously diagnosed. Because vasculitis is a potentially life-threatening disorder, it should be a focus of the diagnostic evaluation.

Adolescent↗

PubMed2003

Scleritis and other ocular side effects associated with pamidronate disodium.

PURPOSE: To evaluate reported cases of scleritis and other ocular side effects associated with pamidronate disodium, with emphasis on previously unreported cases of scleritis. DESIGN: Observational case series. METHODS: Case reports from the National Registry of Drug-Induced Ocular Side Effects (Casey Eye Institute), the Food and Drug Administration, the World Health Organization, and the literature were reviewed to determine possible adverse ocular side effects associated with pamidronate disodium. The World Health Organization's Causality Assessment Guide was used to categorize an adverse drug reaction. RESULTS: Seventeen cases of unilateral scleritis and one case of bilateral scleritis occurred, usually within 6 hours to 2 days after intravenous pamidronate disodium. Six patients had positive rechallenge testing with the scleritis occurring after a repeat drug exposure. Other ocular side effects with positive rechallenge data, associated with pamidronate disodium, include blurred vision, nonspecific conjunctivitis, ocular pain, bilateral anterior uveitis, and episcleritis. CONCLUSIONS: This is the first report, with rechallenge data, of any drug causing scleritis. Pamidronate disodium can cause vision-threatening diseases, which may require discontinuing the drug in some uveitis cases and, in this series, all cases of scleritis.

Aged↗

PubMed2003

Anterior segment indocyanine green angiography in anterior scleritis and episcleritis.

OBJECTIVE: To evaluate the pattern of anterior segment indocyanine green (ICG) angiography in episcleritis and scleritis. DESIGN: Prospective comparative (paired-eye) observational case series. PARTICIPANTS: Twenty subjects presenting clinical diseases compatible with episcleritis or scleritis. METHODS: Anterior segment ICG angiography was performed according to a standard protocol in subjects presenting either episcleritis or scleritis. Photographs of the anterior segment were taken in the early phase (up to 3 minutes after dye injection), intermediate phase (10-12 minutes) and late phase (30-45 minutes). The inflamed zones were compared with the same regions of the controlateral eye. The amount of protein ICG exudation was scored by a masked observer as follows: zero for no exudation, one for slight exudation, two for moderate exudation, and three for severe exudation. MAIN OUTCOME MEASURES: Evaluation of dye leakage, which reflects protein exudation, with anterior segment ICG angiography in episcleritis and scleritis. RESULTS: Twenty subjects with a mean age of 43 +/- 15 years (7 male, 13 female) were enrolled in the study. Thirteen subjects had anterior scleritis (7 nodular, 5 diffuse, and 1 scleromalacia perforans), and 7 subjects had episcleritis. Only 1 out of 7 subjects with episcleritis showed a slight ICG leakage (a score of one), whereas all subjects with scleritis had ICG leakage scores of one or more (P = 0.0005, Fisher exact test). CONCLUSIONS: ICG angiography of the anterior segment of the eye is a good clinical test to differentiate episcleritis from scleritis.

Adult↗

PubMed1994

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↗

PubMed1999

Use of topical steroid therapy in the management of nonnecrotizing anterior scleritis.

BACKGROUND: Nonnecrotizing anterior scleritis may be treated with a variety of therapies, including topical steroid therapy, systemic therapy with nonsteroidal antiinflammatory drugs (NSAIDs) and systemic steroid therapy. This study was carried out to determine the efficacy of topical steroid therapy in treating diffuse and nodular scleritis. METHODS: A phase I/II descriptive study was conducted. Thirty-two consecutive patients with nonnecrotizing anterior scleritis referred to a tertiary care ophthalmology cornea and uveitis practice in Ottawa were enrolled between September 1995 and February 1997. The patients received 1% prednisolone acetate, administered topically every 2 hours for at least 2 weeks. The drug was tapered off thereafter based on the clinical response. A successful treatment outcome was defined as resolution of scleritis without the need for systemic steroid or NSAID therapy by 2 months after initial presentation. RESULTS: The 2-month success rate was 47%. Of the 17 patients in whom treatment failed, 5 (29%) still had some evidence of scleritis at 2 months despite systemic treatment with steroids. There was no difference between the two groups in the rate of first recurrence of scleritis (log-rank test). INTERPRETATION: Although topical steroid therapy failed in over half of the patients, a significant number were spared systemic steroid therapy with its potential side effects. Despite the moderately high failure rate, topical steroid therapy could be considered as first-line treatment for nonnecrotizing anterior scleritis, especially in cases in which the likelihood of complications from systemic steroid or NSAID therapy is high.

Administration, Topical↗

PubMed2001

Self-inflicted anterior scleritis.

PURPOSE: To describe and clinically characterize a syndrome of self-inflicted scleritis. STUDY DESIGN: Case reports and literature review. METHODS: Two patients had persistent scleritis at presentation. Both did not respond to prescribed therapy, including systemic corticosteroids and immunosuppression. The first had unexplained, diffuse anterior scleritis and persistent linear keratoconjunctival abrasions after a work-related injury. Several objective indicators pointed to concealed noncompliance with medications. The second patient was a medical assistant with diffuse, unilateral anterior scleritis and unexplained visual loss. Systemic work-up was negative. She had pharmacologic mydriasis and keratoconjunctival abrasions at presentation. RESULTS: Systemic therapy was stopped in both patients. The first patient, who was in the process of requesting permanent disability status, showed persistent inflammation on each follow-up visit. The second patient improved with no further therapy after she was confronted with objective indicators of a self-inflicted condition. CONCLUSIONS: Although the presentation of self-inflicted scleritis can be similar to that of idiopathic or autoimmune anterior scleritis, the former may show additional findings of traumatic conjunctival and corneal abrasions. Indicators of self-inflicted etiology, of which malingering is one such entity, include evidence of concealed noncompliance with prescribed treatments and lack of response to potent antiinflammatory and immunosuppressive agents. Correct diagnosis including early psychiatric evaluation in all such cases, may help prevent unnecessary treatment and unjustified work-related compensation.

Administration, Topical↗

PubMed1997

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↗

PubMed1999

Scleritis: a clinicopathologic study of 55 cases.

OBJECTIVE: By a clinicopathologic study, to evaluate the histopathologic features associated with various causes of scleritis. DESIGN: Retrospective observational case series. PARTICIPANTS: Enucleated globes or biopsy specimens obtained from 55 cases of clinically diagnosed necrotizing scleritis. METHODS: On the basis of their histologic appearance, these cases were divided into four morphologic groups: (1) zonal necrotizing granulomatous scleral inflammation; (2) nonzonal diffuse scleral inflammation, with or without granulomatous process; (3) necrotizing inflammation with microabscesses, with or without evidence of micro-organisms in the section studied; and (4) sarcoidal granulomatous inflammation. The clinical charts were reviewed for the presence of any associated disease. RESULTS: There were 14 (25.4%) cases in the first group; 12 had clinical evidence of systemic autoimmune diseases, including 8 cases of rheumatoid arthritis and 1 each of polychondritis, Goodpasture syndrome, Wegener granulomatosis, and collagen vascular disease; of the remaining 2 cases, 1 patient had a history of herpes zoster ophthalmicus, and the other had no history of any systemic autoimmune or infectious disease. None of the 19 (34.5%) patients characteristic of group 2 had any history of systemic autoimmune or infectious disease. Eleven of the 21 (38.2%) patients in group 3 had infections, including Pseudomonas spp., gram-positive cocci, Haemophilus spp., Actinomyces spp., and fungi; in the 10 remaining cases, no micro-organisms could be detected. The one case in group 4 was diagnosed as sarcoidosis. CONCLUSIONS: On the basis of their histologic features, rheumatoid scleritis and related systemic autoimmune-mediated necrotizing scleral inflammations could be differentiated from either idiopathic or infectious scleritis; however, the histologic features of rheumatoid scleritis were similar to those of necrotizing scleritis associated with other systemic autoimmune diseases.

Adult↗

PubMed2004

[Posterior scleritis simulating acute posterior multifocal placoid pigment epitheliopathy].

INTRODUCTION: Posterior scleritis is a rare but probably underdiagnosed process. The lesions may cause diagnostic confusion with other diseases such as intraocular tumors and orbital or cerebral involvement. In the case presented, posterior scleritis simulated acute posterior multifocal placoid pigment epitheliopathy. CASE REPORT: A 43-year-old woman presented with a history of pain and eye redness with lid swelling in the left eye followed by visual deterioration. On fundus examination, there was disc and macular edema with multiple grayish-white placoid and deep chorioretinal lesions in the posterior pole. Fluorescein angiography was compatible with acute posterior multifocal placoid pigment epitheliopathy, but the inflammatory context was unusual in this disorder. An orbital computed tomographic scan and b-scan ultrasonography showed posterior scleral thickening and the diagnosis of posterior scleritis was made. Etiological investigations were negative. A pulse of corticosteroids was started and the patient's symptoms and signs improved on clinical and angiographic examination. DISCUSSION: Posterior scleritis may present with a range of clinical findings. Its clinical features may simulate many other diagnoses such as acute posterior multifocal placoid pigment epitheliopathy. Management is different in each situation: abstention in acute posterior multifocal placoid pigment epitheliopathy and a high dose of corticosteroids or even immunosuppressive therapy in posterior scleritis. CONCLUSION: This report shows that posterior scleritis must be evoked in patients with unilateral fundus appearance of acute posterior multifocal placoid pigment epitheliopathy associated with an inflammatory context. Ultrasonography or MRI must be carried out.

Adult↗

PubMed2004

Anterior scleritis: three case reports and a review of the literature.

BACKGROUND: Scleritis is a rare ocular condition involving vasculitis. It can result in severe ocular morbidity and has a high association with underlying systemic disease and mortality. Management can be challenging and under-diagnosis is a concern. Treatment usually requires systemic medications and co-management with a medical specialist in the appropriate field to manage any underlying systemic etiology. METHODS: Three cases are presented: idiopathic nodular scleritis with complete recovery; bilateral, diffuse anterior scleritis with rheumatoid arthritis; and scleromalacia perforans treated with chemotherapeutic agents and co-managed with a rheumatologist. A search of the English language literature is reported, also. RESULTS: The reported cases exemplify much of our knowledge regarding scleritis. The literature review focuses on the challenges of an accurate diagnosis and management. Management is challenging and, although standards exist, controversy remains. The literature discusses the relationship of scleritis to underlying systemic disease and the significant implication of this association. CONCLUSION: Knowledge of scleritis may aid in determining a timely and accurate diagnosis and treatment of both the ocular and any underlying systemic conditions, thus decreasing morbidity and mortality.

Adult↗

PubMed2006

Recurrent posterior scleritis--report of a case.

Posterior scleritis is a serious ocular inflammatory and potentially blinding disorder. It is uncommon and often under-recognized due to its varied presentations, and general ophthalmologists are not familiar with it. Posterior scleritis may be idiopathic or associated with systemic diseases. Visual disturbances such as blurring or distortion are the commonest symptoms. There may be pain, tenderness, and deep-seated discomfort in and around the orbit. One study reported a 49% recurrence rate of posterior scleritis. Posterior scleritis responds well to systemic nonsteroidal anti-inflammatory agents, systemic steroids, and immunosuppressive agents. Recurrences are known to occur despite therapy. However, multiple recurrences in posterior scleritis are uncommon. We report a patient with posterior scleritis who had one episode in the right eye and four episodes in the left eye over a 35-month period.

Adult↗

PubMed2005

Masquerade scleritis.

PURPOSE: To report two cases in which malignancy masqueraded as scleritis, delaying the diagnosis. METHODS: Two patients initially diagnosed and treated for unilateral scleritis were referred for management of persistent inflammation. Additional evaluation uncovered underlying malignant processes. RESULTS: The first patient presented with scleritis initially responsive to systemic corticosteroids, with relapse one month later. Upon referral, peripheral fundus examination revealed elevated lesions. Additional studies confirmed the diagnosis of choroidal melanoma. The patient was treated with proton-beam irradiation. The second patient developed necrotizing scleritis unresponsive to systemic steroids, methotrexate, and cyclophosphamide. A scleral biopsy disclosed an undifferentiated high-grade carcinoma, likely metastatic. Exenteration was performed. CONCLUSIONS: Scleritis can present a diagnostic challenge. It is often the sole initial manifestation of an occult systemic problem. Treatment-resistant scleritis should raise the suspicion of an infectious or malignant masquerade.

Adenocarcinoma↗

PubMed1991

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↗

PubMed1991

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↗

PubMed1995

[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↗

PubMed1993

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↗

PubMed1997

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↗