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PubMed2003

[Echographic findings in posterior scleritis].

In the submitted paper the authors present an account on the importance of ultrasonographic examination by the method of standardized echography for early and correct establishment of the diagnosis scleritis posterior. They mention the echographic image of posterior scleritis and the specific acoustic criteria which are important from the aspect of the differential diagnosis. They analyze differences of the echographic image in posterior scleritis, melanoma of the choroid, metastatic carcinoma of the choroid and haemangioma of the choroid. When knowing the specific acoustic criteria the success rate of diagnosing posterior scleritis is high. The diagnosis is important for provision of adequate treatment and prevention of ophthalmological complications. Standardized echography is a non-invasive examination method suitable not only for assessment of the diagnosis of posterior scleritis but also for check-up examinations and follow up of the effectiveness of therapy.

Adult↗
PubMed2005

Stenotrophomonas maltophilia keratitis and scleritis.

BACKGROUND: Stenotrophomonas maltophilia is a seldom-reported pathogen in ocular infections. The report describes six cases of Stenotrophomonas maltophilia (S. maltophilia) keratitis and scleritis. To our knowledge, this is the foremost report of S. maltophilia scleritis. METHODS: Laboratory reports of patients diagnosed with S. maltophilia ocular infections were collected from the ophthalmic department of Chang-Gung memorial hospital from January 1, 2000, through December 31, 2003. On evaluation of risk factors, isolates, antibiotic sensitivities, and response to the treatment ensued. RESULTS: Of the 6 reported cases, 5 related bacterial keratitis and 2 scleritis. (One case reported S. maltophilia keratitis and secondary scleritis.) The primary risk factor in such cases is ocular surgery. The organism cultured was the single isolate in three cases (50%). The susceptibility test showed that 50%, 83%, and 100% of the isolates were sensitive to ceftazidime, a combination of trimethoprim and sulfamethoxazole, and ciprofloxacin respectively. DISCUSSION: Ocular surface compromise such as penetrating keratoplasty was a primary risk factor of S. maltophilia keratitis in our study. The results of isolates and the antibiotic sensitivities were different from previously published results. Our cases responded well to antibiotic therapy and antibiotic therapy combined with conjunctival autografting. One case of S. maltophilia keratitis and secondary scleritis had a poor prognosis, arguably associated with a co-infection of Mycobacteria chelonae.

Adult↗
PubMed1997

Increased expression of matrix metalloproteinases in vivo in scleritis tissue and in vitro in cultured human scleral fibroblasts.

Scleritis is a sight-threatening inflammatory disorder of the eye characterized by the degradation of scleral matrix. Matrix metalloproteinases (MMPs) are ubiquitous proteolytic enzymes important in physiological and pathological processes, the activity of which is stringently controlled by the action of a family of natural antagonists, the tissue inhibitors of matrix metalloproteinases (TIMPs). We hypothesized that enhanced expression of MMPs, without the negative regulatory influence of TIMPs, may be a key feature of tissue destruction in inflammatory eye diseases, such as scleritis. The aim of this study was to localize and characterize cells expressing MMPs and TIMPs in sclera affected by necrotizing scleritis and, in a parallel study, to establish whether cytokines modulate MMP expression in cultured human scleral fibroblasts. In situ hybridization and immunohistochemical analyses indicated that resident scleral fibroblasts as well as inflammatory cells such as macrophages and T lymphocytes express stromelysin, gelatinase B, and TIMP-1 in necrotizing scleritis tissue. In addition, cytoplasmic immunoreactivity for tumor necrosis factor-alpha, an inducer of MMPs, was detected in infiltrating inflammatory cells. Cultured scleral fibroblasts stimulated with the combination of interleukin-1 alpha plus tumor necrosis factor-alpha increased TIMP-1 mRNA twofold above constitutive levels. By contrast, these cytokines induced a sevenfold increase in the steady-state levels of stromelysin mRNA. Using Western blotting, stromelysin and TIMP-1 protein production paralleled mRNA induction in cytokine-stimulated human scleral fibroblasts. Culture supernatants harvested from cytokine-stimulated human scleral fibroblasts were subjected to sodium dodecyl sulfate polyacrylamide gel electrophoresis gelatin substrate zymography. Our results revealed a prominent 92-kd gelatinolytic band corresponding to gelatinase B, which was inducible with interleukin-1 alpha. These data provide evidence for our hypothesis, that an imbalance between enzyme/inhibitor ratios may be the underlying mechanism of the tissue destruction characteristic of scleritis. Our results demonstrate the potential involvement of MMPs and their modulation by cytokines produced by infiltrating inflammatory cells in destructive ocular inflammation.

Adult↗
PubMed1987

Intravenous pulse methylprednisolone in scleritis.

We treated 14 patients with scleritis with intermittent pulse doses of intravenous methylprednisolone. There was 13 patients with anterior scleritis and one patient with posterior scleritis. A grading system was developed to quantitate the degree of scleral inflammation and to follow up the response to treatment. A standard protocol of intravenous administration of methylprednisolone was followed, commencing with 1 g on three occasions in the first week. Additional immunosuppression was required in six patients. The therapy improved the patients' conditions, with a significant reduction in the severity of the scleritis in all patients. Side effects included psychological disturbances, hypertension, and elevated glucose levels, but no patient required cessation of treatment. Pulse methylprednisolone treatment alone or in combination with other immunosuppressive agents is an effective therapy in severe scleritis and has fewer potential side effects than more conventional regimens of corticosteroid administration.

Adolescent↗
PubMed1978

Clinical manifestations of brawny scleritis.

We studied four patients with posterior brawny scleritis. Two underwent enucleation for suspected melanoma, and in the other two, the correct diagnosis was made and effective therapy begun. Of seven other eyes with brawny scleritis from other sources, five were enucleated after diagnosis of choroidal melanoma and one for suspected intraocular tumor. This experience and other previous reports indicate the high incidence of diagnostic confusion regarding brawny scleritis. We therefore emphasized clinical symptoms and signs of brawny scleritis: inflammation, tenderness or pain of the globe, history of collagen vascular disease, proptosis, bilaterality, and retinal and choroidal detachment. A preserved normal choroidal vascular pattern over an elevated subretinal mass may be indicative of posterior brawny scleritis. Scleral biopsy is useful for tissue diagnosis. Radioactive phosphorus uptake tests and ultrasonography may erroneously indicate choroidal melanoma and lead to enucleation of a potentially salvageable globe.

Adult↗
PubMed1976

Scleritis and temporal arteritis.

Thirty consecutive patients with severe scleritis or episcleritis were admitted as in-patients to the Medical Ophthalmology Unit and assessed for systemic disease. There were seventeen women and thirteen men. The mean age was 53 with a median of 57 (range 23-83). Eighteen of the patients had scleritis: eleven of these had evidence of connective tissue disease and three of them had temporal arteritis. Twelve patients had episcleritis: six of them had a collagen disease and one of them developed temporal arteritis. This high incidence of temporal arteritis in association with scleritis has not been previously reported. It is important to diagnose and treat overt temporal arteritis early with parenteral steroids so that ischaemic papillopathy can be avoided. A higher incidence of collagen diseases than previously described is reported in episcleritis. It is thought that this is secondary to selection since patients with the usual self-limiting episcleritis are not normally referred for further in-patient investigation. In no patient was more than one significant diagnosis made. There was no significant medical illness in only 11% of patients with scleritis and 33% of patients with episcleritis. The majority of the non-collagen diseases (e.g. hypertension) were not previously recognized. In none of the patients with temporal arteritis was the diagnosis made before admission. It is concluded that full examination and investigation for underlying disease is indicated in both scleritis and severe episcleritis.

Aged↗
PubMed1985

Necrotizing scleritis. A clinico-pathologic study of 41 cases.

Scleritis is not a single clinical or pathologic entity. It has several distinct forms. A clinico-pathologic study of 41 cases of necrotizing scleritis suggests that these different histopathologic forms of disease may reflect different mechanisms of immunopathogenesis. These cases were divided into three main groups: (1) scleral inflammations associated with various systemic autoimmune diseases, including 11 cases of rheumatoid arthritis, three cases of Wegener's granulomatosis, one case of polychondritis, and one case of Goodpasture's syndrome; (2) infectious scleritis, consisting of four cases of herpes zoster ophthalmicus and two cases of pseudomonas scleritis; and (3) idiopathic scleritis, without evidence of systematic disorder, consisting of 19 cases. The first group exhibited predominantly necrosis of the sclera surrounded by granulomatous inflammation and vasculitis. None of these cases showed lymphoid follicles, or healing attempts manifested by proliferation of fibroblasts and blood vessels at the site of inflammation. The idiopathic group revealed few small foci of scleral necrosis and mainly non-granulomatous inflammation. In addition, there was evidence of proliferation of granulation tissue and lymphoid follicles in this group of eyes.

Adult↗
PubMed2004

Nodular scleritis and panuveitis with erythema elevatum diutinum.

PURPOSE: To report a case of nodular scleritis and panuveitis associated with erythema elevatum diutinum, a rare immunocomplex-mediated skin disease. DESIGN: Observational case report. METHODS: A 22-year-old woman who was diagnosed with erythema elevatum diutinum developed nodular scleritis and panuveitis of the right eye. She had experienced peripheral ulcerative keratitis with corneal perforation. RESULTS: All other known causes of nodular scleritis and panuveitis were investigated and ruled out. CONCLUSIONS: Erythema elevatum diutinum should be considered as an underlying systemic disease associated with nodular scleritis and panuveitis as well as peripheral keratitis.

Adult↗
PubMed1992

Six cases of scleritis associated with systemic infection.

Isolated scleritis (without keratitis) associated with infections is uncommon, and correct diagnosis and appropriate therapy for it are often delayed. Six patients with infection-associated scleritis were seen at our institution between May 1983 and May 1990 (these patients represented 4.6% of all patients with scleritis [six of 130 patients] in that period). Three of these cases were associated with systemic infections. One was associated with syphilis, one was associated with tuberculosis, and one was associated with toxocariasis. Three cases resulted from local infections. One was associated with infection with Proteus mirabilis, one was associated with infection with herpes zoster virus, and one was associated with infection with Aspergillus. The Aspergillus infection developed after trauma and the P. mirabilis-induced infection developed after strabismus surgical procedures. Four of the six cases were initially misdiagnosed and inappropriately managed. Correct diagnosis was made seven days to four years after onset of symptoms. Review of systems, scleral biopsy, culture, and laboratory investigation were used to make the diagnosis. Differential diagnosis of scleritis must include infective agents.

Aged↗
PubMed1990

Scleritis in relapsing polychondritis. Response to therapy.

The authors reviewed the records of 11 patients with relapsing polychondritis associated with active scleritis and analyzed the immunopathologic characteristics of ocular tissue from three of these. Seven patients (63%) required cytotoxic drugs, alone or in combination with low-dose oral corticosteroids. Only one patient was treated successfully with systemic corticosteroids alone. Two patients were controlled with dapsone and one with an oral nonsteroidal anti-inflammatory drug. Dapsone, which has been reported to be effective in the treatment of relapsing polychondritis, did not control the destructive scleral inflammation in six (75%) of eight patients; two (50%) of four patients with diffuse anterior scleritis were controlled with this drug. Patients with nodular and necrotizing scleritis were controlled with azathioprine and cyclophosphamide, respectively. These data suggest that the ocular manifestations of relapsing polychondritis, especially nodular and necrotizing scleritis, are less amenable to treatment with systemic corticosteroids and/or dapsone and that more potent immunosuppressants (azathioprine and cyclophosphamide) may be required to treat these lesions successfully. Results of histologic and immunofluorescent examination of conjunctival and/or scleral biopsy specimens from three patients confirmed the vasculitic nature of the eye lesions in this disease.

Adrenal Cortex Hormones↗
PubMed1991

Necrotizing scleritis after ocular surgery. A clinicopathologic study.

Necrotizing scleritis may appear after trauma to the sclera. The authors studied 10 patients in whom necrotizing scleritis developed after ocular surgery. The interval between surgery and onset of scleritis varied from 2 weeks to 6 months. Nine patients (90%) were found to have an underlying autoimmune vasculitic systemic disease, which was subsequently treated with immunosuppression. One patient was found to have a local infectious process, which was treated with antibiotics. Appropriate studies led to the discovery and subsequent treatment of a systemic disease or an infectious process in 6 of the 10 patients; the other 4 patients had been previously diagnosed. Results of immunohistochemical studies on resected conjunctival and/or sclera suggest local immune complex deposition, increased HLA-DR expression, and increased helper T-cell participation in conjunctiva and/or scleral tissues after trauma in patients with underlying systemic autoimmune vasculitic disease. The results emphasize the need for meticulous diagnostic pursuit of potentially lethal systemic autoimmune vasculitic disease in patients with necrotizing scleritis after intraocular surgery.

Aged↗
PubMed1991

Immunopathology of scleritis.

Conjunctival and scleral biopsies from 25 patients with necrotizing scleritis and 5 patients with recurrent nonnecrotizing scleritis were studied by histopathologic, immunofluorescence, and immunoperoxidase techniques. Vasculitis with fibrinoid necrosis and neutrophil invasion of the vessel wall was present in 75% of the scleral and 52% of the conjunctival specimens. Vascular immunodeposits were found in 93% of the scleral and 79% of the conjunctival tissue tested by immunofluorescence techniques. A dramatic increase in the number of inflammatory cells over normal controls was detected in both tissues by immunoperoxidase techniques. In the conjunctival epithelium, there were significantly more T-helpers, macrophages, and B cells. In the conjunctival substantia propria, there were significantly more T cells of all types, macrophages, and B cells. Likewise, scleral specimens showed an increase over controls of T cells of all types and macrophages. HLA-DR expression was dramatically increased in both tissues. Immune-complex-mediated vasculitis plays a pivotal role in the pathogenesis of necrotizing scleritis and recurrent nonnecrotizing scleritis. Induced HLA-DR expression on ocular nonimmune cells and T cell controlled responses also may participate.

Adult↗
PubMed1992

Scleritis as an initial manifestation of choroidal malignant melanoma.

PURPOSE: The purpose of this article is to present the unusual circumstances in which malignant melanoma of the choroid can masquerade as scleritis, thus confounding its diagnosis. METHODS: Three cases of plaque-like malignant melanomas of the choroid are reported that, on initial examination, had features of scleritis. The events leading to their eventual correct diagnosis also are presented. RESULTS: In each case, there was ocular pain, blurred vision, anterior chamber and/or vitreous cavity cellular reaction, and an exudative retinal detachment associated with an ill-defined, relatively flat variably pigmented choroidal mass. In all three cases, the inflammatory component responded promptly to corticosteroid treatment and was accompanied by visual improvement. In two eyes, shrinkage of the choroidal mass accompanied the corticosteroid treatment, lending support to a working diagnosis of scleritis. By demonstrating expansion of the choroidal masses, examination of sequential fundus photographs influenced the decision to enucleate the eyes for presumed malignant choroidal melanoma. CONCLUSION: Clinicians should be alert to the circumstances in which malignant melanomas of the choroid can masquerade as scleritis. Careful evaluation by ophthalmoscopy, ultrasonography, fundus photography, and subsequent sequential examination is necessary to arrive at the correct diagnosis.

Adult↗
PubMed1992

Diagnostic value of anti-neutrophil cytoplasmic antibodies in scleritis associated with Wegener's granulomatosis.

Serum antineutrophil cytoplasmic antibodies (ANCAs) are a sensitive and specific marker for generalized Wegener's granulomatosis. However, ANCA sensitivity and specificity in identifying patients in whom ophthalmic signs constitute the presenting or only definitive manifestation of Wegener's granulomatosis have not been tested. The authors report on 7 patients in whom scleritis was the initial manifestation leading to the diagnosis of Wegener's granulomatosis. Six had the limited form of Wegener's granulomatosis. Results of serum ANCA tests were positive in all these patients. In contrast, the serum ANCA was negative in 54 patients with ocular inflammation due to other disorders; 16 of these patients had scleritis. Serial ANCA titers reverted to normal in only two of the four patients with Wegener's granulomatosis who attained clinical remission. One of the patients who did not revert to normal experienced relapse 2 months after discontinuation of therapy. Antineutrophil cytoplasmic antibodies appear to be both sensitive and specific for Wegener's granulomatosis-associated scleritis, and testing is useful in the evaluation of patients with scleritis.

Adolescent↗
PubMed1995

Therapy of nonnecrotizing anterior scleritis with subconjunctival corticosteroid injection.

OBJECTIVE: To determine the safety and efficacy of subconjunctival triamcinolone (Kenalog) in treating nonnecrotizing anterior scleritis. DESIGN: The authors conducted a retrospective review of all patients treated with depot subconjunctival corticosteroid injection for scleritis from January 1988 to May 1993. Response to therapy was determined by subjective improvement in pain and a decrease in clinical signs of ocular inflammation. All patients received subconjunctival injections of triamcinolone by the same technique, and the minimum observation period for complications was 6 weeks. RESULTS: Eighteen patients (90%) had relief of their symptoms with clinically observable improvement in inflammation, whereas two patients (10%) responded poorly. Nine patients (45%) required no further therapy. Average symptom-free interval was 18 weeks in patients with recurrent scleritis. No complications of scleral thinning, perforation, or glaucoma occurred in any patients. CONCLUSION: Subconjunctival triamcinolone injection is highly efficacious in treating nonnecrotizing anterior scleritis without unreasonable risk of thinning and/or perforation and should be considered as adjunctive therapy in localized disease.

Adult↗
PubMed1996

Scleritis and mucosal-associated lymphoid tissue lymphoma: a new masquerade syndrome.

PURPOSE: To present a new masquerade syndrome showing features of mucosal-associated lymphoid tissue (MALT) lymphoma associated with choroidal white dots and scleritis. Differentials including systemic lymphoma, central nervous system lymphoma, and etiologies of white-dot syndromes and scleritis are discussed. PATIENTS AND METHODS: A 42-year-old man who had decreased vision and ocular redness of his right eye for 4 years had a biopsy-proven diffuse anterior and posterior scleritis associated with intense circumferential perilimbal chemosis and ipsilateral yellow-white choroidal dots. A new conjunctival biopsy was performed because of unresponsiveness to high-dose systemic steroid and cyclophosphamide therapy. Immunostains for lymphocyte markers were preformed. RESULTS: A morphologically and immunohistochemically typical, monotypical mu-kappa immunoglobulin light chain secreting B-cell MALT-lymphoma was diagnosed. Eighteen months after completion of radiotherapy, the patient recovered completely, except for the choroidal dots, which remained unchanged. CONCLUSION: When scleritis, even histologically proven, fails to respond to immunosuppressive therapy, a new biopsy is mandatory to rule out a misdiagnosed MALT lymphoma.

Adult↗
PubMed2003

Bilateral combined retinal and choroidal detachment in antineutrophil cytoplasmic antibody-positive scleritis.

PURPOSE: To describe a rare complication of scleritis in a patient with positive serum antineutrophil cytoplasmic antibodies (ANCA). METHODS: We present a case report of a patient who developed bilateral retinal and choroidal detachment associated with ANCA-positive scleritis. RESULTS: Oral steroids alone were insufficient to maintain sustained remission of ocular inflammation. Clinical relapse of the scleritis was associated with ANCA titres becoming positive again. A combination of cyclophosphamide and steroids was successful in achieving control of the intraocular inflammation over a follow-up period of 18 months. CONCLUSION: Bilateral combined retinal and choroidal detachment is a potentially blinding complication of ANCA-positive scleritis. Monitoring of ANCA titres is useful in the management of the disease.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1998

Successful management of Aspergillus scleritis by medical and surgical treatment.

BACKGROUND: Inflammatory scleral disease is frequently associated with autoimmune disorders and only occasionally caused directly by an infective agent. Fungal infections primarily involving the sclera are rare, and the outcome is generally poor. Here we report three patients with post-operative Aspergillus scleritis who were successfully managed by medical therapy and surgical intervention. PATIENTS: Scleral infection with Aspergillus sp. was diagnosed 6 and 5 months after cataract extraction in a 76-year-old diabetic and an 82-year-old woman respectively, and in a 54-year-old man 3 months after trabeculectomy. Swabs and/or scrapings had not been conclusive and the diagnosis of Aspergillus infection was established in all cases only after scleral biopsy. RESULTS: The infection was eliminated in all cases. This was achieved in one eye by treatment with systemic and topical amphotericin B. The two patients with fungal scleritis after cataract extraction required in addition to the medical therapy (oral itraconazole, topical econazole and amphotericin B) scleral excisions and patch grafts to control infection. CONCLUSION: Fungal scleritis may remain undiagnosed for months. A scleral biopsy may be necessary to establish this diagnosis. Prolonged systemic antifungal therapy alone may not eradicate fungal infection. Surgical excision improves the outcome of fungal scleritis.

Aged↗