Search

Objective: This study aimed to evaluate the possible safety and effectiveness of infliximab in patients with active scleritis., Study Design: Prospective, nonrandomized, open-label pilot study (Protocol No. 04-EI-0065)., Participants: Five patients with active anterior scleritis., Methods: This single-centre, pilot study of infliximab for the treatment of active anterior scleritis was conducted at the National Eye Institute, National Institutes of Health, between 2003 and 2007. Scleritis patients with active disease who had used at least 1 conventional immunosuppressive agent in the past were included. Primary outcome was a 2-step decrease in scleral inflammation within 14 weeks. Patients received infliximab (5 mg/kg) at baseline, at weeks 2 and 6, and every 4 weeks through week 30, after which the infusion interval was increased (week 36, 48)., Results: All patients met the primary outcome by achieving quiescence of their active scleritis by week 14 with no additional immunosuppressives. However, after 14 weeks 1 patient developed new-onset intraocular inflammation that did not respond to reinduction and was terminated from the study. Side effects attributable to infliximab included ear infection with transient decreased hearing, urinary tract infection, lower respiratory tract infection, and facial rash in 1 patient and urinary tract infection, diarrhea, upper respiratory tract infection, nasal congestion and headache, mouth sores, head tremor, and occasional numbness and tingling in extremities in another patient, all of which resolved spontaneously or with appropriate treatment., Conclusions: Infliximab may be considered as a viable option in treating patients with active scleritis; however, patients should be monitored closely for potentially serious side effects.

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.

Purpose: To describe the technique and review the indications and success of tectonic corneal lamellar grafting for the management of severe scleral melts after pterygium surgery., Design: Retrospective, noncomparative, interventional case series., Participants: Twenty cases of severe scleral necrosis after pterygium surgery (1993-1999)., Intervention: Tectonic corneal lamellar grafting. Surgery involved (1) removal of all devitalized or infected scleral tissue surrounding the melt; (2) use of lamellar or full-thickness donor corneal tissue, fashioned to fit the scleral defect exactly or a 0.25-mm diameter larger; and (3) placement of a pedicled or free conjunctival flap over the corneal lamellar graft., Main Outcome Measures: Eradication of progressive scleral necrosis, preservation of globe integrity, eradication of infection, and preoperative and postoperative visual acuity., Results: Sixteen (80%) of 20 cases developed severe scleral necrosis that required tectonic surgery after bare sclera pterygium excision with mitomycin C or beta-irradiation. Surgery was also therapeutic to eradicate progressive infection in 6 cases of infective scleritis that did not respond to maximal medical treatment. Scleral melting presented 1 month to 20 years after initial pterygium surgery in healthy, immune-competent adults. Therapeutic and tectonic success was achieved in 19 cases (95%); in 1 case, recurrence of fusarium fungal infection led to severe graft necrosis and intraocular spread. Among the cases of infectious scleritis, three eyes required repeat lamellar grafting to successfully eradicate infection., Conclusions: Tectonic and therapeutic lamellar keratoplasty, combined with aggressive antibiotic therapy, preserved globe integrity and eradicated infection in cases of severe scleral melting after pterygium surgery.

Purpose: To report the case of a patient with undiagnosed Hodgkin's lymphoma who presented with coexistent unilateral nodular episcleritis and scleritis., Design: Interventional case report and literature review, Methods: Review of clinical history, laboratory findings, histology of episcleral and cervical lymph node biopsies, and follow-up., Results: A 20-year-old female presented with a 5-month history of redness and pain in her left eye, with associated symptoms of dyspnea, malaise, and fever. The patient was found to have multifocal nodular episcleritis and scleritis that was not responsive to topical steroids or systemic nonsteroidal anti-inflammatory treatment. Laboratory tests subsequently revealed evidence of systemic inflammation, and radiologic studies showed extensive mediastinal and cervical adenopathy. A cervical lymph node biopsy showed Reed-Sternberg cells and a chronic lymphocytic infiltrate consistent with nodular sclerosing Hodgkin's lymphoma. Histopathologic analysis of an episcleral nodule revealed a necrotizing granuloma with vasculitis. Systemic chemotherapy was instituted for the Hodgkin's disease; this therapy abolished the nodular scleritis., Conclusions: This case raises the possibility of concurrent undiagnosed systemic vasculitis with only an ocular manifestation with Hodgkin's lymphoma, either as a coincidence or as a paraneoplastic syndrome. Moreover, it emphasizes the important role of tissue biopsy in establishing diagnosis and directing treatment., (Copyright 2003 by the American Academy of Ophthalmology.)

Purpose: To report an unusual presentation of pyoderma gangrenosum with ocular and extracutaneous involvement., Design: A single, interventional case report., Methods: A 28-year-old female presented with nodular scleritis in the right eye and left orbital inflammation lasting 1 year. The patient initially responded to systemic steroids, but then ocular inflammation became more severe, associated with onset of fever, myalgias, and elevation of the erythrocyte sedimentation rate, accompanied by formation of multiple cutaneous abscesses., Main Outcome Measures: Ocular and systemic disease control., Results: The diagnosis of pyoderma gangrenosum was made 1 year after the occurrence of ocular symptomatology, when the patient presented with ocular and skin lesions and abscesses in the spleen and liver., Conclusions: Pyoderma gangrenosum is a rare, necrotizing, noninfectious ulcerating skin disease. Ocular and extracutaneous involvement rarely occur, but the disease should be included in the differential diagnosis of scleritis and orbital inflammation.

Objective: This study describes the clinical features, management, and outcome of 19 patients who had severe Acanthamoeba sclerokeratitis (ASK) unresponsive to conventional management, requiring systemic immunosuppression to control disease., Design: Retrospective, non-comparative, interventional case series., Participants: Records of all patients with Acanthamoeba keratitis treated at Moorfields Eye Hospital between 1989 and 2000 were reviewed. From more than 200 patients, 19 who developed ASK treated with systemic immunosuppression were identified., Main Outcome Measures: Visual acuity, level of pain, and degree of inflammation were recorded after immunosuppressive treatment., Results: ASK requiring immunosuppression occurred in 20 eyes of 19 patients (11 males and 8 females). The mean age (mean +/- standard deviation) at onset was 38.6 +/- 13.2 years. On presentation, best-corrected visual acuity was counting fingers or worse in 11 eyes (55%), 6/18 to 6/60 in 5 eyes (25%), and 6/12 or better in 4 eyes (20%). The mean time between onset of initial symptoms of Acanthamoeba keratitis and commencement of systemic immunosuppression was 4.8 +/- 3.5 months. The mean duration of immunosuppression required to control inflammation was 7.2 +/- 3.9 months. Severe scleritic pain remained uncontrolled in two patients and resulted in enucleation. Best-corrected visual acuity at final follow-up was counting fingers or worse in eight eyes (40%), 6/18 to 6/60 in six eyes (30%), and 20/40 or better in six eyes (30%). The mean follow-up period after resolution of inflammation was 24.3 +/- 20.9 months (range, 0.2-59.7 months)., Conclusions: ASK is an uncommon complication of Acanthamoeba keratitis. The scleritis associated with this infection seems to be an immune-mediated response. After topical amebicidal treatment, systemic immunosuppression may be required to control the pain and tissue destruction associated with ASK.

Objective: To describe the characteristics and systemic disease associations of episcleritis in childhood., Design: Retrospective, observational case series., Participants: Twelve children diagnosed with episcleritis between July 1981 and June 1998. METHODS/TESTING: Complete eye and systemic evaluations., Main Outcome Measures: Characteristics of episcleritis and presence and nature of concurrent systemic disease., Results: The 12 children (10 boys and 2 girls) ranged in age from 13 months to 16 years. Five children had bilateral simple episcleritis, one had bilateral nodular episcleritis, and six had unilateral simple episcleritis. The eye examination was otherwise normal and recovery was uneventful in all cases. Six of the nine children older than 5 years of age had one of the following rheumatologic diseases: systemic lupus erythematosus, juvenile rheumatoid arthritis, spondyloarthropathy, inflammatory bowel disease, rheumatic fever, or polyarteritis nodosa. All three children younger than 5 years of age had simple episcleritis, an antecedent viral illness, and presented within 2 months of each other., Conclusions: Episcleritis is a rare occurrence in childhood, especially in children younger than 5 years of age. In older children, it is frequently associated with rheumatologic disease.

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.

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.

Background: Pseudomonal scleritis is a serious and potentially blinding infection that usually is resistant to medical management., Methods: Results for three patients with pseudomonal scleritis who were treated with both topical anti-infectives and a combination of intravenous ceftazidime and aminoglycoside are presented in this case series., Results: All three patients had a rapid response to the addition of combination intravenous drug therapy to topical therapy; eradication of the infection and healing of the ocular surface occurred within 8 weeks. Only one patient, in whom cystoid macular edema developed, lost useful vision as a result of the infection., Conclusions: Combination therapy with intravenous ceftazidime and aminoglycoside may be more effective than single-intravenous agents when used in addition to topical antibiotics and may obviate the need for adjunctive surgical procedures, such ascryotherapy, surgical extirpation, or conjunctival recession.

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.

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.

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.

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.

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.

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.

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.

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.

The clinical features of 290 patients with scleral inflammation were reviewed to determine whether a classification based on the anatomical site and clinical appearance of the disease at presentation reflected its natural history. The authors' results confirm that the majority of patients remain in the same clinical category throughout the course of their disease. Of the 104 (35.9%) patients who experienced a recurrence of their disease, only 12 had progressed from diffuse to nodular disease, and 10 patients who originally had nodular disease developed scleral necrosis. Patients with necrotizing scleritis were older than patients in the other groups and more frequently had an associated systemic disease than patients with either diffuse or nodular disease; necrotizing scleritis was the most difficult disease to treat. Diffuse anterior scleritis had a lower incidence of visual loss (9%) than either nodular scleritis (26%) or necrotizing disease (74%), and, therefore, the authors consider nodular scleritis a disease of intermediate severity between diffuse scleritis and necrotizing disease. In this series, 12% of patients presented with posterior scleritis, and visual loss was most frequent in this group (84%).

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.

We describe a 56-year-old woman with benign hypergammaglobulinemic purpura in whom scleritis, acute stromal keratitis and peripheral corneal furrowing developed. Initial therapy with topical antibiotics and steroids failed to control the scleritis and keratitis. Subsequent treatment with plasmapheresis led to a decrease in circulating immune complex and serum immunoglobulin levels, with resolution of the ocular inflammation. After 8 years of follow-up the serum IgG and IgM levels were again elevated; however, the corneal disease remained quiescent. Plasmapheresis may have a role in the therapy of this and other immune-complex-mediated ocular diseases.

Although toxoplasmosis is the most common infectious cause of posterior intraocular inflammation, it is rarely described in association with scleritis. The authors present five cases of toxoplasmosis with scleritis. Two of the five cases were diagnosed clinically and serologically as having toxoplasmosis. Their retinochoroiditis and scleritis responded well to medical therapy. Retinochroiditis and scleritis that was refractory to treatment developed in the other three patients, two of whom had been receiving immunosuppressive therapy for systemic diseases. Their therapeutic regimens did not include treatment for toxoplasmosis. All three eyes became blind and were enucleated. Results of pathologic examination of all three enucleated eyes showed Toxoplasma gondii in the retina. There was severe inflammation of the retina, choroid, and sclera. Toxoplasmosis should be considered in the clinical differential diagnosis of scleritis associated with retinochoroiditis, particularly in immunosuppressed patients.