Maite Sainz de la Maza

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.

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.

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.

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.

Epidemiology of uveitis in a Western urban multiethnic population. The challenge of globalization

To report the anatomical pattern and etiological spectrum of uveitis in an urban multi‐ethnic population from Barcelona, Spain. General and specific epidemiological data for the most prevalent aetiologies are also calculated.

Cataract surgery in ocular cicatricial pemphigoid.

The authors report the results of their experience with cataract surgery in 20 patients (26 eyes) with biopsy-proven cicatricial pemphigoid. All patients were on systemic immunosuppression at the time of surgery (dapsone, azathioprine, cyclophosphamide, or combinations) and were treated with perioperative oral corticosteroids. Patients were evaluated pre- and postoperatively for conjunctival inflammation, conjunctival cicatrization, degree of keratopathy, and disease stage. No patient progressed in disease stage. Vision improved an average of 3.5 Snellen lines (-3 to +8). Worse outcome was associated with chemotherapy intolerance or the presence of any preoperative conjunctival inflammation. Thirteen patients remained on immunosuppressives for the entire study. Corneal ulcers developed postoperatively in three patients in whom continued immunosuppression was not tolerated. Possible mechanisms for inflammatory exacerbation after surgery are discussed. Results indicate that after successful abolition of all conjunctival inflammation through chemotherapy, cataract surgery may be safely performed in patients with cicatricial pemphigoid.

Infectious scleritis: report of four cases.

While systemic autoimmune diseases are the main possibilities in the differential diagnosis of scleritis, other less common etiologies such as infections must also be considered. The authors report four cases of infectious scleritis to review predisposing factors, clinical characteristics, methods of diagnostic approach, and response to therapy. Two patients had primary scleritis and two patients had secondary scleritis following extension of primary corneal infection (corneoscleritis). Diagnoses included three local infections (one each withStaphylococcus. Acanthamoeba, and herpes simplex) and one systemic infection (Lyme disease). Stains, cultures, or immunologic studies from scleral, conjunctival, and/or corneal tissues, and serologic tests were used to make the diagnosis. Medical therapy, including antimicrobial agents, was instituted in all patients, and surgical procedures were additionally required in two patients (scleral grafting in one and two penetrating keratoplasties in another); the patient who required two penetrating keratoplasties had corneoscleritis and underwent eventual enucleation. Infectious agents should be considered in the differential diagnosis of scleritis.

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.

Scleral Grafting for Necrotizinf Scleritis

Although systemic immunosuppressive chemotherapy is effective in halting progressive necrotizing scleritis, the onset of its action may be too slow to prevent profound scleral thinning and/or traumatic or spontaneous perforation. Scleral homografts may be used to maintain the integrity of the globe until immunosuppressive drugs can take effect. The authors reviewed their experience with scleral homografts in 12 patients with progressive necrotizing scleritis; eight (all with autoimmune disease) had concomitant chemotherapy and four (two with autoimmune disease) did not. Grafts remained stable in patients receiving both surgical and drug therapy over a mean follow-up of 12 months. One graft melted after discontinuation of chemotherapy, but regrafting and renewed immunosuppression salvaged the eye. Grafts in two of the patients not initially given chemotherapy melted rapidly (within 14 and 45 days, respectively). Both eyes were salvaged by regrafting and/or addition of chemotherapy. Though rarely successful by itself against necrotizing scleritis, scleral grafting is a useful adjunct to chemotherapy.

Ocular Adverse Events of Systemic Inhibitors of the Epidermal Growth Factor Receptor: Report of 5 Cases

PURPOSE To describe the ocular effects associated with the administration of the systemic epidermal growth factor receptor (EGFR) inhibitors panitumumab and erlotinib. DESIGN Retrospective, noncomparative interventional case series. PARTICIPANTS Ten eyes of 5 patients in treatment with systemic EGFR inhibitors, 4 patients with erlotinib for end-stage lung carcinoma, and 1 patient with panitumumab for end-stage colorectal cancer. METHODS Data collected from charts included gender, age at presentation, systemic disease, and clinical presentation in each eye. MAIN OUTCOME MEASURES Demographics on presentation and clinical findings. RESULTS Multiple epithelial defects were observed in all 10 eyes, corneal melting and thinning were observed in 3 eyes of 2 patients, 2 eyes of 1 patient presented with lower lid ectropion, and 2 eyes of 2 patients presented with corneal perforation, both requiring a penetrating keratoplasty. CONCLUSIONS Severe ocular side effects, including corneal perforation, may be associated with the use of the EGFR inhibitors panitumumab and erlotinib.