Scleritis in patients with granulomatosis with polyangiitis (Wegener).

AIMS: To describe and compare clinical features, complications and outcomes in patients with granulomatosis with polyangiitis (GPA)-associated scleritis with those seen in idiopathic and other autoimmune-associated scleritis, and to further describe the features that may serve as an indicator of life-threatening systemic disease. METHODS: We retrospectively reviewed electronic health records of all patients with scleritis seen at two tertiary care centres. Of 500 patients, 14 had GPA-associated scleritis and were included in this analysis. Measures included were age, gender, laterality, visual acuity and underlying systemic or ocular diseases. Clinical features (location, pain, inflammation) and ocular complications of these patients (decrease of vision, concomitant anterior uveitis and ocular hypertension) were studied and correlated. RESULTS: Fourteen of 500 patients with scleritis were GPA associated. Most of the patients with GPA-associated scleritis presented with sudden onset, bilateral, diffuse anterior scleral inflammation, with moderate-or-severe pain. Vision loss was not significantly different, and pain was more severe in these patients than in those with idiopathic scleritis. When compared with patients with other underlying autoimmune diseases, there were no significant differences found in epidemiological or clinical signs. Necrotising scleritis and corneal involvement were more commonly observed in GPA than in idiopathic scleritis and other autoimmune diseases and are often the presenting feature of the disease. CONCLUSIONS: The presence of necrotising changes or corneal involvement in the setting of scleral inflammation is highly suggestive of an underlying systemic vasculitis, of which GPA is the most common. These features should alert the doctor/optometrist and prompt a thorough diagnostic approach and an aggressive treatment given that it could reveal a life-threatening disease.

Clinical features and presentation of posterior scleritis: a report of 31 cases.

PURPOSE: To describe clinical features, ocular complications, and visual outcomes of patients with posterior scleritis. METHODS: Clinical characteristics of a subset of 31 patients with posterior scleritis were studied and compared with 469 patients with anterior scleritis. RESULTS: Of 500 patients, 31 (6.2%) had posterior scleritis. Most patients presented with subacute (80.6%), unilateral (61.3%) scleral inflammation. Pain was moderate to severe in 54.8% of patients. Concomitant anterior scleritis was observed during follow-up in 77.4% of patients and in all patients with moderate to severe pain. Patients with posterior scleritis were significantly younger (43.6 vs. 54.4 years, p < 0.001) and had significantly higher decrease of vision (29.0 vs. 14.9%, p = 0.027) than those with isolated anterior scleritis. CONCLUSIONS: Posterior scleritis must be considered in patients with decrease of vision, mild to severe pain, optic disc edema, and/or posterior uveitis. Moderate to severe pain may be associated with poorer visual outcome.

Predictive value of selected biomarkers, polymorphisms, and clinical features for oligoarticular juvenile idiopathic arthritis-associated uveitis.

PURPOSE: Uveitis is the most common extra-articular manifestation of juvenile idiopathic arthritis (JIA) and is associated with considerable morbidity. The aim of this study was to examine the risk factors associated with uveitis in oligoarticular JIA. METHODS: We conducted a chart review of 86 patients with oligoarticular JIA to assess if antinuclear antibody (ANA) status, gender, and age at JIA onset were associated with the development of uveitis. Biomarkers such as cytokine gene polymorphisms, erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were also assessed. RESULTS: Twenty-seven patients exhibited oligoarticular JIA-associated uveitis. Only the ESR at arthritis onset and the patient's age at arthritis onset were related to uveitis development in our patient sample. CONCLUSIONS: An age-associated risk of uveitis was observed in children younger than 3 years at the time of JIA onset. ESR values at arthritis onset higher than 22 mm/h were also related to uveitis development.

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.

Clinical features and presentation of infectious scleritis from herpes viruses: a report of 35 cases.

PURPOSE: To describe clinical features and presentation of infectious scleritis resulting from herpes viruses. DESIGN: Retrospective case series. PARTICIPANTS: Thirty-five patients out of 500 with scleritis. METHODS: We reviewed the electronic health records of 500 patients with scleritis, 35 of whom were diagnosed with herpes virus infection, seen at 2 tertiary referral centers. We studied the clinical features and ocular complications of this subset of patient with scleritis. MAIN OUTCOME MEASURES: Correlation between classification, severity, and symptoms (i.e., pain) and diagnosis of herpetic-associated scleritis. Vision loss, presence of associated uveitis, keratitis, glaucoma, or systemic disease were documented over the follow-up period. Other outcome measures included epidemiologic data: age, gender, laterality, visual acuity, duration of symptoms, and underlying systemic or ocular diseases. RESULTS: Of 500 patients with scleritis, 47 (9.4%) had an underlying infectious cause. Thirty-five (74.4%) of these were diagnosed with herpes virus infection, 5 (10.6%) with tuberculosis, and the remaining 7 (14.8%) with other infectious disease. Patients with herpes-associated scleritis were analyzed as a group and then compared with those with idiopathic scleritis. Most patients with herpetic scleritis presented with acute (85.7%) and unilateral (80%) scleral inflammation. Pain was moderate or severe in 68.6% of the patients. The most common type of scleritis was diffuse anterior in 80% (n = 28), followed by nodular anterior 11.4% (n = 4), and necrotizing in 8.6% (n = 3). Necrotizing anterior scleritis was more commonly seen in patients with herpetic scleritis versus patients with idiopathic disease (8.6% vs 1.2%; P<0.05). Unilaterality was also more common in herpetic scleritis (80%) than in idiopathic disease (56.7%; P<0.05). Vision loss was significantly greater in herpetic than idiopathic scleritis (34.3% vs 11.5%; P<0.001). CONCLUSIONS: The association between scleritis and infectious disease may be higher than previously reported by other series. Herpes viruses account for 7% of all scleritis cases and its diagnosis may be challenging when there is not a classically diagnostic clinical picture. We present the observed clinical features of herpetic scleritis and describe the clinical differences at presentation between patients with idiopathic scleritis and those with herpes infection.