Clinical Response to Tocilizumab in Severe Thyroid Eye Disease.

Tocilizumab, in a preliminary study, was reported to be an effective therapy for moderate to severe thyroid eye disease. The authors describe the clinical response of 2 severe thyroid eye disease cases refractory to intravenous steroids and orbital decompression. Both patients demonstrated improved clinical activity scores with minimal side effects after tocilizumab therapy. In addition, post tocilizumab orbital fat biopsies demonstrated benign adipose tissue without evidence of inflammatory cells.

A case of bilateral trochleitis in adult-onset Still's disease.

OBJECTIVES: We describe the first case of adult-onset Still's disease (AOSD) presenting with trochleitis and successfully treated with an interleukin-1 receptor antagonist. METHODS: This is a descriptive case report of one patient, identified in consultation with the inpatient ophthalmology consult service. RESULTS: A 23-year-old Caucasian male initially presented with complaints of sore throat, myalgias, rash, and fever. After he failed to respond to antibiotic therapy for presumed streptococcal pharyngitis and developed left eye pain with upgaze, he was admitted to the hospital. Laboratory workup revealed elevated inflammatory markers and liver enzymes. He was eventually diagnosed with AOSD. During his hospitalization he developed binocular vertical diplopia, worsening of left eye pain on upgaze, and exquisite tenderness over the region of the left trochlea, consistent with trochleitis. This diagnosis was supported by magnetic resonance imaging (MRI). Soon after onset of left eye symptoms, the right eye developed identical symptoms. He was started on intravenous corticosteroids, which led to the rapid resolution of his ocular and systemic symptoms. However, after transition to oral corticosteroids, his symptoms recurred. He was started on anakinra, an interleukin-1 (IL-1) receptor antagonist, which led to resolution of his ocular and systemic symptoms. CONCLUSIONS: Trochleitis is an unusual form of orbital inflammation localized to the superior oblique tendon and trochlea complex. It has been associated with a number of systemic inflammatory conditions including systemic lupus erythematous, rheumatoid arthritis, and juvenile idiopathic arthritis. This is the first case of a patient with AOSD presenting with trochleitis. Treatment with IV corticosteroids and anakinra resulted in rapid resolution of his ocular and systemic symptoms.

Salzmann nodular degeneration.

PURPOSE: To describe the characteristic, clinical, and epidemiological features of Salzmann nodular degeneration. METHODS: Retrospective review of cases. All patients with diagnosis of Salzmann nodular degeneration examined on the Cornea and External Disease Service in the Department of Ophthalmology and Vision Science at the University of California, Davis, were included in this review. Demographic features, clinical characteristics, treatment regimens, surgical procedures, and outcomes were recorded. Descriptive statistics, correlation statistics in bilateral cases, and simple regression models were constructed to assess the effect of potential indicators of severity. RESULTS: One hundred eighty eyes of 108 patients were included in this review. Seventy-nine patients (72.2%) were female and 29 (27.8%) were male. Seventy-two patients had bilateral disease. The mean age for all patients was 60.8 (13-92) years, and the mean follow-up time was 61.2 months (0-357 months). 76.1% of all eyes were from White. Mean logarithm of the minimum angle of resolution best-corrected visual acuity was 0.24 (Snellen equivalent 20/35, range -0.12 to 2.60, SD 0.44). Decreased visual acuity was the most common symptom in 30.6% of patients. The visual axis was affected in 30% of the cases. Meibomian gland dysfunction was the most common coexistent condition, identified in 41.7% of the cases. For bilateral cases, Spearman correlations for best-corrected visual acuity, magnitude of astigmatism, spherical equivalent, and disease extension were statistically significant (P = 0.001). The number of quadrants affected was found to be a significant predictor for astigmatism (P = 0.01). Surgery was indicated in 41 eyes of 30 patients. Decreased visual acuity was the most common indication for superficial keratectomy. Patients with more than 1 quadrant of the cornea affected or those in which the central visual axis was involved were more likely to require surgery (P = 0.015 and 0.0001, respectively). The surgical outcome was satisfactory in 90.2% of the cases; 9 eyes (21.9%) developed recurrences. CONCLUSIONS: Salzmann nodular degeneration is a disease of uncertain etiology in which inflammation of the ocular surface may play a role. It predominantly affects women in the sixth decade of life. Management with conservative therapy is generally adequate, and in cases that require surgical intervention, simple nodulectomy is usually effective.

Cellular reorganization in the human retina during normal aging.

PURPOSE: To characterize the nature and extent of neuronal reorganization in the human retina during normal aging. METHODS: Retinas of young (18-34 years old) and aged (68-77 years old) human donors were examined. Immunocytochemical methods and antibodies directed against Go-alpha, protein kinase C, parvalbumin, calbindin, calretinin, and choline acetyltransferase were used to stain different retinal cell types. Confocal images of retinal sections from the optic disc to the peripheral edge were taken at three eccentricities, and the density and length of cellular processes were quantified with neuroanatomical analysis software. RESULTS: Dendritic fibers of rod and On-cone bipolar cells were found to extend well beyond the normal boundary of the outer plexiform layer (OPL) into the outer nuclear layer (ONL) in aged retinas. Length and density of these elongated fibers were significantly greater in aged than in young retinas. This phenomenon demonstrated a clear spatial gradient that was most prevalent in the periphery and was infrequent in the central region of the retina. Horizontal cells, which normally make triad synaptic connections with photoreceptors and bipolar cells, also had dendrites that extended into the ONL in aged retinas, and these were spatially juxtaposed with the elongated dendrites of bipolar cells. CONCLUSIONS: Rod and On-cone bipolar cells, as well as horizontal cells of the human retina, undergo extensive dendritic reorganization during normal aging. Although literature on aging has tended to emphasize degenerative and regressive changes, the present findings provide evidence for a remarkable degree of cellular plasticity in the aged human retina.

Dendrites of rod bipolar cells sprout in normal aging retina.

The aging nervous system is known to manifest a variety of degenerative and regressive events. Here we report the unexpected growth of dendrites in the retinas of normal old mice. The dendrites of many rod bipolar cells in aging mice were observed to extend well beyond their normal strata within the outer plexiform layer to innervate the outer nuclear layer where they appeared to form contacts with the spherules of rod photoreceptors. Such dendritic sprouting increased with age and was evident at all retinal eccentricities. These results provide evidence of retinal plasticity associated with normal aging.