Rosanna Dammacco

Th1 polarization of the immune response in Behçet's disease: a putative pathogenetic role of interleukin-12.

OBJECTIVE To investigate whether immunologic abnormalities in patients with Behçets disease (BD) are related to abnormalities of the Th1/Th2 ratio. METHODS Th1/Th2 cytokine production by peripheral blood lymphocytes (PBL) from 31 patients with BD, 11 patients with inflammatory arthritis, and 10 healthy blood donors was evaluated by intracellular immunofluorescence staining. Serum interleukin-12 (IL-12) levels were measured using an enzyme amplified-sensitivity immunoassay. The effect of recombinant IL-12 (rIL-12) on spontaneous and Fas-mediated apoptosis of phytohemagglutinin (PHA)-stimulated PBL was evaluated by flow cytometry using propidium iodide (PI) staining and a bromodeoxyuridine (BrdU)/PI procedure. RESULTS Intracellular immunofluorescence staining of IL-2, IL-4, and interferon-gamma (IFNgamma) in CD3+ lymphocytes from BD patients demonstrated a strong polarization of the immune response toward the Th1 pathway that correlated with the progression of BD. Peripheral Th1 cells were significantly increased in patients with active disease (n = 14) as compared with those in patients in complete remission (n = 17), patients with inflammatory arthritis, and normal donors. In addition, serum IL-12 levels were correlated with peripheral Th1 lymphocytes and disease progression. Apoptotic analysis revealed that PHA-activated PBL from patients with active disease were highly sensitive to spontaneous and Fas-mediated activation-induced cell death. However, addition of rIL-12 to complete medium prevented this spontaneous and Fas-induced apoptosis and enhanced the proliferation of Th1 lymphocytes. CONCLUSION Taken together, these results indicate that a strong Th1 immune response occurs in active BD and suggest that IL-12 plays a substantial part in the pathogenesis of BD. By preventing spontaneous and Fas-induced cell death, in fact, it results in an abnormal growth of autoreactive Th1 lymphocytes that could contribute to the prolonged inflammatory autoimmune condition of BD.

Intravitreal triamcinolone acetonide in refractory pseudophakic cystoid macular edema: functional and anatomic results.

Purpose To evaluate safety and efficacy of intravitreal triamcinolone acetonide (TAAC) injections in the treatment of refractory pseudophakic cystoid macular edema (CME). Methods Seven eyes of six patients (age range: 50–74) with pseudophakic CME resistant to standard treatment received intravitreal injections of 4 mg of TAAC with all vehicle. Mean preinjection duration of CME was 18.3 months. A mean of 2.1±1.2 (range 1 to 4) treatments were performed in four eyes (57.1%) when visual acuity deteriorated towards baseline levels. Visual acuity assessment, optical coherence tomography (OCT), and fluorescein angiography (FFA) were performed pre- and postoperatively to evaluate results of TAAC injections. Intraocular pressure (IOP) and complications related to treatment were assessed. Results After 11.1±3.9 months, mean best-corrected visual acuity (BCVA) increased (p=0.019) from 20/132 to a best value of 20/38. Mean macular thickness decreased from 517.29±146.98 mm to a best value of 263.71±83.13 mm (p=0.0018). Area of fluorescein leakage decreased (p<0.0001) from 11.84±0.93 mm2 at baseline to a minimal value of 3.86±0.98 mm2. The anatomic and functional improvement appeared after 1 month from the intravitreal injection and persisted through at least 3 months of follow-up. At the end of follow-up BCVA, macular thickness, and area of fluorescein leakage did not differ from baseline. Four eyes (57.1%) developed IOP values higher than 21 mmHg, controlled by topical treatment. Two patients developed an endophthalmitis-like reaction. Conclusions Intravitr eal TAAC was relatively safe and effective in resistant cases of pseudophakic CME with a temporary beneficial effect on visual acuity and macular edema.

Three-port lens-sparing vitrectomy for aggressive posterior retinopathy of prematurity : Early surgery before tractional retinal detachment appearance

Purpose Aggressive posterior retinopathy of prematurity (APROP) may suddenly develop into tractional retinal detachment (TRD), often resulting in poor vision if untreated. The aim of the current study is to examine the anatomic results and complications of lens-sparing vitrectomy (LSV) for stage 3 APROP, before TRD appearance. Methods A retrospective, noncomparative, consecutive case series of 13 eyes of 9 patients (mean gestational age 24.1±0.9 weeks [range: 23–25 weeks], mean birthweight of 725.8±107.9 grams [range: 598–897 grams]) with stage 3 APROP was carried out. The eyes did not respond to at least one session of retinal laser photocoagulation, showing signs of disease progression. All eyes underwent 20-gauge LSV before retinal detachment appearance. Results All eyes underwent 20-gauge three-port LSV and intraoperative additional laser photocoagulation. At the end of the surgery, five eyes were tamponaded with air; in eight eyes, a balanced salt solution was left in the vitreous cavity. After 13.5±5.3 months of follow-up (range: 4–22), the retina was completely attached in all eyes, without any signs of progression. The authors did not observe any intraoperative or postoperative complications. Conclusions Surgical approach to stage 3 APROP refractory to laser photocoagulation could be effective and safe in order to avoid the progression of the disease.

Orbital pseudotumor in a child: diagnostic implications and treatment strategies

Orbital pseudotumor is a benign, idiopathic, non-infectious and non-neoplastic clinical syndrome characterized by the presence of an inflammatory mass at orbital level with no identifiable cause. The disease is rarely observed in the pediatric population. This article describes a relapsing bilateral orbital pseudotumor in a young girl. The diagnostic implications and treatment strategies are discussed.

Solitary eyelid Kaposi sarcoma in an HIV-negative patient.

Purpose: To describe a case of localized Kaposi sarcoma (KS) of the eyelid in an HIV-seronegative patient. Methods: An 80-year-old man developed an ulcerated nodular tumor-like mass that grew rapidly on his left upper eyelid. There were no similar lesions elsewhere. The eyelid lesion was completely excised and histopatologically examined. Serological analyses and molecular biologic techniques, including polymerase chain reaction, were used. Results: Laboratory examinations were within normal limits, and serology for HIV was negative. Histological sections revealed a vascular proliferation composed predominantly of small slit-like blood vessels and epithelioid spindle cells, supporting the diagnosis of KS. Polymerase chain reaction was positive for human herpesvirus 8. During a 2-year follow-up, no recurrences, development of new lesions, or HIV seroconversions were observed. Conclusion: This is a classic KS involving only the eyelid in an HIV-negative patient. Location in the eyelid is a possible, albeit rare, initial solitary manifestation of KS in elderly HIV-negative patients. Surgery is both safe and effective.

Ocular Involvement in Systemic Lupus Erythematosus: The Experience of Two Tertiary Referral Centers

ABSTRACT Purpose: To assess the prevalence of the ocular manifestations related to the disease and/or ascribable to the administration of potentially toxic drugs in a cohort of 98 patients with systemic lupus erythematosus (SLE). Methods: Retrospective, observational study reporting the experience of two tertiary referral centers. Results: Overall, an ocular involvement was detected in 29 patients (29.6%), sometimes preceding of months the diagnosis of SLE, more often revealed at diagnosis or throughout its course. More than a single ocular manifestation was found in 20 of the 29 patients with ophthalmological findings (68.9%). The array of ocular morbidity included, in a decreasing order of frequency, cataracts, keratoconjunctivitis sicca, glaucoma, discoid lesions of eyelids, episcleritis, retinopathy, vortex keratopathy, choroidopathy and retinal detachment, central retinal vein occlusion, and hydroxychloroquine-induced toxic maculopathy. Conclusions: It is advised that a multidisciplinary team for the diagnosis and treatment of SLE should regularly include the presence of an ophthalmologist.

Cogan’s Syndrome

Cogan’ syndrome is a multisystemic disorder characterized by the almost unfailing combination of inflammatory ocular disease and sensorineural hearing loss. In addition to constitutional symptoms, clinical features may also include cardiovascular, gastrointestinal, and neurological manifestations with variable frequency. Skin and mucous membranes, kidney and urogenital apparatus are rarely involved. The etiology remains undefined whereas an autoimmune pathogenesis is sustained by experimental and clinical observations. Given its rarity and clinical heterogeneity, the diagnosis is often delayed with possible progression to impairment of visual acuity and auditory dysfunction up to deafness. Therapy is based on the administration of glucocorticoids and immunosuppressive agents. Tumor necrosis factor-alpha blockers, the B-cell depleting monoclonal antibody rituximab, the anti-interleukin-6 receptor monoclonal antibody tocilizumab, and the immunomodulatory leflunomide have also been employed in single patients. Cochlear implantation can be used for hearing rehabilitation. Finally, stent-graft insertion and prosthetic aortic replacement can be extremely useful to correct the consequences of aortitis and large vessel aneurysms.

Bilateral acute angle-closure glaucoma as the presenting feature of a systemic leukocytoclastic vasculitis

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