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Takayasu arteritis (TAK) is a rare granulomatous vasculitis of unknown etiology that mainly affects the aorta and its major branches. The aim is to describe the clinical features, diagnostic procedures, pathogenesis, and management of TAK in a longitudinal cohort of patients recruited within a single region of southern Italy. The cohort included 43 patients who were diagnosed with TAK and followed up according to a standard protocol, in a collaboration between four university tertiary referral centers and a regional hospital. Clinical and imaging classification criteria were those established by the American College of Rheumatology. Thirty-five patients (81.4%) were female, and the mean age at disease onset was 32.6 (range 16-54) years. Angiographic assessment of the vascular involvement allowed disease classification in five different types. Clinical features ranged from constitutional symptoms in the early inflammatory stage of the disease to cardiovascular ischemic symptoms in the late, chronic stage. Noninvasive imaging techniques were employed to assess the extent and severity of the arterial wall damage and to monitor the clinical course and response to therapy. Medical treatment, based on pathogenetic insights into the roles of humoral and cell-mediated immune mechanisms, included glucocorticoids mostly combined with steroid-sparing immunosuppressive agents and, in patients with relapsing/refractory disease, biologic drugs. Significant clinical and angiographic differences have been detected in TAK patients from different geographic areas. Patients with life-threatening cardiovascular and neurologic manifestations as well as sight-threatening ophthalmologic signs and symptoms should be promptly diagnosed, properly treated, and closely followed up to avoid potentially severe consequences.

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: Intravitreal TAAC was relatively safe and effective in resistant cases of pseudophakic CME with a temporary beneficial effect on visual acuity and macular edema.

Rosanna Dammacco,1 Giovanni Alessio,1 Ermete Giancipoli,2 Patrizia Leone,3 Anna Cirulli,3 Leonardo Resta,4 Angelo Vacca,3 Franco Dammacco3 1Department of Ophthalmology and Neuroscience, University of Bari “Aldo Moro”, Medical School, Bari, Italy; 2Department of Biomedical Sciences, Ophthalmology Unit, University of Sassari, Sassari, Italy; 3Department of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro”, Medical School, Bari, Italy; 4Department of Emergency and Organ Transplantation, University of Bari “Aldo Moro”, Medical School, Bari, ItalyCorrespondence: Franco DammaccoDepartment of Biomedical Sciences and Human Oncology, University of Bari “Aldo Moro”, Medical School, Polyclinic, Piazza Giulio Cesare, 11, Bari 70124, ItalyTel +39 080 5478 863Fax +39 080 5478 820Email francesco.dammacco@uniba.itPurpose: Giant cell arteritis (GCA), a chronic vasculitis of the large and medium-sized arteries, affects people > 50 years of age. This study assessed the prevalence of visual manifestations and other clinical features at presentation in an Italian cohort of GCA patients. Recent advances in the pathophysiology, diagnosis, and therapy of GCA are also reviewed.Methods: This retrospective, single-center study conducted by the ophthalmology and internal medicine clinics of one university recruited 56 patients from 2005 to 2016 and followed them for 11– 54 months.Results: Ocular involvement was diagnosed in 19 patients (33.9%), with permanent vision loss in 19.6% (7.1% of the cohort with bilateral vision loss). Arteritic anterior and posterior ischemic optic neuropathy were diagnosed in 11 patients (57.9%) and 1 patient (5.3%), respectively, cotton wool spots in 3 patients (15.8%), central retinal artery occlusion in 2 patients (10.5%), and anterior segment ischemia and multifocal choroidal ischemia in 1 patient each (5.3%). Polymyalgia rheumatica was associated with GCA in 44.6% of the patients. The most common extra-ocular manifestation was constitutional symptoms (82.1% of the patients). Large-vessel involvement, including of the ascending aorta, aortic arch, and left axillary artery, was diagnosed by magnetic resonance or computed tomography (CT) angiography and 18FDG positron emission/CT. Glucocorticoids (GCs) remain the standard-of-care worldwide, but methotrexate, provided as a steroid-sparing drug in 41% of the patients, resulted in earlier tapering, a lower cumulative dose of GCs, and a lower rate of relapse. Among the combinations of GCs and immunosuppressive drugs proposed to treat GCA, only tocilizumab has effectively induced and maintained disease remission.Conclusion: According to our data and literature reports: a) GCA is a systemic disease; b) its diagnosis is expedited by the adjunct use of imaging techniques; c) insights into the pathogenesis of GCA may allow an improved, differentiated therapeutic approach.Keywords: giant cell arteritis, ocular manifestations, diagnostic imaging, glucocorticoids, pathogenetic advances, tocilizumab