Nathalie Butel

Small vessel involvement in Takayasu's arteritis

OBJECTIVES To describe the small retinal and systemic vessel involvement in Takayasus arteritis. METHODS We described 3 patients with Takayasus arteritis and small retinal vessel occlusion seen in our department between 2004 and 2011. We performed an extensive literature review and provided a global analysis of small retinal vessel involvement in Takayasu arteritis (i.e., total number of patients analyzed=9). RESULTS Seven patients had small retinal artery occlusion, and two had venous involvement. Four cases were inaugural of the disease (44.4%). Takayasus arteritis was extended (Type V) in the majority of patients presenting with small retinal vessel occlusion (5/9, 55.6%), and 8/9 reported cases (88.9%) presented with involvement of the supra-aortic branches. Immunosuppressive regimen allowed an improvement in 5/9 patients and stabilization in 1/9, but the situation worsened in 3/9 patients. The visual outcome was severe, and 3/9 patients (33.3%) experienced irreversible blindness. CONCLUSION Occlusion of small retinal vessels is a rare and severe microcirculatory complication in Takayasus arteritis, as well as necrotizing cutaneous vasculitis or myocarditis. Small retinal vessel involvement can be inaugural of the disease and seriously impact the visual prognosis in TA patients.

Anakinra in the Treatment of Patients with Refractory Scleritis: A Pilot Study

ABSTRACT Purpose: This study aimed to evaluate the safety and efficacy of anakinra for severe and refractory scleritis. Methods: Ten patients with severe (i.e. at least 2 ocular relapses per year despite treatment) and refractory [i.e. at least to one disease modifying antirheumatic drugs (DMARDS)] scleritis were treated with anakinra (100 mg/day subcutaneously). Scleritis was associated with inflammatory systemic diseases in 60% of cases. The remission rate defined the primary outcome. Results: Ninety percent of patients were complete responders with a mean follow-up of 19.4 months after starting anakinra. The corticosteroids daily dose decreased from 18.3 ± 4.1 mg to 4.2 ± 4.9 mg, (p < 0.05), at initiation of anakinra and at end of follow-up, respectively. Associated immunosuppressants were stopped in all cases except one. Side effects were observed in 4 patients who did not need anakinra withdrawal. Conclusions: This pilot study suggests the efficacy of anakinra in patients with refractory scleritis.

Fluorescein and OCT Angiography Features of Takayasu Disease

ABSTRACT Purpose: Evaluate OCT (optical coherence tomography) angiography (OCTA) features in patients with Takayasau disease. Methods: The OCTA was analyzed to evaluate perifoveal anastomatic capillary arcade disruption, microaneurysms. The foveal avascular zone (FAZ) was measured for superficial (SCP) and deep capillary plexus. Results: There were 26 eyes included. In OCTA, 11 eyes presented ruptures of the perifoveal anastomotic capillary arcade in SCP. A total of 5 had microaneurysms. The average SCP FAZ was increased to 0.34 mm2 in Takayasu compared to 0.27 mm2 in control patients. Conclusion: Fluorescein angiography is the gold standard to describe Takayasu retinopathy ischemic signs. Macular abnormalities are uncommon in Takayasu patients as retinopathy signs are mostly located in peripheral retina. This study reveals that most of our patients present an enlargement of the FAZ then highlights the relevance of OCTA to evaluate macular ischemia as a complement to usual retina global study in fluorescein angiography.