EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis.

In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life-threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

Acute optic neuropathy in Behçet disease.

PURPOSE: To report a patient with Behcet disease presenting with acute optic neuropathy. METHODS: A 47-year-old man was admitted to the authors' clinic owing to sudden visual loss in the left eye. Ophthalmologic and systemic examinations were performed. RESULTS: Visual acuity was 4/10 in the left eye. Fundus examination demonstrated swollen optic disc with blurred margins. There was relative afferent pupil defect in the same eye. Mega-dose corticosteroid treatment was started with the diagnosis of acute optic neuropathy. Ocular findings resolved within 2 weeks. CONCLUSIONS: Behçet disease rarely presents with acute optic neuropathy.

Acute optic neuropathy in Behet disease.

PURPOSE: To report a patient with Behet disease presenting with acute optic neuropathy. METHODS: A 47-year-old man was admitted to the authors clinic owing to sudden visual loss in the left eye. Ophthalmologic and systemic examinations were performed. RESULTS: Visual acuity was 4/10 in the left eye. Fundus examination demonstrated swollen optic disc with blurred margins. There was relative afferent pupil defect in the same eye. Mega-dose corticosteroid treatment was started with the diagnosis of acute optic neu-ropathy.Ocular findings resolved within 2 weeks. CONCLUSIONS: Behet disease rarely presents with acute optic neuropathy. (Eur J Ophthal-mol 2004; 14: 578-80).

Limbal autograft and allograft transplantations in patients with corneal burns.

AIM: To investigate and compare the surgical outcomes of limbal autograft and limbal allograft transplantations in patients with corneal burns. METHODS: In total, 20 patients (n=22 eyes) with chemical burn and two patients (n=2 eyes) with thermal burn were included in this study. Limbal autograft or limbal allograft transplantation surgery was performed in all patients. HLA-typing was tested before allograft surgeries. Limbal allografting was performed in all eyes using donor tissue from live relatives. Systemic cyclosporine A was administered for immunosuppression. RESULTS: The corneal surface was successfully reconstructed in all eyes (100%) after limbal autografting, two eyes required additional amniotic membrane transplantation and one eye required allografting. The mean follow-up period for limbal autografts was 13.9 +/- 7.0 months. Limbal allografting failed to reduce corneal vascularity and opacification in five (55.6%) eyes and was successful only in four (44.4%) eyes (mean follow-up 16.2 +/- 11.2 months) (P=0.002). In all, 15 eyes undergoing limbal autografting completed re-epithelialization of the cornea at a mean of 35.6 +/- 60.2 days. The mean epithelial healing time in nine eyes undergoing limbal allografting was 13.0 +/- 7.3 days (P=0.525). After limbal autografting, functional vision (> or =1/10) was attained in 12 (80%) eyes. Only one eye (11.1%) achieved functional vision after limbal allografting (P=0.036). Penetrating keratoplasty was performed in three patients following limbal allografting. No cyclosporine-associated side effects were observed. CONCLUSIONS: Limbal autograft transplantation is an effective and safe procedure for unilateral corneal burns. It seems that limbal allograft transplantation is better combined with penetrating keratoplasty for a better visual outcome and higher graft survival rate. Systemic immunosuppression seems to be necessary for limbal allografts even in the presence of HLA-matched donor tissues.