多发性大动脉炎(takayasu arteritis,TA)是一种主要累及主动脉及其重要分支,包括冠状动脉或肺动脉的慢性、肉芽肿性血管炎,是儿童时期最常见的大血管炎[1]。据Kerr G S等[2]报道约30%TA于儿童时期起病。由于缺乏特异的临床表现,儿童TA...多发性大动脉炎(takayasu arteritis,TA)是一种主要累及主动脉及其重要分支,包括冠状动脉或肺动脉的慢性、肉芽肿性血管炎,是儿童时期最常见的大血管炎[1]。据Kerr G S等[2]报道约30%TA于儿童时期起病。由于缺乏特异的临床表现,儿童TA延迟诊断时间约为成人的4倍[2],病死率可高达35%[1]。近年来,对儿童TA研究不断深入,对儿童TA的认知也不断加深。本研究就儿童TA的流行病学、发病机制、临床特征、活动性评估、诊断及治疗进展方面作一综述。展开更多
Case report: A 34- year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiret...Case report: A 34- year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiretinal neovascular membrane in the LE. Topical and systemic oral steroids and antiviral medication were prescribed. One year later, optic disc hyperaemia and swelling and macular oedema became apparent in the LE. Pulsed intravenous steroids were administered for when a nasal septum perforation and vitreous haemorrhage in the LE diagnosed. The eye was enucleated 3.5 years after the initial complaint. Necrotizing granulomatous tissue replacing the sclera and subconjunctival granulomatous tissue were observed. Six months later, oedema and neovascularization of the right optic disc were observed and cyclophosphamide was started,with regression of the clinical signs. No systemic abnormalities have so far become apparent. Discussion: Posterior scleritis is most often observed in patients with no signs of associated systemic autoimmune disease. The diagnosis in this case is most probably one of Wegener’ s granulomatosis (WG). InWG, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis. Eye involvement with WG has been reported in up to 58% of cases. Evaluation of the scleritis patient should include a detailed medical history, chestX-rays, blood tests, autoantibody serology and analysis of urinary sediment. Cyclophosphamide is the treatment of choice for patients with ocular manifestations of WG, polyarteritis nodosa or rheumatoid arthritis, either alone or in combination with systemic steroids. Visual loss is expected in 85% of individuals with severe necrotizing posterior scleritis.展开更多
Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology ...Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset of corticosteroid dependency, iatrogenic Cushing’ s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.展开更多
文摘多发性大动脉炎(takayasu arteritis,TA)是一种主要累及主动脉及其重要分支,包括冠状动脉或肺动脉的慢性、肉芽肿性血管炎,是儿童时期最常见的大血管炎[1]。据Kerr G S等[2]报道约30%TA于儿童时期起病。由于缺乏特异的临床表现,儿童TA延迟诊断时间约为成人的4倍[2],病死率可高达35%[1]。近年来,对儿童TA研究不断深入,对儿童TA的认知也不断加深。本研究就儿童TA的流行病学、发病机制、临床特征、活动性评估、诊断及治疗进展方面作一综述。
文摘Case report: A 34- year-old man presented with anterior scleral thinning in the right eye (RE) and a painful nodular scleritis in the left eye (LE). Fundus examination showed a healed vasculitis and an inferior epiretinal neovascular membrane in the LE. Topical and systemic oral steroids and antiviral medication were prescribed. One year later, optic disc hyperaemia and swelling and macular oedema became apparent in the LE. Pulsed intravenous steroids were administered for when a nasal septum perforation and vitreous haemorrhage in the LE diagnosed. The eye was enucleated 3.5 years after the initial complaint. Necrotizing granulomatous tissue replacing the sclera and subconjunctival granulomatous tissue were observed. Six months later, oedema and neovascularization of the right optic disc were observed and cyclophosphamide was started,with regression of the clinical signs. No systemic abnormalities have so far become apparent. Discussion: Posterior scleritis is most often observed in patients with no signs of associated systemic autoimmune disease. The diagnosis in this case is most probably one of Wegener’ s granulomatosis (WG). InWG, the diagnosis is based on necrotizing granulomas of the respiratory tract, generalized focal necrotizing vasculitis and focal necrotizing glomerulonephritis. Eye involvement with WG has been reported in up to 58% of cases. Evaluation of the scleritis patient should include a detailed medical history, chestX-rays, blood tests, autoantibody serology and analysis of urinary sediment. Cyclophosphamide is the treatment of choice for patients with ocular manifestations of WG, polyarteritis nodosa or rheumatoid arthritis, either alone or in combination with systemic steroids. Visual loss is expected in 85% of individuals with severe necrotizing posterior scleritis.
文摘Background. Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. Case report. A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset of corticosteroid dependency, iatrogenic Cushing’ s syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. Discussion. Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.
