显微镜多血管炎5例分析

目的　提高对显微镜多血管炎临床特征的认识。方法　报道 5例显微镜多血管炎 ,并进行文献复习。结果　 4例以发热 ,3例以关节肿痛 ,1例以腹痛、腹泻和胃肠道出血为起病症状。 5例都有肾损害 ,3例肺受累。 5例血沉都增快 ,C反应蛋白增高 ,有不同程度贫血和低蛋白血症 ,补体都正常 ,核周型 抗中性粒细胞胞质抗体 (p ANCA)和髓过氧化物酶 抗中性粒细胞胞质抗体 (MPO ANCA)都阳性。结论　不明原因的长期发热、关节肿痛伴肺肾受累 (尤其肾功能急剧恶化的肾小球肾炎而无肾性高血压 ,不明原因的咯血 ) ,补体正常 ,p ANCA和MPO ANCA阳性 ,排除继发性小血管炎 ,应诊断显微镜多血管炎     

Association of cerebral vasculitis with a lupus anticoagulant. A case with brain pathology

Summary The authors report the case of a sixty-five year old woman initially suffering from a thrombocytopenia. The patient was diagnosed as having an autoimmune disease with a lupus anticoagulant, positive antinuclear antibodies and negative anti-DNA antibodies. She then developed an encephalopathy which was fatal despite corticosteroids. Brain pathology revealed a vasculitis with some giant cells, evoking a granulomatous angiitis of the central nervous system. These clinical and biological features suggest a systemic lupus erythematosus with vasculitis or a primary granulomatous angiitis of the central nervous system. Taking into account the clinical manifestations and the presence of a lupus anticoagulant, we finally preferred to identify it as a primary antiphospholipid antibodies syndrome, despite absence of anticardiolipin antibodies. Contrary to thrombosis, vasculitis is rarely associated with an anticardiolipin antibody or a lupus anticoagulant. However, vasculitis in the course of primary antiphospholipid antibodies syndrome has been reported previously as in this case report.     

Die primäre Angiitis des ZNS (PACNS)

Primary angiitis of the CNS (PACNS) is a rare inflammatory disease affecting middle-aged patients. The angiitis is focal and segmental in distribution, involving small and medium-sized leptomeningeal and intracranial vessels. The most frequent presenting symptoms are headaches, focal neurologic deficits, and confusion. In addition, aphasia, neuropsychological deficits, and seizures may occur. The paper presents a review of the literature. In addition, a typical case of PACNS is presented. This case demonstrates that diagnosis of PACNS is difficult and brain biopsy is essential to confirm it.     

原发性中枢神经系统血管炎的临床及影像学分析

目的探讨原发性中枢神经系统血管炎的临床和影像学表现,以明确诊断要点。方法临床或病理证实的中枢神经系统血管炎8例,其中男性3例,女性5例。年龄从19～73不等,平均年龄40．3岁。全部行MRI检查,其中增强4例;8例行MRA检查,分析所有病例的临床资料和MR片。结果8例PACNS中,8例位于幕上,以额叶及顶叶多见,其中3例为双侧多发;幕下4例位于脑干及脊髓。影像学上,多数病灶边界模糊,与相应血管分布区一致,累及灰质和自质,但以白质为主,可有水肿,增强后有不同程度增强,呈斑片样或脑回样,甚至呈结节样,类似肿瘤。血管成像可以发现血管炎的征象。结论原发性中枢神经系统血管炎的临床和影像学表现多样,MRI具有一定价值,MRA及DSA对诊断帮助较大,确诊需要活检病理证实。     

Inflammatory bowel disease (Crohn's disease) in a Spanish patient with pars plana exudates: Report of a new case and review of the literature

Several ocular manifestations have been found in Crohn's disease patients, most often affecting the anterior segment. This paper presents the case of a young woman with pars plana exudates in whom Crohn's disease was later diagnosed. To the authors' knowledge, this is only the second report of Crohn's disease and concomitant pars plana exudates.     

中枢神经系统原发性血管炎(附9例报告及文献复习)

目的　探讨中枢神经系统原发性血管炎 (PACNS)的临床、影像学及病理特点。方法　回顾分析 9例诊断为中枢神经系统原发性血管炎患者的临床表现、MRI、血管造影、病理活检以及治疗转归情况并结合文献分析其特点。结果　PACNS患者呈急性或亚急性起病 ,病程可以为进展性或缓解复发交替 ,临床表现以头痛、智能减退、偏瘫及癫痫样发作为主。实验室检查无特殊发现。MRI以大脑半球长T1长T2 信号的类圆形病变为主 ,多无占位效应 ,可见强化。病理检查以淋巴细胞性血管炎为主。 80 %患者应用皮质类固醇激素和免疫抑制剂联合治疗 6个月 ,病情控制预后良好。结论　PACNS临床表现多样 ,通过临床、影像学以及病理活检等联合应用可进行生前诊断。皮质类固醇激素和免疫抑制剂联合治疗可控制病情     

Systemic necrotizing angiitis with asthma: Causes and precipitating factors in 43 cases

Causes and precipitating factors for systemic necrotizing angiitis (NA) with asthma were sought in 43 patients, focusing on a history of vaccination and desensitization. Mean age of patients was 43.2 years. Diagnosis was based on histopathologic findings in 25 patients, arteriography in 2, and clinical criteria in 16. History of allergic manifestations (asthma, rhinitis, eczema, urticaria) was present in the family of 19 patients. Forty-two patients presented with asthma before development of NA and 23 of them were treated with steroids. Nineteen subjects gave a history of desensitization and 5 of vaccination in the 4 weeks preceding the disease. The main symptoms of NA were asthma in 43, fever in 25, weight loss in 31, peripheral neuropathy in 29, cutaneous signs in 25, digestive signs in 16 (abdominal pain, digestive bleeding, bowel perforation), noninfectious pneumopathy with pulmonary infiltrates in 33. Eosinophilia was 8,212±6,214/mm³. Antigen HBs was found in 2 of 30 patients. Prognosis of NA with asthma was good in 15 patients who recovered completely from the disease. Seven patients died and the other patients improved but remained under treatment. The survival curve showed that 75% of patients were alive after 60 months. Our findings suggest that different causes can be considered responsible for NA, and that, in cases of NA with asthma, there is reason to consider vaccination and desensitization as precipitating factors.     

Primary angiitis of the spinal cord presenting as a conus mass: long-term remission

BACKGROUND: Primary angiitis of the central nervous system (CNS) is a rare vasculitic disorder that typically involves the brain and, less frequently, the spinal cord without involvement of the blood vessels outside the CNS. CASE DESCRIPTION: We present a case of a 52-year-old woman who developed a conus syndrome linked to an enhancing mass of her lower thoracic spinal cord, lumbar cord, and conus. Spinal cord biopsy performed for diagnostic purposes in the setting of progressive neurological deficit confirmed angiitis of the spinal cord. Therapy with steroid and cyclophosphamide was associated with long-term (3 years) clinical and imaging remission of the lesion. CONCLUSIONS: The prognosis of primary CNS angiitis is dismal with most cases progressing to death. Long-term remission is unusual. Aggressive therapy with steroid and cytotoxic agents may improve survival.     

Lymphomatoid granulomatosis with primary manifestation in the skeletal muscular system

Summary Lymphomatoid granulomatosis (LYG) has been recognized as a peculiar disease which primarily affects the lungs and occasionally the upper respiratory tract. Typical histopathologic lesions present features of a nonneoplastic lymphoproliferative disorder associated with necroses of the infiltrated tissues and with angiodestructive processes. The authors observed a peculiar case in which lesions typical for LYG first developed in the striated muscles. In this case multiple tumorous muscular infiltrates and affections of the pharynx, larynx, paranasal sinuses, and orbita preceded the infiltration of the lung, which was first recognized 6 months after the first muscular infiltrate and 2 months before death.Because primary muscular manifestation of LYG has never been reported, this case is remarkable both for its unusual manifestation and for its striking irresponseveness towards therapy. Clinical and experimental data suggest impairment of T-cell functions.We acknowledge the financial support provided by the Fonds Kampf dem Krebs