08.05 系统性血管炎.pptVIP

Palpable Purpura Behcet’s Syndrome Behcet’s Syndrome Clinicopathologic entity Characterized by Recurrent episodes of oral and genital ulcers Iritis Cutaneous lesion Pathologic: leukocytoclastic venulitis Hepatitis B Virus-Associated Vasculitis Seen in 10% to 50% of polyarteritis nodosa cases Presents as a systemic vasculitis with abnormal liver function tests Tissue damage is due to immune complexes Therapy includes steroids, antiviral agents, and occasionally apheresis Wrap up: Don’t Miss It Viral infections can mimic many rheumatic and vasculitic syndromes Key associations Hepatitis B—polyarteritis nodosa Hepatitis C—cryoglobulinemia HIV—“seronegative” rheumatic syndromes Wrap up: General Concepts About Vasculitis Treatment Tissue damage with vasculitis requires early diagnosis and treatment Combinations of high-dose steroids and cytotoxic drugs are commonly used Effective treatment can improve outcome There is a delicate balance between treatment efficacy and toxicity Well-defined clinical outcomes are needed to guide the intensity and duration of treatment Wrap up: Points to Remember When a patient has a complex multisystem inflammatory picture—think vasculitis If a vasculitic disorder is considered, search for its cause Employ tests and biopsies when indicated, but remember to treat the patient, not the test Rapid diagnosis and treatment is often organ or lifesaving Consider viral associated rheumatic/vasculitis syndromes when the autoantibody results are not typical Thank you for your attention! 男性，58岁，已婚，黑龙江 主诉：反复腹痛1月余，加重半月 腹痛病史： 1月前开始间断发作腹部疼痛，局限于脐周 多发生于夜间，疼痛可忍受，无放射痛，与饮食无关 近半月患者自觉腹痛加重，呈持续发作，以脐周为甚,向腰部放射 疼痛剧烈不能忍受，尤以平卧位剧烈,坐位时疼痛稍减轻 * 影响脏器广泛且尤为严重，如颈动脉或椎动脉狭窄和闭塞，引起脑和头面部不同程度的缺血，如头昏、头痛、记忆力下降、视力下降/失明、失语、晕厥、偏瘫、昏迷，甚至死亡；锁骨下动脉、髂总动脉受累致上下肢缺血，表现为脉搏减弱或出现无脉症，肢体发冷，麻木，无力，酸痛，肌肉萎缩致不能提物或间歇性跛行；肾动脉狭窄可引起肾血管性高血压、肾衰竭；肠系膜血管狭窄可出现腹痛腹泻，便血，肠梗阻，严重者出现节段性肠坏死；冠状动脉炎症可发生心绞痛或心肌梗塞，累及心脏瓣膜引起瓣膜关闭不全，肺动脉受累致肺动脉高压，最终引发心力衰竭mortality has been reported to be as high as 35% at 5 yrs Type I only the br