丙基硫氧嘧啶致抗中性粒细胞胞质抗体阳性小血管炎二例

丙基硫氧嘧啶 (PTU)是硫脲类抗甲状腺药 ,是临床最常用的抗甲状腺功能亢进药物之一。抗中性粒细胞胞质抗体(ANCA)是原发性小血管炎的血清学诊断工具。近年来发现有少数服用PTU的甲状腺功能亢进患者临床上出现了小血管炎的表现 ,并且在这些患者血清中也可检测到ANCA。现将我院近年发现的 2例PTU相关的ANCA阳性小血管炎报道如下。例 1 :女性 ,6 0岁。因皮疹、关节痛半年 ,泡沫尿 1个月入院。患者 3年前查血小板减低 ,骨髓细胞学示“巨核细胞增多伴成熟障碍” ,查自身抗体均阴性 ,临床诊断为特发性血小板减少性紫癜 (ITP)。予环孢菌…     

抗甲状腺药物治疗彩超不同声像特征原发性甲亢的疗效观察

目的探讨抗甲状腺药物治疗两种彩超不同声像特征原发性甲亢的疗效。方法随机选择均匀回声型(均匀组)及不均匀回声型(不均匀组)原发性甲亢患者各31例,两组均用抗甲状腺药物治疗,于治疗前后检测甲状腺功能、甲状腺抗体,同时对甲状腺进行彩超检查,观察甲状腺声像、彩色血流分布,检测甲状腺大小及上动脉血流频谱参数。结果两组治疗前各检测指标差异无统计学意义(P>0.05),经抗甲状腺药物治疗后两组疗效比较差异有统计学意义(P<0.01)。均匀回声型治疗效果明显优于不均匀回声型(P<0.01),不均匀回声型组其危险度高于均匀回声型组(OR=12.923,P=0.000,95%CI:3.225～51.781)。结论抗甲状腺药物对原发性甲亢均匀回声型疗效较好,对不均匀回声型疗效较差,这对临床治疗方法的选择有重要的参考意义。     

甲状腺功能亢进及抗甲状腺药物与肝损害

甲状腺功能亢进(甲亢)、抗甲状腺药物均可引起肝损害。甲亢引起肝损害比较常见,可分为肝酶的升高和黄疸,其对肝功能损害的原因是多方面的,与甲状腺激素的直接毒性作用、高代谢、心力衰竭及免疫等因素有关;治疗原则以控制甲亢为主,甲亢治愈后,肝功能大多数可恢复正常。抗甲状腺药物引起肝损害的诊断通常采用排除法,其发病机制目前主要认为与机体的异质性反应有关;亚临床肝损害时不需停用抗甲状腺药物,如果肝损害显著,立即停药是治疗的关键。     

应加强对抗甲状腺药物治疗所致粒细胞缺乏症的认识

治疗甲状腺功能亢进症(甲亢)的药物,主要有甲巯咪唑(methimazole,MMI)及丙硫氧嘧啶(propyl-thiouracil,PTU).不良反应包括皮肤瘙痒、皮疹、白细胞减少、血管炎、药物性肝病等.当外周血中性粒细胞计数绝对值＜0.5×109/L时称为粒细胞缺乏症,易伴发各种感染,可出现败血症危及生命.因此,重视抗甲状腺药物(antithyroid drugs,ATD)所致的粒细胞缺乏症有重要意义.     

抗甲状腺药物的免疫抑制作用

抗甲状腺药物有免疫抑制作用。其机制为改善T淋巴细胞亚群,使甲状腺自身抗体滴度下降;通过清除和抑制氧自由基及细胞因子的产生、抑制甲状腺自身抗原的递呈而起免疫抑制作用;还可以引起T淋巴细胞凋亡,并通过以上机制使甲亢患者实现临床和免疫缓解的目标。     

抗甲状腺药物治疗——Graves病治疗的首选

Graves病是甲亢中最常见的类型,是一种与自身抗体(TRAb)相关的自身免疫性疾病。其具体机制还不十分明确。Graves病治疗有抗甲状腺药物(ATD)、放射性碘(RAI)和手术3种传统的治疗方法。惟有ATD治疗是针对免疫因素,最终使TRAb转阴而治愈甲亢。中外文献反复强调ATD治疗是所有Graves甲亢的基础治疗,也是世界各地(除美国外)治疗甲亢的首选药物,其不破坏甲状腺滤泡结构,安全有效,药物作用可逆,不会造成永久性甲减。ATD治疗不仅能单选首选,还可作为手术和同位素治疗前的优选方案。美国ATA主席Cooper教授(NEJM,2005)提出了Graves甲亢的治疗路径可以给予我们一些临床指导。     

咖啡酸片治疗Graves病患者抗甲状腺药物所致粒细胞减少症的疗效观察

Graves病是临床上常见的疾病,在应用甲巯咪唑、丙基硫氧嘧啶等抗甲状腺药物治疗甲状腺功能亢进症(甲亢)的过程中,部分患者可引起粒细胞减少,严重者可导致粒细胞缺乏,甚至危及生命.我们采用咖啡酸片治疗抗甲状腺药物所致的粒细胞减少症,取得了很好的临床疗效.现报道如下.     

抗甲状腺药物致粒细胞缺乏症6例临床分析

目的探讨抗甲状腺药物致粒细胞缺乏症(粒缺)的临床特点。方法回顾性分析1994-01~2004-05北京中日友好医院收治的6例抗甲状腺药物致粒缺患者的临床资料。结果6例粒缺患者均为女性,年龄17~65岁,其中5例由他巴唑所致,1例丙基硫氧嘧啶所致,5例在服药后2个月内发生粒缺,1例在半年后发生。临床表现为突发高热咽痛。5例治愈,粒细胞在停药后2周内恢复,1例死亡。结论粒缺为严重的不良反应,不可预测,在甲亢患者开始药物治疗的2个月内,应高度警惕粒缺的发生。     

抗甲状腺药物联用甲状腺素治疗老年甲状腺机能亢进症

抗甲状腺药物(ATD)的免疫抑制作用已逐渐被人们所认识，ATD通过抑制Graves disease(GD)的免疫反应来提高甲状腺机能亢进症(甲亢)的缓解率。他巴唑治疗后是否联用甲状腺素(L-T4)的问题争议许多，国内尚未见老年甲亢患者联用L-L4的报道。我们对ATD联用L-L4治疗老年甲亢患者进行临床观察。     

丙基硫氧嘧啶致抗中性粒细胞胞浆抗体阳性多浆膜腔积液1例

丙基硫氧嘧啶 (PTU)是硫脲类抗甲状腺药,是临床最常用的抗甲状腺药物之一。抗中性粒细胞胞浆抗体 (AN CA)是原发性小血管炎的重要血清免疫学诊断依据 [1]。现将我院确诊的PTU所致ANCA阳性病 1例进行临床分析,并结合文献加以讨论。1　临床资料患者女性, 30岁。因怕热、心慌、多汗 3年,不规则发热伴胸闷、胸痛、气急 3个月,于 2004-04-03收住我院内分泌科。患者 3年前因怕热、心慌、突眼、甲状腺肿大,血三碘甲状腺原氨酸(T3 )、四碘甲状腺原氨酸 (T4 )增高、促甲状腺激素(TSH)下降在我院门诊诊断为甲亢,用他巴唑每天 30mg, 1周后…     

Central nervous system vasculitis caused by propylthiouracil therapy: a case report and literature review.

Antineutrophil cytoplasmic antibodies (ANCA) are associated with vasculitis, including vasculitis induced by drugs such as the thionamides. The affected organ systems in thionamide-induced vasculitis have been primarily renal, musculoskeletal, and dermatologic. We describe the first case of thionamide-induced central nervous system vasculitis presenting as confusion, with complete resolution after discontinuation of propylthiouracil. We review the literature and summarize 42 additional cases of thionamide-induced ANCA-positive vasculitis since 1992. Propylthiouracil was responsible in 93% of cases and the predominant ANCA pattern on immunofluorescent staining was perinuclear (p-ANCA). Clinical improvement occurred after drug discontinuation in 93%, steroid therapy was used in some cases. The mean duration of treatment with thionamides was 35 months prior to presentation. Long-term medical treatment with thionamides for hyperthyroidism may increase the risk of this severe side effect.     

Systemic lupus erythematosus vasculitis: A current therapeutic overview

Opinion statement The development of systemic lupus erythematosus (SLE) vasculitis is of prognostic value. The earlier the vasculitis is treated, the better the prognosis for SLE. Cutaneous vasculitis is common in SLE, whereas visceral vasculitis is rare. Skin SLE vasculitis is successfully treated with antimalarials, but its discontinuation may result in an SLE flare even among patients in remission. When visceral SLE vasculitis is encountered, or when a disease state is perceived to be life-threatening, a more aggressive therapy is warranted. A combination of medications, plasmapheresis, and intravenous immunoglobulin treatment, along with high-dose steroids and cytotoxic drugs, are typically employed in the treatment of severe SLE vasculitis. Finally, patients with SLE vasculitis may benefit from a number of autoimmune disease therapies currently under investigation, such as switching cytokine responses from Th1 to Th2, and the manipulation of toll-like receptors, chemokines, and FcR receptors. Specific B-cell therapies (eg, anti-Blys, B-cell depletion) may also emerge as potential treatments for SLE vasculitis.     

A large ulcer and cutaneous small-vessel vasculitis associated with syphilis infection

Cutaneous vasculitis (CV) is a condition with cutaneous manifestations and possible systemic involvement. The causative factors or associated diseases are usually drugs, infection, collagen vascular disease, or malignancy. Syphilis as a cause of cutaneous vasculitis is rare. We report the case of a large cutaneous ulcer and small-vessel vasculitis associated with syphilis infection. We suggest that in apparently idiopathic CV or a chronic ulcer refractory to treatment, screening should be performed to detect any underlying infection such as syphilis. It is important to have a rapid and accurate diagnosis because the lesions are very contagious, but may be rapidly and completely cured by early administration of antibiotic treatment.     

Rapidly progressive glomerulonephritis with glomerular crescent formation in rheumatoid arthritis

Summary Systemic vasculitis is a well-known complication of rheumatoid arthritis (RA). Rapidly progressive glomerulonephritis is commonly associated with vasculitis syndromes but rarely found in RA. This report describes two RA patients with complicating systemic vasculitis who developed rapidly progressive glomerulonephritis with glomerular crescent formation. One recovered after treatment with corticosteroids and cytostatic drugs and the other died of severe systemic vasculitis despite similar therapy.     

血管炎生物制剂的研究进展

血管炎的具体病种很多、病情复杂。有相当数量的血管炎患者在接受常规治疗后会出现病情的加重或反复。近些年,生物制剂单用或与其他药物联用在治疗难治性血管炎和控制血管炎复发方面发挥了一定的优势。2012年欧洲抗风湿病联盟将血管炎分为7类,本文主要对近几年治疗这7类血管炎的生物制剂的研究进展作一综述,希望能为临床医师提供参考。     

Ophthalmologic manifestations of systemic vasculitis: report of six cases and review of the literature

PURPOSE: Study of characteristics of ocular involvement in systemic vasculitis. METHODS: We describe six cases of systemic vasculitis with ocular involvement observed between 1992 and 2000. These cases are compared with those reported in the literature. RESULTS: Our patients suffered from Wegener's granulomatosis (four cases), periarteritis nodosa and Churg-Strauss syndrome. Ocular manifestations were conjunctivitis, scleritis, orbital pseudotumor, optic neuritis and extraocular muscle palsy. These manifestations are similar to those reported in the literature. Their treatment requires steroids and immunosuppressive drugs. In one of our cases, intravenous immunoglobulins were effective in controlling an optic neuritis. CONCLUSION: Ocular involvement in systemic vasculitis may concern any orbital structure. It usually occurs during the course of vasculitis but may be one of its first manifestations. It requires an appropriate treatment to prevent ophthalmic complications and especially blindness.     

Primary cutaneous small vessel vasculitis

Disorders associated with cutaneous vasculitis include numerous well-described etiologies. Primary cutaneous vasculitis limits discussion to primary leukocytoclastic vasculitis, essential mixed cryoglobulinemia, urticarial vasculitis, Henoch-Sch?nlein purpura, and erythema elevatum diutinum. Although the therapeutics for these disorders are based on limited data, we attempt to construct a consensus opinion on the management of primary cutaneous vasculitis. Therapy of primary cutaneous vasculitis is indicated for symptomatic or systemic involvement, because cutaneous small vessel vasculitis is frequently a self-limited, single episodic disease. Conservative, symptomatic treatment includes leg elevation, warming, antihistamines, and nonsteroidal anti-inflammatory drugs. For mild recurrent disease, colchicine, dapsone, and prednisone are first-choice agents. Systemic or severe cutaneous disease requires more potent immunosuppression (eg, prednisone, azathioprine, or mycophenolate mofetil). Plasmapheresis/plasma exchange and intravenous immunoglobulin are viable considerations for refractory disease, but are cumbersome and expensive modalities. There is insufficient evidence to advocate the use of new biological or monoclonal antibody therapies in primary cutaneous vasculitis.     

白细胞碎裂性血管炎23例临床分析及文献复习

目的总结皮肤白细胞碎裂性血管炎的病因、临床表现、诊断与治疗，以进一步提高对皮肤小血管炎的认识。方法回顾分析北京协和医院1990-2001年经临床及皮肤活检病理证实为白细胞碎裂性血管炎的住院患者的病因、临床表现、诊断与治疗。结果23例患者中超敏性血管炎(HV)20例，其中1例2．5年后确诊为韦格纳肉芽肿病(WG)，荨麻疹性血管炎2例，系统性红斑狼疮(SLE)1例。20例HV有较独特的临床特点。23例患者中男性14例，女性9例，平均年龄28．7岁，以多形性皮肤损害为主要表现，双下肢尤其小腿伸侧受累为主，经中等剂量激素和，或免疫抑制剂治疗均获缓解。结论白细胞碎裂性血管炎可为多种疾病的表现。狭义的皮肤白细胞碎裂性血管炎即HV，多由感染和／或药物引起，临床多见于青壮年，以多形性皮疹为主要表现，系统性损害少，预后好。     

Propylthiouracil-induced autoimmune syndromes: 11 case report

The objective of this study was to report 11 cases of propylthiouracil (PTU)-induced autoimmune syndromes. We describe the clinical presentation, course, and outcome of 11 patients and compare clinical features between PTU-induced lupus and PTU-induced vasculitis. Of our 11 patients, 7 patients had vasculitis and 4 patients had lupus. Patients with vasculitis were olderand had a longer duration of treatment in comparison with lupus. P-ANCA were predominantly found in PTU-induced vasculitis, but also found in lupus, while ANA and anti-dsDNA were often found in lupus. Some difference of renal and pulmonary involvement was often found between PTU-induced vasculitis and lupus. Most of patients needed steroids or immunosuppressive drugs. Vasculitis in the cases of PTU-induced autoimmune phenomena is often found than lupus. P-ANCA in both PTU-induced lupus and vasculitis can all present, but there are differences in clinical and outcome features to diagnosis.     

Immune disorders and rheumatologic manifestations of viral hepatitis

Infection with hepatotropic viruses is not limited to the liver and can lead to the development of various immunological disorders (the formation of cryoglobulins, rheumatoid factor, antinuclear antibodies, autoantibodies specific for autoimmune hepatitis and primary biliary cholangitis, and others), which can manifest as glomerulonephritis, arthritis, uveitis, vasculitis (cryoglobulinemic vasculitis, polyarteritis nodosa, Henoch-Schonlein purpura, isolated cutaneous necrotizing vasculitis), and other rheumatologic disorders, and be a trigger for the subsequent development of autoimmune hepatitis and primary biliary cholangitis. A further study of the association between autoimmune liver diseases and hepatotropic virus infection would be useful to assess the results of treatment of these associated diseases with antiviral drugs. The relationship of these immune disorders and their manifestations with hepatotropic viruses is best studied for chronic hepatitis B and C. Only isolated cases of these associations are described for hepatitis A. These links are least studied, and are often controversial for hepatitis E, possibly due to their relatively rare diagnoses. Patients with uveitis, glomerulonephritis, arthritis, vasculitis, autoimmune liver diseases should be tested for biomarkers of viral hepatitis, and if present, these patients should be treated with antiviral drugs.