抗磷脂综合征(antiphospholipid syndrome,APS)是自身免疫介导的,以反复动静脉血栓形成、习惯性流产、持续抗磷脂抗体(antiphospholipid antibody,aPL)阳性为特征的一组临床综合征[1].其中有1％的患者呈现爆发性的多发小血管血栓形成、出现多器官受累的临床表现,称为灾难性抗磷脂综合征(catastrophic antiphospholipid syndrome,CAPS)[2].欧洲抗磷脂论坛于2000年建立了CAPS注册系统,用于登记全球自1992年至今的CAPS患者,并定期发布相关临床数据的分析.现分享1例CAPS患者诊治经验,探讨其临床特征及诊治思路,以期提高该病的早期诊断率并降低该病的死亡率.

There are several therapeutic strategies available for the treatment of an acute gout attack and the prevention of recurrent gout flares, and they include nonsteroid anti-inflammatory drugs. This prospective study was aimed at evaluating the efficiency and safety of diacerein in combination with febuxostat on urate control, global assessments of disease activity, self-monitored gouty acute flare times, inflammatory markers, and clinical symptoms associated with their life quantity in patients with refractory gout. A total of 64 patients with refractory gout were sequentially recruited and prescribed with oral febuxostat alone or febuxostat plus diacerein daily for 12 weeks. The intensity of joint pain, numbers of acute flare, disease activity and the levels of serum amyloid A, mature IL-1β, IL-18, C-reactive protein, and urate in individual subjects were routine analyzed. In comparison with that treatment with febuxostat alone, treatment with both drugs for 12 weeks had a better therapeutic effect on reducing the values of visual analog scales, acute flares, and healthy assessment questionnaire scores in these gout patients. Furthermore, treatment with both drugs also significantly reduced the mean daily dose of etoricoxib and the levels of serum IL-1β and serum amyloid A. There was no significant difference in the frequency of patients with adverse effect between these 2 groups of patients. In conclusion, combination of diacerein and febuxostat had better therapeutic effect on reducing acute gout flares, inflammation, and clinical symptoms in patients with refractory gout.

系统性红斑狼疮(SLE)是1种复杂的自身免疫性疾病,以自身组织器官免疫耐受受到破坏,循环中出现多种自身抗体为特征.自身抗体和循环免疫复合物沉积于血管和周围组织中可引发炎症反应而损坏机体.该疾病的临床表现多种多样,其中肾脏和中枢神经系统受累最严重,可危及生命.SLE目前普遍的治疗方法包括使用糖皮质激素以及免疫抑制剂,但这些药物的不良反应也限制了其在部分患者中的使用.随着对SLE致病机制的深入研究,生物靶向治疗药物逐步研发并应用于临床.在类风湿性关节炎(RA)、强直性脊柱炎和炎性肠病等自身免疫性疾病中,生物制剂已被证明有效,而在SLE中生物制剂的使用仍存在争议.本文参阅最新的文献和临床实验结果,系统回顾并展望了目前使用生物制剂治疗SLE的有效性和安全性.

NK/T细胞淋巴瘤是一种少见的恶性疾病,曾被称为多形性网织细胞增生症、血管中心性T细胞淋巴瘤、致死性中线肉芽肿等.该病主要多发于亚洲及南美洲,有报道中国人T细胞淋巴瘤仅占所有淋巴瘤的10％,NK细胞淋巴瘤也仅约占6％[1].由于该病发病率低,早期临床表现多样,临床易误诊.我科收治了1例以反复咽痛起病的NK/T细胞淋巴瘤,发病半年后方确诊.现报道如下.

肌炎/皮肌炎(PM/DM)是一种特发性炎性肌病,是结缔组织病的一种,主要累及骨骼肌及其他器官.有报道指出,在肌炎/皮肌炎患者中呼吸系统受累的发病率高达45％[1].目前治疗该病的主要方法是糖皮质激素及免疫抑制剂.肌炎/皮肌炎合并呼吸系统受累时主要表现为由于肌无力所致的慢性呼吸衰竭,并发肺部感染、肺动脉高压、间质性肺炎等.Selva等[1]对81例肌炎/皮肌炎患者进行分析研究发现,61％的患者有肺部受累,其中有39％的患者表现为间质性肺炎,5例重型,预后不良;22％的患者表现为限制性肺病,其中有3例患者呼吸肌不能维持肺通气功能.由此可见,肌炎/皮肌炎患者间质性肺炎的发病率较高.

患者,男,45岁.10个月前出现头部皮肤剧烈瘙痒,无关节肿痛、肌肉酸痛、晨僵、光敏感等症状.患者多次到当地医院和我院皮肤科就诊,给予对症治疗,皮肤瘙痒缓解不明显.7个月前患者面颊部出现红色皮疹,到外院就诊,行皮肤活检示结合临床考虑"盘状红斑狼疮"可能性大.

类风湿关节炎(rheumatoid arthritis,RA)是一种以关节炎为主要临床表现的慢性炎症性疾病,可引起关节破坏、畸形和功能障碍,以手、足的小关节受累为主,并可累及全身其他系统.早期使用缓解病情抗风湿药(DMARDs)对于延缓关节变形、改善预后起着至关重要的作用.

<正>类风湿关节炎(RA)是一类以关节炎为主要临床表现的慢性炎性疾病,以手、足的小关节受累为主,并可累及全身其他系统。循证医学证实,合理使用缓解病情的抗风湿药(DMARDs)可以缓解RA患者病情,延缓骨破坏。在生物制剂出现以前,传统的抗风湿治疗药

体格检查是医生的基本功。文章阐述了在诊断学理论基础上,发挥教师集体备课的优势,总结了在体格检查教学中采用形象化,师生互动,重难点突出,因材施教的教学方法,同时重视医学生医德医风素质的培养。从而提高了学生诊断思维和实践能力,有效地提高了教学效果和质量。

Objective To investigate the positive rate and concentrations of tumor marker CA125 in sera of patients with systemic lupus erythematosus(SLE),and to analyze the relationship between serum CA125 and SLE disease activity index (SLEDAI),and serous cavity effusion.Methods The tumor marker CA125 in sera of 37 patients with SLE was determined by means of chemiluminescent microparticle immunoassay.The clinical data and results of laboratory examination were collected.The positive rate of serum CA125,and the relationship between serum CA125 and SLEDAI,and serous cavity effusion was statistically analyzed.Results The positive rate of tumor marker CA125 in sera of SLE patients was 32.4% (33.3% in active stage,and 30.8% in remission stage,respectively,P>0.05).The concentration of CA125 in sera of patients with peritoneal effusion or pleural effusion was higher than in those without peritoneal effusion or pleural effusion(P<0.05).Conclusion The positive rate of CA125 in sera is high in SLE patients,which may be related to the serous cavity effusion,but not to the activity of the SLE.

Objective To investigate the positive rate and concentrations of tumor marker CA125 in sera of patients with systemic lupus erythematosus(SLE),and to analyze the relationship between serum CA125 and SLE disease activity index(SLEDAI),and serous cavity effusion.Methods The tumor marker CA125 in sera of 37 patients with SLE was determined by means of chemiluminescent microparticle immunoassay.The clinical data and results of laboratory examination were collected.The positive rate of serum CA125,and the relationship between serum CA125 and SLEDAI,and serous cavity effusion was statistically analyzed.Results The positive rate of tumor marker CA125 in sera of SLE patients was 32.4%(33.3% in active stage,and 30.8% in remission stage,respectively,P>0.05).The concentration of CA125 in sera of patients with peritoneal effusion or pleural effusion was higher than in those without peritoneal effusion or pleural effusion(P<0.05).Conclusion The positive rate of CA125 in sera is high in SLE patients,which may be related to the serous cavity effusion,but not to the activity of the SLE.

类风湿性关节炎(Rheumatoid,RA)是一种慢性炎症性疾病,全球范围内一般人群发病率约为0.5%～1%,病变主要造成患者关节破坏及生活质量严重下降.

Objective:To investigate the effect of the improved demonstration in the physical examination of diagnostics compared with the traditional demonstration.Method:The improved demonstration,based on traditional demonstration,was focused on the difficulties and emphases.And the processes of improved demonstration are conducted step by step.The students of both groups had studied for one term.Finally the results of the both groups' tests were got.Results:The achievements in the experimental group were significantly better than those in the control group(P0.001).Conclusion:The improved demonstration is more valuable for the teachers of the physical examination than traditional demonstration.

Objective To understand the clinical features, treatment and prognosis of pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD). Method In 18 hospitalized patients with PAH associated with CTD, biochemical parameters, auto-antibodies, system involvement, EKG, chest X-ray, 6-minute walk (6MW) distance, hemodynamic measures (UCG), World Health Organization functional class, prognosis, treatment and tolerability were assessed. Results Of 18 cases, there were 17 females and one male. There were 11 cases of SLE, 5 cases of overlap connective tissue disease, one case of systemic scleroderma and one case of mixed connective tissue disease. The positive rate of diagnosis by EKG, chest X-ray and UCG was 27.8%, 38.9% and 100.0% respectively. Seventeen cases were positive for ANA. The positive rate of anti-U1RNP and anti-SSA was 55.6% and 61.1% respectively. All patients received the treatment of glucocorticoid, cyclophosphoramide, prostaglandin (Lipo-PGE1), and prostacyclin (beraprost sodium). On the week 12, WHO class and 6MWdistance were improved in 61.1% of patients (P<0.05). Conclusion PAH is a common complication of CTD, and has a poor prognosis. UCG is helpful to the diagnosis of PAH associated with CTD. The combined use of glucocorticoid, cyclophosphoramide, Lipo-PGE1 and beraprost sodium can improve the prognosis.

Objective To investigate the mRNA expression of a proliferation inducing ligand (APRIL) and its receptors including B cell maturation antigen (BCMA),transmembrane activator.calcium modulator and cyclophilin ligand interactor (TACI) in the peripheral blood mononuclear cells (PBMCs) of patients with systemic lupus erythematosus (SEE).Methods APRIL mRNA、BCMA mRNA and TACI mRNA in PBMCs were detected by real-time quantitative PCR in 66 SLE patients and 25 normal controls.Gene expression level was measured as 2-AACT.Results The expression levels of APRIL mRNA、BCMA mRNA and TACI-mRNA were significantly increased in both active SLE group and stable SLE group compared with those in the normal controls(P<0.01 for all).The expression levels of APRIL mRNA and TACI mRNA in active SLE group were significantly higher than those in stable SLE group(P<0.01,P<0.05,respectively).But there was no significant difierence in the expression levels of BCMA mRNA between the SLE stable and active groups-Beside,the expression levels of APRIL mRNA and TACI mRNA were significantly increased in patients with lupus nephritis (LN) compared to patients with non-LN (P<0.01 for all).Conclusion The expression levels of APRIL and its receptors are significantly elevated in SLE patients.It may suggest that APRIL and its receptors play an important role in the pathogenesis of SLE.

糖皮质激素(glucocorticoids)是治疗慢性炎症性疾病的常用药物,可以引起多种不良反应,其中糖皮质激素所致骨质疏松(glucocorticiod induced osteoporosis,GIOP)是继发性骨质疏松中最常见的一种,可导致椎体、肋骨、髋部等多部位骨折,严重影响患者生活质量并增加社会负担,是糖皮质激素治疗的严重并发症之一.

Objective The removal of apoptotic cells is affected by macrophages.Defective apoptotic cell clearance is associated with pathogenesis of tumor and autoimmune disease.Surface molecule CD14 has been suggested to be involved in the recognition and engulfment of apoptotic cells.The aim of this study is to determine whether CD14 is essential for in vivo phagocytosis of apoptotic cells.Methods Mice for CD14 deficient mice(CD14-/-) and heterozygous mice(CD14-/+) were immunized with T cell dependent antigen 2-phenyl-oxazolone coupled to chicken serum albumin(carrier protein).At days 12,14 and 17 after immunization,spleens were removed and analyzed by immunohistochemistry.Staining with fluorescent labeled reagents allows demonstrating whether CD14-/-macrophages can phagocytose the apoptotic debris generated in the germinal center reaction.Results CD14-/-mice showed normal germinal centre development.There was no accumulation of cell debris.Furthermore,immunohistology showed that apoptotic cells were engulfed by CD14-/-macrophages.Conclusion The experiment demonstrated that CD14-/-macrophages are capable of clearing the apoptotic debris generated in the germinal center reaction.CD14 may not be essential for phagocytosis of cell debris by macrophages.

患者,女性,57岁.因发现血象三少6年,眼干2年,口干2个月,发热1周于2006年11月9日收入院.患者于2000年11月在治疗慢性支气管炎的过程中多次查血常规均示血象三少,作骨髓细胞学检查无异常,使用补血药治疗无明显效果.

CD4+Th细胞活化后根据其分泌的细胞因子和效应功能的不同被区分为Th1和Th2型,Th1细胞分泌白细胞介素(IL)-2、干扰素(IFN)-γ和肿瘤坏死因子(TNF)-α等,介导针对细胞内病原体如细菌、病毒感染引起的细胞免疫,与迟发型超敏反应(DTH)和自身免疫病如系统性红斑狼疮(SLE)、类风湿关节炎(RA)、多发性硬化症(MS)、胰岛素依赖型糖尿病(IDDM)等有关;Th2细胞产生IL-4、IL-5、IL-10和IL-13等,刺激B细胞增殖并分泌抗体,介导体液免疫和过敏反应,与支气管哮喘等过敏性疾病有关.

目的 探讨近端指间关节周围胶原沉积症的临床表现、诊断和鉴别诊断.方法 总结23例患者的一般临床资料、实验室检查、关节累及情况和X线表现并结合文献进行分析.结果 23例患者平均发病年龄(15.3±3.9)岁,男性19例,女性4例,临床表现为慢性、无症状性的近端指间关节(PIP)周围软组织肿胀,多关节受累为主,基本对称,主要累及PIP的2～4关节.实验室检查正常.X线片除受累关节局部软组织肿胀外,无骨质破坏和关节间隙改变.结论 本病是一种少见的良性疾病,多发生于青少年男性,根据其临床表现加上放射学和常规的实验室检查即可作出诊断,无需特殊治疗.需和其它累及PIP软组织肿胀的疾病进行鉴别.