第26例--脓涕、发热、咳嗽、右肺空洞型结节

1 病历摘要 患者:女性,37岁.因发热伴咳嗽、咯痰近2个月,于2001年9月30日入院.患者于2001年8月11日无明显诱因发热(38.5 ℃),伴咳嗽、咯白粘痰,无畏寒、寒战、咯血、胸痛、憋气;于外院查血常规示:白细胞10.2×109/L,中性粒细胞0.883;胸片提示右下肺团块影;予青霉素、链霉素等抗感染治疗10 d后体温恢复正常,但仍有咳嗽、咯痰.进一步行胸部CT检查提示右下肺空洞型肿块、未见纵隔肿大淋巴结及胸腔积液,考虑边缘型肺癌伴右下肺门增大或炎性肉芽肿性病变;于2001年9月3日行右下肺叶切除术.术后病理检查示:肿物为4.0 cm×2.5 cm×3.0 cm,结节型,考虑肺韦格纳肉芽肿病(Wegner′s granulomatosis,WG),气管旁、下肺韧带、肺门及隆突下淋巴结反应性增生.     

疑难病例析评第52例 血小板减少-习惯性流产-喘憋-不能平卧

病历摘要患者女 ,30岁。因咳嗽、喘憋 2 0余天 ,加重 1周于 2 0 0 2年 12月 2日入院。患者于 2 0 0 2年 11月上旬受凉后出现咳嗽、咯少量白黏痰 ,活动后喘憋明显 ,无发热、咯血、胸痛。于外院查血常规示 :白细胞(WBC) 14 9× 10 9/L ,中性粒细胞 0 773;胸部X线片示 :双下肺片状影 ,右下肺为著。考虑“气管炎” ,予阿奇霉素 2 5 0mg/d治疗 ,共 6d ,无效 ;喘憋进行性加重、夜间不能平卧 ,偶伴血丝痰。为进一步诊治收入我院。患者曾有日晒后口腔溃疡、面部发红 ,无皮疹、关节肿痛、口眼干、肌痛、肌无力 ;否认四肢肿痛、癫痫发作病史 ;尿量…     

抗膜突蛋白抗体与系统性硬化病相关肺间质病变的临床相关性研究

Objective To identify a novel auto-antibody in sera of systemic sclerosis (SSc) patients and to analyze its relevance with SSc-associated interstitial lung disease (ILD). Methods The anti-moesin antibody in the sera of 62 SSc patients, who had participated the European League Against Rheumatism's Scl eroderma Trial and Research Group (EUSTAR), were tested by enzyme linked immunosorbent assay (ELJSA). Patients were grouped by high resolution computerized tomography (HRCT) features, pulmonary function test (PFT) abnormalities, inflammatory markers and disease course. The prevalence and titer (Optical density value) of anti-moesin antibody were compared between groups with t and χ2 test. Results The titer of anti-moesin antibody was significantly higher in the SSc-ILD group than non-ILD group (0.156±0.062 vs 0.107± 0.026, P=0.005). Among SSc patients, the diagnostic sensitivity and specificity of the anti-moesin antibody for ILD was 44.0% and 91.7% respectively (Kappa=0.2, P=0.022). Anti-rnoesin antibody was more prevalent in SSc patients with HRCT features of honeycomb-like lesion, lobular septal thickening and mediastinal lymphadenopathy (P<0.05). SSc patients with deteriorated total lung volume (TLC %) had higher titer of anti-moesin antibody significantly (0.172±0.067 vs 0.133±0.039, P=0.011), as the same tendency in patients with decreased diffusing capacity of the lung for carbon monoxide (DLco% ) but without statistical significant difference (0.153±0.580 vs 0.120±0.340, P=0.089). The anti-moesin antibody was equally prevalent between abnormal ESR, C reactive protein, immunoglobulin and complements groups and their normal controls (P> 0.05). Group of patients who had SSc courses more than or less than 5 years demonstrated similar anti-moesin antibody titers (0.146±0.047 vs 0.164±0.077, P=0.272). However, patients with ILD courses less than 12 months had higher liter of the antibody than controls (0.182±0.073 vs 0.138±0.049, P=0.040). Conclusion This study suggests that the novel anti-moesin antibody has comparatively high specificity for SSc-associated ILD patients, which may contribute to further understanding the pathogenesis of ILD in SSc patients. Further investigations are deserved to evaluate the application of anti-moesin antibody in facilitating early screening and evaluation of ILD.     

误诊为系统性红斑狼疮Jaccoud关节的Rhupus综合征一例

患者女,50岁,因关节肿痛、面部红斑19年,蛋白尿13年就诊.患者于1991年开始出现双手近端指间关节肿痛、晨僵30 min左右,伴面部红斑、无光敏.     

CREST综合征合并原发性胆汁性肝硬化一例

CREST综合征是系统性硬化症（SSc）的一种特殊类型,合并原发性胆汁性肝硬化（PBC）非常少见,现报告如下。     

系统性红斑狼疮并发蛛网膜下腔出血的临床特点

目的探讨系统性红斑狼疮(systemic lupus erythematosus,SLE)并发蛛网膜下腔出血(subarachnoid hemorrhage,SAH)患者的临床特点及预后。方法总结分析1983年1月至2012年1月就诊于北京协和医院的12例SLE并发SAH患者的临床资料。结果 SLE并发SAH患者住院期间的病死率高达42%(5/12)。SLE并发SAH的症状以头痛为主(75%)。系统性红斑狼疮疾病活动指数平均为(17.5±4.5)分。死亡患者在SAH发生早期即出现意识障碍者占80%(4/5),合并严重感染者占60%(3/5)。结论 SAH是SLE的少见且致命的并发症。对于出现意识障碍或合并感染的SLE患者,应当提高警惕,积极治疗SLE原发病及SAH,提高生存率。     

波生坦在结缔组织病中的应用

内皮素(endothelin,ET)是日本科学家于1988年发现的一种由21个氨基酸组成的肽类物质~([1]),它具有调节血管张力,促进细胞分裂等生理作用;而在疾病中,则是一个关键的致病因子,促进血管收缩、炎症和纤维化.     

系统性红斑狼疮病情评估体系解读

系统性红斑狼疮(systemic lupus erythematosus,SLE)是一种累及多器官系统的自身免疫性疾病.SLE临床表现多样:既可有单纯的皮肤黏膜损伤,又可合并肾脏等重要脏器损伤;严重程度不一:既可是轻微隐匿的,也可是严重而致命的;同时SLE病情有自发缓解和加重的波动过程.导致SLE表型复杂的原因不仅与疾病本身相关,还会受到并发症及药物不良反应等因素的影响.     

第10届干燥综合征国际研讨会会议纪要

<正>第10届干燥综合征(Sjgren’s syndrome,SS)国际研讨会于2009年10月1日至3日在美丽的法国小城布雷斯特举行,来自世界42个国家近300名风湿病学和免疫学代表聚集一堂,广泛、深入地探讨了SS病因、发病机制和治疗等问题。