未分化结缔组织病合并IgA肾病一例

未分化结缔组织病（undifferentiated connective tissue diseases,UCTD）可以为某一种弥漫性结缔组织病的早期阶段或顿挫型，部分患者可能是一种独立的疾病。UCTD的肾损害发生率在11％左右，多有抗核抗体（ANA）阳性，临床表现可有水肿、高血压、蛋白尿、血尿和血清肌酐水平升高等，较少有造成严重肾功能不全者。UCTD的肾脏病理类型未见系统报道，现将1例UCTD合并IgA肾病的患者报告如下。     

未分化结缔组织病

未分化结缔组织病（UCTD）又称为未分化风湿病（URD）。1980年LeRoyetal最早提出未分化结缔组织综合征（UCTS）的概念,针对该病引起了风湿病学者们的广泛研究。直至1989年,Mosca等提出了UCTD的概念,并建立了UCTD的初步分类标准且被用于临床。本病具有多个结缔组织病的临床和免疫学实验室特点,但又不能满足任何一种特定疾病;换言之,这些临床与实验室特点是与某种分类位置的结缔组织病相关,被定义为一种独立的临床诊断疾病。     

未分化结缔组织病致继发性膜性肾病1例

未分化结缔组织病(UCTD)患者中有20%～40%发展成一种确定的结缔组织病(CTD);50%～60%的患者仍然维持在UCTD状态;10%～20%的患者症状逐渐减轻,但始终未发展成一种肯定的CTD。目前报道的CTD累及肾脏时通常临床表现轻微,多为镜下血尿或少量蛋白尿,且对肾脏受累病理类型报道较少。本文报道1例确诊UCTD合并继发性膜性肾病(MN)     

未分化结缔组织病合并妊娠的研究进展

未分化CTD是育龄期女性最常见的风湿病之一,可增加不良妊娠事件风险,同时妊娠可能诱发未分化CTD病情活动、进展为SLE等明确的风湿病。目前未分化CTD越来越引起临床医师的关注,但其与妊娠的相互影响尚未阐明,且缺乏公认的妊娠期治疗和管理推荐。此文对历年未分化CTD与妊娠的研究及最新临床探索进行综述,阐述两者之间的联系和孕...     

肺结节病误诊为未分化型结缔组织病一例

患者 ,女性 ,67岁。因关节疼痛、乏力 2年 ,视力模糊 1年 ,胸闷、心悸 1年 ,干咳 2个月入院。 2年前患者因关节疼痛在风湿科诊断为未分化型结缔组织病 ,予以间断口服强的松及芬必得等治疗 ,症状不缓解。 1年前因心悸、胸闷在心内科诊断为频发性室早、Ⅱ度房室传导阻滞 ,植入心脏起搏器。最近 2个月干咳频繁而入本科 ,拟诊为弥漫性肺间质纤维化。既往无气管炎、肺结核和心律失常等病史。入院体检 :慢性病容 ,无皮下结节及皮疹 ,全身浅表淋巴结不肿大 ,两肺无干湿罗音 ,心率 77次 /分 ,肝脾不肿大 ,无关节畸形及杵状指。B超检查显示 :胆囊炎…     

未分化结缔组织病合并皮肤非结核性分枝杆菌感染1例报告

患者女,39岁.因咳嗽、胸闷3 a,肢体肿胀伴发热4个月,加重半月入住我科.病程中有双手雷诺现象.查体:T 39 ℃、R 24次/min、P 120次/min.双下肺闻及细湿罗音,双前臂、右小腿软组织红、肿、热,双前臂内侧及右腘窝触及多个皮下红肿性硬结,边界不清,压痛不明显.白细胞 16.11×109/L,中性粒细胞 89.3%,血小板 333×109/L;24 h尿蛋白定量0.16 g;免疫十项、血液、骨髓、痰培养及痰涂片抗酸染色均阴性;抗核抗体1∶ 1 000(核均质型)、抗磷脂抗体-IgG( );血沉 66 mm/h,C反应蛋白54.65 mg/L,补体C3 0.89 g/L.     

结缔组织病并发肺部感染9例临床分析

目的探讨结缔组织病并发肺部感染的临床特点及治疗。方法对9例住院的结缔组织病合并肺部感染痰培养阳性的患者临床资料进行分析。结果结缔组织病合并肺部感染的临床症状并不明显;并发肺部感染的患者大多有贫血、血清白蛋白降低,肺间质纤维化;痰培养均为革兰阴性杆菌和真菌感染,提示结缔组织病患者并发肺部感染多为革兰阴性杆菌和真菌感染。结论结缔组织病合并肺部感染的临床症状并不明显,只要有咳嗽、咳痰的症状,就是做痰培养的指征。贫血、血清白蛋白降低、肺间质纤维化是结缔组织病患者并发肺部感染的易患因素。结缔组织病患者并发肺部感染多为革兰阴性杆菌和真菌感染,应及时给以相应治疗。     

严重脓毒症和脓毒性休克的治疗新进展

一、严重脓毒症的流行和研究状况 脓毒症是指感染合并全身炎症反应,严重脓毒症是指脓毒症合并脓毒症诱导的器官功能障碍或组织低灌注。组织低灌注是指收缩压（SBP）〈90mmHg（1mmHg=0．133kPa）或平均动脉压〈70mmHg或收缩压下降〉40mmHg,或在缺乏其他低灌注原因下按年龄下降〉2个标准差,脓毒性休克是指在充分液体复苏后仍表现低灌注。脓毒症引起的组织低灌注的临床表现包括脓毒性休克、乳酸增高或少尿。     

槐杞黄颗粒治疗未分化结缔组织病的疗效观察

<正>未分化结缔组织病(UCTD)是一组具有系统性自身免疫性疾病的临床和血清学表现,但不符合特定结缔组织病(CTD)诊断标准的疾病^[1]。大多数患者表现为关节症状、干燥症状、皮疹、光过敏等一个或多个症状。随时间进展,部分患者转化为明确的CTD,包括系统性红斑狼疮、系统性硬化症等^[2]。早期治疗干预达到延缓或中止部分患者转化为CTD将很大程度减轻患者经济、心理负担。因此不断深入对UCTD的研究,拓展相应治疗手段意义重大。     

Unclassified or undifferentiated connective tissue disease.

This chapter deals primarily with the evidence available regarding the use of the term unclassified or undifferentiated connective tissue disorders (CTDs) to refer to patients with manifestations suggestive, but not diagnostic, of either defined CTDs, a well-defined overlap syndrome (manifestations and criteria for two or more CTDs), or mixed CTD (MCTD). Possible outcomes for these patients include remaining undifferentiated, differentiating into a defined CTD or going into remission. MCTD and atypical CTD (term used by the parties involved in the 'Silicone breast implant' controversy) are only briefly discussed.     

Meningitis in mixed connective tissue disease complicated by herpes virus infection: case report

The authors report a rare case of a female patient diagnosed with mixed connective tissue disease (MCTD). After a few years in remission, the patient acquired herpes zoster infection followed by a disease flare. Disease activity was accompanied by the development of meningitis. To determine whether the meningitis was caused by the previous herpes virus infection or was aseptic meningitis associated with the activity of MCTD raised important differential diagnostic issues. Repeated laboratory assessments of the patients sera and cerebrospinal fluid revealed leukocytopenia, high anti-U1 ribonucleoprotein autoantibody level, increased immune complex, and decreased complement concentrations. The administration of corticosteroids resulted in rapid improvements in clinical symptoms and laboratory indicators.     

An unusual case of undifferentiated connective tissue disease presenting as cardiac tamponade

Cardiac tamponade as an initial manifestation of undifferentiated connective tissue diseases (UCTD) is extremely rare, with only one case reported in literature thus far. We describe here, a case of a middle-aged man who presented with symptoms of fatigue, exertional dyspnea and orthopnea. His physical exam was significant for anasarca, elevated JVP and pulsus paradoxus. Chest X-ray showed pleural effusions and cardiomegaly, electrocardiogram revealed electrical alternans and a transthoracic echocardiogram demonstrated massive pericardial effusion with hemodynamic compromise. There was clear evidence of tamponade on right heart catheterization. All common causes of pericardial effusion were assiduously excluded before working up the patient for connective tissue disorders, which revealed a high antinuclear antibody titer (1:160), grossly elevated SSA, SSB antibodies and increased C-reactive protein levels (13.04 mg/dl). Patient had no signs or symptoms suggestive of systemic sclerosis (xerophthalmia or xerostomia) and did not meet criteria for any other known connective tissue diseases. He was therefore diagnosed with UCTD, and successfully treated with colchicine after emergency pericardiocentesis. This case presents UCTD as a rare cause of cardiac tamponade and large pericardial effusions and suggests that colchicine can be used to treat UCTD-associated effusions. These patients once diagnosed, are at risk of developing known connective tissue diseases within 5 years of disease onset and should be followed up in clinic periodically.     

A case of acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease

The authors describe a case of a 43-year-old woman affected by acquired partial lipodystrophy associated with localized scleroderma and undifferentiated connective tissue disease. Moreover, they review the literature on pathogenetic mechanisms suspected to be involved in partial lipodystrophy and their association with autoimmune diseases.     

A case of mixed connective tissue disease complicated with hypertrophic obstructive cardiomyopathy

A 54-year-old female was diagnosed as mixed connective tissue disease (MCTD) complicated with secondary Sj?gren's syndrome. Although she had no dyspnea on exertion, the chest X-ray showed cardiomegaly with interstitial pneumonia. The echocardiogram demonstrated asymmetric hypertrophy of the interventricular septum. Diagnosis of hypertrophic obstructive cardiomyopathy (HOCM) was confirmed by left ventriculography and myocardial biopsy. She was treated with prednisolone, resulting in improvement of swollen hand, elevated muscle enzymes and interstitial pneumonia. A rare complication of HOCM with MCTD was described.     

A case of mixed connective tissue disease complicated with thrombotic thrombocytopenic purpura

Thrombotic thrombocytopenic purpura (TTP) is a rare complication of mixed connective tissue disease (MCTD). In this report, we describe the case of a 73-year-old Japanese woman with MCTD who developed fever, thrombocytopenia, and microangiopathic hemolytic anemia and was diagnosed with MCTD together with TTP. The activity of von Willebrand factor (vWF) cleaving metalloprotease ADAMTS13 was low and considered to have contributed to the disease activity of TTP. The patient died despite intensive treatment of plasma exchange (PEX) and steroid pulse therapy. Autopsy results revealed that the kidneys had platelet and fibrin thrombi, which occluded capillaries and arterioles. These findings were compatible with TTP and the decreased activity of ADAMTS13 was considered to be associated with the disease activity of TTP.     

Coexistence of ankylosing spondylitis and undifferentiated connective tissue disease

We report the case of a 45-year-old Caucasian woman suffering from ankylosing spondylitis and undifferentiated connective tissue disease in whom the prevailing clinical features were retinal vasculitis and inflammatory low back pain. HLA typing revealed the concomitant presence of B27 and DR2 antigens. We hypothesise that the uncommon coexistence of ankylosing spondylitis and connective tissue disease in the same patient could be due to the exceptional association of HLA- B27 with the DR2 antigen.     

Subclinical pulmonary involvement assessed by bronchoalveolar lavage in patients with early undifferentiated connective tissue disease

OBJECTIVE: To assess the presence of neutrophil and lymphocyte fibrosing alveolitis by bronchoalveolar lavage in patients with early undifferentiated connective tissue disease (EUCTD) and systemic sclerosis (SSc). METHOD: Bronchoalveolar lavage (BAL) was performed in 13 patients with EUCTD who exhibited signs of lung involvement by non-invasive methods including lung function tests and high resolution computed tomography. The mean age of cases was 48.1 +/- 6.6, and the mean disease duration was 1.8 +/- 0.8 years. Differential cell counts of BAL were evaluated. Eleven patients with systemic sclerosis and 5 healthy control subjects were also investigated. RESULTS: Eleven of the 13 EUCTD and 10 of the 11 SSc patients showed an elevated total cell number (above the median cell/ml of control + 2 SD) in the BAL fluid. In patients with EUCTD, the lymphocyte count was elevated in 6, and the polymorphonuclear neutrophil count in 2 patients. One of the patients with EUCTD had simultaneously elevated lymphocyte and neutrophil granulocyte counts. In the SSc group, 6 patients had an elevated lymphocyte and 6 an increased neutrophil count. Three of these cases had both increased neutrophil and elevated lymphocyte counts, simultaneously. CONCLUSION: Subclinical, predominantly lymphocyte alveolitis can be present in patients with EUCTD. Patients with SSc tend to exhibit neutrophil alveolitis.     

Breast implants in patients with differentiated and undifferentiated connective tissue disease

Objective. To assess the frequency of breast implantation and the relationship of the implants to the onset of symptoms in patients with differentiated and undifferentiated connective tissue disease (CTD). Methods. We evaluated an inception cohort of patients with differentiated and undifferentiated CTD and symptoms of < 12 months duration when enrolled in 1983–1987. The risk of having breast implants in those patients with early symptoms of CTD was determined in comparison with that in a non-concurrent control group. Results. Only 3 of 323 women in the cohort had historical, physical, or chest radiographic evidence of breast implantation. In 1 of the 3 patients, the symptoms of CTD began before the breast implantation. The odds ratio was calculated at 1.15, with a 95% confidence interval ranging from 0.23 to 3.41. Conclusion. This study showed an absence of significant risk for prior breast implantation surgery in patients with well-defined or undifferentiated CTD.