结缔组织病所致间质性肺疾病的研究进展

结缔组织病（CTD）包括许多种疾病,如红斑狼疮、硬皮病、类风湿关节炎、干燥综合征、皮肌炎/多发性肌炎及血管炎等。间质性肺病多发生于CTD中,其中早期表现为肺泡炎,而晚期则表现为弥漫性肺间质纤维化,病程终末期可出现呼吸衰竭,因为其发生率和病死率较高,目前已成为研究热点。该病病因和发病机制复杂,预后及治疗方法和效果取决于组织病理类型、风湿病的基础情况及相关的肺动脉高压。本文就CTD所致间质性肺病的现代认识进行综述。     

关注结缔组织病相关间质性肺疾病

结缔组织病（connective tissues disease,CTD）是风湿性疾病中的一大类,常累及全身多个系统,表现为慢性炎症性自身免疫病,当侵犯呼吸系统时,可出现间质性肺疾病（interstitial lung disease,ILD）、胸膜炎和肺动脉高压等,见表1。CTD与ILD同时存在时,常称为结缔组织病相关间质性肺     

结缔组织病相关间质性肺病与自身抗体的相关性研究进展

间质性肺疾病(ILD)是结缔组织病(CTD)的重要并发症之一,结缔组织病相关间质性肺病(CTD-ILD)的发病率和病死率较高,并且早期确诊有一定困难,CTD-ILD与自身抗体相关性的研究对其预防、早期确诊、指导治疗及预后极为重要.为了便于临床能正确诊断和及时治疗CTD-ILD,现将常见于CTD的自身抗体及CTD患者发生ILD的临床特点进行综述.     

结缔组织病相关间质性肺疾病的临床研究进展

结缔组织病（CTD）是一组累及全身多系统的慢性炎症性自身免疫病,其共同病理变化为淋巴细胞致敏产生免疫损伤,导致血管壁、间质的纤维素样坏死性炎症及随后引起多脏器的胶原纤维增生。由于肺间质、肺血管、支气管及胸膜均富含疏松结缔组织和血液供应,CTD常累及肺和胸膜,引起间质性肺疾病（ILD）、肺血管炎和胸膜炎等。     

结缔组织病合并间质性肺疾病的研究进展

间质性肺疾病普遍发生于结缔组织病中，因为它的发生率和病死率较高，目前已成为研究的热点。近几年，在病理分型方法、诊断和治疗方面，结缔组织病合并间质性肺疾病有了一些新的进展．在此作一综述。     

305例结缔组织病相关间质性肺病临床特点分析

目的分析结缔组织病相关间质性肺病(Connective tissue disease associated interstitial lung disease,CTD-ILD)临床特征。方法回顾性分析305例西京医院临床免疫科CTD-ILD的临床特征及类风湿关节炎间质性肺病(Rheumatoid arthritis associated interstitial lung disease,RA-ILD)和干燥综合征间质性肺病(Sjogren's syndrome associated interstitial lung disease,p SS-ILD)患者胸部高分辨CT特点。结果 CTD患者ILD的发病率为11.78%,其中多发性肌炎相关ILD的发病率最高(53.13%),其次为抗中性粒细胞胞浆抗体相关性血管炎(40.74%)、混合结缔组织病(35.14%)和硬皮病(29.73%)。p SS-ILD和RA-ILD在CTD-ILD中所占比例最大,分别为24.92%和23.61%,两者约占所有CTD-ILD的一半。结论 CTD患者ILD的发病率高,RA-ILD和p SS-ILD是CTD-ILD的主要疾病,加强对CTD-ILD的早期筛查和早期治疗非常重要。     

352例结缔组织病并间质性肺疾病临床特点分析

目的本研究通过比较分析结缔组织病并间质性肺疾病（connectivetissuediseaseandinterstitiallungdisease,CTD-ILD）患者的临床特点,旨在提高临床医师对CTD-ILD疾病的认识水平。方法收集并回顾性分析2011年1月1日至2012年12月31日于郑州大学第一附属医院初诊为CTD-ILD的患者的临床特征及影像学特点。结果CTD-ILD患者352例,男女发病比例为1：2．67,平均发病年龄（53．07±13．65）岁。CTD-ILD患者全身症状发生率高于呼吸系统症状。CTD-ILD的高分辨率CT表现多为对称性磨玻璃影、网格状、胸腔积液等改变,主要累及中肺和下肺。结论CTD-ILD多发生于育龄期女性,部分肺部病变首发于CTD症状之前,并可独立进展。高分辨率CT有助于ILD的早期诊断及治疗方案制定。     

结缔组织病合并间质性肺病重症感染预后因素分析

目的回顾分析结缔组织病(CTD)合并间质性肺病(ILD)重症感染的危险因素、死亡原因及治疗方法。方法回顾性分析2014年5月至2017年5月RICU收治的67例CTD合并ILD终末期患者,明确诊断为CTD,包括系统性红斑狼疮(SLE)、类风湿关节炎(RA)、多发性肌炎/皮肌炎(PM/DM)、系统性硬化病(SSC)、干燥综合征(p SS)等,分析ILD发生情况及临床资料、治疗及预后情况。结果 1.存活组与死亡组患者在临床症状、体征、合并基础病、影像学组间差异无统计学意义。临床症状两组均有发热、咳嗽、呼吸困难为主要表现,影像以网格、磨玻璃、蜂窝及实变为主要表现。2.两组实验室检查比较,死亡组患者Ig G、Ig A、Ig M水平明显高于存活组(P 0. 05)、死亡组患者氧合指数明显低于存活组(P 0. 05)。3.双因素logistic回归,结果示有创呼吸机通气和无创呼吸机通气是影响预后的独立预测因子。4.本实验中将积极治疗后入ICU,是否行无创呼吸机通气和有创呼吸机各分为两组,分别统计患者的生存期,以28天生存期为上界,生存曲线显示,应用有创呼吸机组患者的生存期为13. 1±1. 73天,未应用有创呼吸机患者的生存期为19. 1±2. 22天,两者具有显著差别(P=0. 003)。在无创呼吸机组,生存期无统计学意义(P 0. 05)。结论 CTD合并ILD死亡组患者中Ig G、Ig A和Ig M显著升高,氧合指数明显降低,有创呼吸机通气和无创呼吸机通气是影响预后的独立预测因子,应用有创呼吸机患者的生存期明显低于未应用有创呼吸机组。建议患者CTD合并ILD终末期患者首先给予经鼻高流量吸氧,并尽量避免行有创机械通气和无创通气拖延抢救时机。     

结缔组织病相关间质性肺病的治疗进展

结缔组织病( Connective tissue disease,CTD)是一组异质性自身免疫性疾病,包括系统性红斑狼疮( SLE )、系统性硬化症( SSc )、特发性炎性肌病(ⅡM)、干燥综合征(SS)和类风湿关节炎(RA)[1].多种CTD可累及肺间质,导致弥漫性肺泡及其毛细血管功能单位的丧失,严重影响患者的生活...     

Interstitial lung disease in connective tissue disorders

Some of the most pressing challenges associated with interstitial lung disease (ILD) are how best to define, diagnose, and treat connective tissue disease-associated ILD (CTD-ILD)--disorders with potentially substantial morbidity and mortality. In this focused review, we address aspects of prognosis for CTD-ILD and what indices might predict outcome, together with lessons that can be learnt from clinical trials of systemic sclerosis-associated ILD and idiopathic pulmonary fibrosis and how these lessons might be applied to future studies of CTD-ILD.     

Interstitial lung disease in connective tissue disorders

Interstitial lung diseases (ILD) associated with connective tissue disorders differ from idiopathic ILD in several aspects, although most of them are comparable. In most patients, ILD occurs during the course, or at the time of diagnosis of connective tissue disease. Opportunistic pulmonary infections, together with adverse effects of treatment should always be discussed. The prevalence of ILD varies among the different connective tissue disorders. Thus, ILD is frequently encountered in patients with systemic sclerosis and to a lesser degree in patients with myositis. As compared to idiopathic ILD, histopathological aspects of ILD associated with connective tissue diseases are more frequently those of non-specific interstitial pneumonia, whereas usual interstitial pneumonia is rare. Other ILD, such as organized pneumonia, interstitial lymphoid pneumonia, diffuse alveolar damage and alveolar hemorrhage are occasionally encountered. ILD must be detected early in the course of collagen disorders by performing computed tomodensitometry and pulmonary function tests. The prognosis of connective tissue associated ILD is better than that of idiopathic ILD. The treatment requires corticosteroids and/or immunosuppressants, depending on the nature of the associated connective tissue disease and ILD progression.     

Interstitial lung disease in the connective tissue diseases

The connective tissue diseases (CTDs) are inflammatory, immune-mediated disorders in which interstitial lung disease (ILD) is common and clinically important. Interstitial lung disease may be the first manifestation of a CTD in a previously healthy patient. CTD-associated ILD frequently presents with the gradual onset of cough and dyspnea, although rarely may present with fulminant respiratory failure. Infection and drug reaction should always be ruled out. A diagnosis of idiopathic ILD should never be made without a careful search for subtle evidence of underlying CTD. Treatment of CTD-ILD typically includes corticosteroids and immunosuppressive agents.     

结缔组织病肺间质病变的临床特点及治疗效果分析

目的探讨不同结缔组织病肺间质病变（CTD-ILD）的临床特点和治疗效果。方法分析82例CTD-ILD患者的临床特征及胸部高分辨CT（HRCT）特点并观察治疗后HRCT的变化。结果（1）SSc—ILD的发生率最高,为70．0％。其次为pSS—ILD、PM／DM-ILD、RA—ILD、SLE—ILD,系统性红斑狼疮继发的ILD（SLE—ILD）发病年龄（26．2±7．8）较其他CTD-ILD早（P〈0．05）。RA—ILD、SSc—ILD病程较长,pSS—ILD、SLE一[LD和PM—ILl）病程较短。RA．ILD出现活动后气促少见（P〈0．05）。pSS—ILD出现呼吸道症状（40％）和体征（50％）最多见。85．7％SSc-ILD出现雷诺现象,支持雷诺现象与ILD相关。（2）SLE—ILD以磨玻璃影多见,丽蜂窝状改变较少;pSS-ILD以蜂窝状改变和纵隔淋巴结肿大相对较多。（3）大部分患者治疗后肺部间质病变吸收好转（56．7％）,其中88．2％SLE—ILD吸收好转,28．4％患者肺部病变处于静止,14．9％患者间质病变较前进展。结论CTD—ILD患者一旦确诊,应尽早用激素联合免疫抑制剂治疗。     

Interstitial lung disease in the patient who has connective tissue disease

Interstitial lung disease is a common complication of many of the connective tissue diseases. Because the prognosis, degree of reversibility, and optimal therapy differs for each disease presentation, a thorough knowledge of the pulmonary presentations of each connective tissue disease is important. Additionally, the challenge of finding the patient who has occult connective tissue disease in an interstitial lung disease clinic is discussed.     

结缔组织病相关间质性肺病的诊治策略

结缔组织病(connective tissue disease,CTD)是一组全身性自身免疫性疾病,病变累及多种脏器。由于肺和胸膜均富含胶原、血管等结缔组织,因此 CTD 大多可以损伤肺和胸膜等呼吸系统多个器官,包括：呼吸肌、胸膜、肺血管、气道、肺实质和肺间质,且部分患者呼吸道表现为首发症状。间质性肺病(interstitial lung disease,ILD)在 CTD 中十分常见,发生率在数个 CTD 病种中超过50%。与之相对应,15%~30%初诊为特发型间质性肺炎(idiopathic interstitial pneumonia,IIP)的患者最终被确认符合 CTD-ILD 诊断。ILD 是导致 CTD 患者死亡的重要原因之一。CTD 包括包括系统性红斑狼疮(SLE)、类风湿性关节炎(RA)、原发性干燥综合征(pSS)、多发性肌炎(PM)、皮肌炎(DM)、系统性硬化(SSc)和混合性 CTD (MCTD)等。本文就 CTD-ILD 的诊治策略作一简要综述。     

Hypertrophic pachymeningitis and undifferentiated connective tissue disease: a case report and review of the literature

A 63-year-old man with hypertrophic pachymeningitis (HP) and an undifferentiated connective tissue disease is described. Combined therapy with prednisolone and azathioprine improved his symptoms. The association between an undifferentiated connective tissue disease and HP is discussed.     

Systemic connective tissue disease complicated by Castleman's disease: report of a case and review of the literature

Castleman's disease is an atypical lymphoproliferative disorder characterized by the prevalence of B CD5-positive cells in the marginal zone. Autoimmune manifestations have often been reported, but the association of Castleman's disease with systemic autoimmune syndromes has been rarely described. However, many authors stress the difficulties in distinguishing between connective tissue disease and Castleman's disease in most cases. To clarify this issue, we describe a patient and review the literature reports of all cases of Castleman's disease associated with a connective tissue disease. A 19-year old woman presented with autoimmune thyroiditis and polymyositis. Seven years after the onset she developed a systemic inflammatory flare and a burst of autoimmunity, followed by generalized lymphoadenopathy. A mediastinal lymph node biopsy led to the diagnosis of Castleman disease of mixed type. Chemotherapy was given, with rapid response of the lymphoproliferative disorder but persistence of the underlying autoimmune disorder. The plasma concentration of B-lymphocyte stimulator (BLyS) was high (13.3 ng/mL) at the diagnosis of Castleman's disease. It fell dramatically after chemotherapy (4.97 ng/mL), even though it remained just above the mean BLyS value found in healthy blood donors (3.37+/-0.78 ng/mL).Castleman's disease can present autoimmune traits. In our patient, Castleman's disease complicated the course of a connective tissue disorder several years after the onset. We hypothesize that chronic stimulation of B-cell clones, particularly CD5+, by BLyS could favor the development of both autoimmune diseases and a broad range of lymphoproliferative disorders (such as Castleman's disease). This is the first report of increased BLyS levels in a patient with Castleman's disease, supporting a possible pathogenetic role of BLyS in the development of an autoimmune disorder and of a B lymphoproliferative disorder years later.