不容忽视的结缔组织病并发肺间质病变

肺间质病变（ILD）是与多种结缔组织病（CTD）相关的一类疾病。CTD-ILD病理类型复杂,影像学表现各异,临床治疗困难,是引起CTD患者死亡的重要原因。本文综述了常见结缔组织病并发间质性肺病的组织病理、影像学、临床特点以及相关的治疗进展。     

结缔组织病肺间质病变的临床、影像和病理诊断

目的了解结缔组织病肺间质病变（connective tissue disease-interstitial lung disease,CTD-ILD）的临床、影像及病理学特征,探讨CTD-ILD影像学与病理类型间的相关性。方法通过高分辨计算机体层成像（high-resulution computed tomography,HRCT）技术了解132例弥漫性结缔组织病合并肺间质病变患者的肺间质病变影像学特点及分型,经皮肺穿刺活检技术进行肺间质病变病理分型。结果与系统性红斑狼疮（systemic lupuserythematosus,SLE）、多发性肌炎（polymyositis,PM）、系统性硬化症（systemic sclerosis,SSc）、类风湿关节炎（rheumatoid arthritis,RA）相比,原发性干燥综合征（primary Sj觟gren＇s syndrome,pSS）、皮肌炎（dermatomysitis,DM）、血管炎肺间质病变的呼吸道症状更明显。从影像学上看CTD-ILD表现多种多样：SLE以磨玻璃影多见,pSS以实变影多见,DM、PM、血管炎以实变影及毛玻璃影多见,SSc和RA以网格影多见。不同疾病肺间质病理类型有显著性差异,SLE、pSS、DM以非特异性肺间质炎（nonspecific interstitial pneumonia,NSIP）为主,SSc以寻常型肺间质炎（usual interstitial pneumonia,UIP）为主。病理类型NISP在影像学上多表现为毛玻璃样实变病变,UIP则更多表现为网格样蜂窝样病变,与其他影像学表现比较有统计学差异（P〈0.05）。用力肺活量和一氧化碳弥散能力下降在病种、影像学及病理改变上均无统计学差异。结论HRCT对诊断CTD-ILD有高敏感性及特异性,影像学改变可帮助临床医生推测其组织病理类型,便于随访和疗效的评价,减少肺活检的危险,是目前诊断CTD-ILD的重要手段。     

Coexistence of Marfan-like Connective Tissue Disease with Morphologic Left Ventricular Non-compaction

We treated a man with co-incident Marfan-like connective tissue disease with morphologic left ventricular non-compaction (LVNC). He underwent valve-sparing aortic root replacement because of aortic root dilation at 43 years old. Pathological findings of the aorta revealed cystic medio-necrosis, consistent with Marfan syndrome. He developed congestive heart failure caused by LVNC at 47 years old. His daughter had scoliosis, and he had several physical characteristics suggestive of Marfan syndrome. We herein report a rare case of a patient who had Marfan-like connective disease with an LVNC appearance.     

结缔组织病相关肺动脉高压血清标记物研究现状

结缔组织病相关肺动脉高压（CTD-PAH）因其发病机制存在自身免疫和炎症反应,故其病情评估指标除适用于特发性肺动脉高压的标记物外,文献提示还可能存在以下几种血清学标记物：（1）自身抗体,如抗内皮细胞抗体、抗成纤维细胞抗体、抗β2-糖蛋白1（β2-GP1）抗体以及抗U1RNP和U3RNP抗体;（2）炎症因子,包括趋化因子（如CX3CL1,CCL2和RANTEs）、生长因子（如血小板源性生长因子等）;（3）其他血清标记物如N-末端尿钠肽原、endoglin等。这些标记物可能成为提示或预测CTD-PAH更为特异的标记物,值得进一步探讨和证实。     

类风湿关节炎相关肺间质病变的危险因素、临床和影像学特点

目的分析类风湿关节炎(RA)相关肺间质病变(RA-ILD)的危险因素、临床特点及影像学特征,以利于早期诊断、合理治疗。方法回顾性总结233例住院诊治RA患者的临床资料,比较RA-ILD组与单纯RA组的临床特点。结果 RA-ILD的发生率为20.6%(48/233)。RA-ILD组患者平均发病年龄、平均病程、大量吸烟率、类风湿因子水平、抗环瓜氨酸肽抗体阳性率、抗核周因子阳性率和抗角质蛋白抗体阳性率均高于单纯RA组,差异有统计学意义(P<0.05);双手X线Ⅰ期改变百分数较单纯RA组低,差异有统计学意义(P<0.05)。26例(54.17%)RA-ILD患者有呼吸系统临床表现,包括咳嗽、活动后呼吸困难和肺部爆裂音;22例(45.83%)通过肺高分辨CT(HRCT)和/或肺功能(PFT)检查诊断。HRCT改变依次为纤维条索/网格影33例(68.75%)、斑片渗出影/实变影21例(43.75%)、磨玻璃影15例(31.25%)、蜂窝状影11例(22.92%)以及胸腔积液5例(10.42%);PFT均有弥散功能减退。结论 RA-ILD发病年龄较大、病程较长,与疾病严重性相平行,而与疾病活动性不相关。HRCT及PFT对早期诊治RA-ILD有重要价值。     

Tacrolimus Treatment for Post-COVID-19 Interstitial Lung Disease

With expansion of the COVID-19 pandemic, reports of post-COVID-19 interstitial lung disease (ILD) have been emerging. However, there are few reports regarding treatment. Some reports indicate that corticosteroids are effective for post-COVID-19 ILD, but the use of long-term corticosteroid carries risks of side effects. We administered tacrolimus to an elderly patient with post-COVID-19 ILD who suffered a respiratory failure relapse during steroid tapering. The respiratory status improved with tacrolimus in the post-acute phase, but pulmonary fibrosis progressed in the late phase. Tacrolimus may be effective for treating post-COVID-19 ILD in the post-acute phase, but it does not halt progression of pulmonary fibrosis.     

Mixed Connective Tissue Disease Associated with Skin Defects of Livedoid Vasculitis

A 21-year-old woman who had a 2-year history of mixed connective tissue disease (MCTD) developed rapidly evolving ulcers consistent with livedoid vasculitis (LV) in all distal extremities. She presented clinically with Raynaud’s phenomenon, polyarthritis and swollen hands; serologically with high titres of ANA and anti-nRNP; and immunogenetically with HLA-DR4 and HLA-DR53. Although there was initial success in treatment except for the skin defects over the ankles, the patient died from disseminated intravascular coagulation. We suggest that LV may be a poor prognostic manifestation in MCTD. Received: 9 August 1999 / Accepted: 25 January 2000     

Intravenous Immunoglobulin Therapy for Refractory Interstitial Lung Disease Associated with Polymyositis/Dermatomyositis

Interstitial lung disease (ILD) associated with polymyositis/dermatomyositis (ILD-PM/DM), including amyopathic dermatomyositis (ADM), is recognized as an important condition because it frequently causes death, despite intensive therapy with high-dose corticosteroid and immunosuppressive agents, such as cyclosporine A and cyclophosphamide. Intravenous immunoglobulin therapy (IVIG) has shown efficacy for myopathy associated with PM/DM, but its usefulness for ILD-PM/DM is unclear. This study was designed to investigate the efficacy of IVIG for refractory ILD-PM/DM. A review was made of medical charts of five patients (2 men and 3 women) who were treated with IVIG for refractory ILD-PM/DM resistant to high-dose corticosteroid and cyclosporine A and/or cyclophosphamide. One patient had acute ILD-PM and four patients had acute ILD-ADM. Of the five patients, one patient with ILD-PM and one patient with ILD-ADM survived. No adverse reactions were seen due to IVIG treatment. There were no critical differences in the clinical parameters and clinical courses between survivors and nonsurvivors. IVIG treatment is safe and could be an effective salvage therapy for refractory ILD-PM/DM in certain cases, suggesting that further controlled trials are worthwhile.     

Antitussive Effect of a Chest Band in Patients with Interstitial Lung Disease: The Preliminary Results from a Pre-post Intervention Study

Objective Evidence supporting the efficiency of clinically administered therapies against interstitial lung disease (ILD)-related cough is limited. Thus, we conducted a study to evaluate the efficacy of short-term use of chest bands on cough in patients with ILD. Methods This pre-post intervention study was performed at two university hospitals between April 2017 and August 2020. Scores of the visual analog scale (VAS) for cough severity (in terms of frequency and intensity), Leicester Cough Questionnaire (LCQ)-acute, and frequency scale for symptoms of gastroesophageal reflux disease (FSSG) were assessed before and after the use of the chest band (24/48 hours). Patients The study included patients with idiopathic interstitial pneumonias (IIPs) or connective tissue disease-associated interstitial lung disease (CTD-ILD). Results Four patients with IIPs and seven with CTD-ILD were included in the analysis. The cough intensity and LCQ-acute total score improved significantly after the use of the chest band (p=0.007 and p=0.005, respectively), although the cough frequency showed no significant reduction (p=0.074). Furthermore, the FSSG total and acid-reflux symptom scores improved (p=0.018 and p=0.027, respectively), and a negative correlation between the change in LCQ-acute total score and that in FSSG score for acid-reflux symptoms was observed (Spearman rho =-0.841, p=0.001). Conclusion The results of the current study suggest that chest bands might be useful for treating chronic refractory cough in patients with ILD and gastroesophageal reflux disease. However, these results should be interpreted with caution due to methodological limitations associated with this study.