The emerging role of ultrasound in detecting interstitial lung disease in patients with rheumatoid arthritis

ObjectiveTo investigate the potential role of US in the detection of ILD in a cohort of patients with RA.MethodsPatients with diagnosis of RA were consecutively enrolled. All patients underwent pulmonary examination, laboratory data, DLCO measure, chest HRCT and radiographs, and US examination. A healthy group was included as control group. US was performed according the 14-intercostal space scanning protocol using the following semiquantitative scale [0 = normal (≤ 5 B-lines); 1 = slight (≥ 6 and ≤ 15 B-lines); 2 = moderate, (≤ 16 and ≥ 30 B-lines); 3 = severe (≥ 30 B-lines)].ResultsA total of 74 RA patients and 74 healthy controls were included. Thirty of 74 patients (40.5%) showed US signs of ILD with respect to the healthy controls (3 subjects, 4.1%) (P < 0.001); whereas HRCT showed ILD in 27 (36.4%) of 74 patients. Among the 30 patients that showed US findings of ILD, 17 (56.6%) were asymptomatic from respiratory view-point. The sensitivity and specificity of US were 92% and 89% respectively. A positive correlation between US and HRCT findings were found (P < 0.001) whereas no correlation was found with chest radiographs and DLCO findings. Positive association between US findings and DAS28-ESR, anti-CCP and RF (P < 0.01 for each respectively) was found. Feasibility, represented by the mean time spent to perform the pulmonary US assessment was 7.8 minutes (± SD 1.2, range 6 to 10 minutes).ConclusionsOur results support the potential of US in detect accurately ILD in patients with RA and provide a rationale to consider it as a friendly screening tool to be implemented in early phases of the disease.     

女性系统性红斑狼疮患者骨代谢异常的危险因素分析

摘要：目的 探讨骨代谢、骨密度与外周血淋巴细胞亚群的关系，初步探索免疫细胞在系统性红斑狼疮( systemic lupus erythematosus，SLE)骨代谢异常中的作用。方法 采用双能X线吸收法 （dual energy X-ray absorptiometry, DEXA ）检测SLE患者的骨密度，同日检测患者的外周血淋巴细胞亚群分布。结果 共纳入92名SLE女性患者，患者的平均年龄及病程分别为（42.1±14.1）岁、（84.4±73.0）个月。有62.0 %（n=57）患者存在骨代谢异常，其中44.6 %（n=41）患者为骨量减少，17.4 %（n=16）患者并发骨质疏松症，后者还包括9.8 %（n=9）发生脆性骨折的患者。全身骨密度最低值与年龄（r= –0.291，P=0.005）、病程（r=–0.239，P= 0.022）、绝经状态（r= –0.288，P=0.005）及碱性磷酸酶（r= –0.221，P= 0.033）均呈负相关。Logistics分析显示绝经状态（OR=13.0，P＜0.001，95% CI 3.43~49.5）、LDL-C（OR =5.74，P=0.002，95% CI 1.93~17.0）及外周血CD4+T细胞（OR =1.08，P = 0.025，95% CI 1.01~1.16）是骨代谢异常的影响因素，而年龄（OR=1.12，P＜0.001，95% CI 1.06~1.19）是骨质疏松的影响因素。结论 SLE患者骨代谢异常的发生率高，外周血淋巴细胞亚群分布可能与其发病存在相关性。     

Aortitis and periaortitis: The puzzling spectrum of inflammatory aortic diseases

Aortitis and periaortitis are inflammatory diseases of the aorta and its main branches; they differ in the extension of inflammation, which is confined to the aortic wall in aortitis, and spreads to the periaortic space in periaortitis. Aortitis is classified as non-infectious or infectious. Non-infectious aortitis represents a common feature of large-vessel vasculitides but can also be isolated or associated with other rheumatologic conditions. Periaortitis can be idiopathic or secondary to a wide array of etiologies such as drugs, infections, malignancies, and other proliferative diseases. Notably, both aortitis and periaortitis may arise in the context of IgG4-related disease, a recently characterised fibro-inflammatory systemic disease. Prompt recognition, correct diagnosis and appropriate treatment are essential in order to avoid life-threatening complications.     

不同性别系统性红斑狼疮患儿的临床特征及随访比较

目的 对不同性别系统性红斑狼疮（SLE）患儿的临床特征及随访情况进行比较。方法 回顾性分析2008~2018年确诊为SLE的≤14岁患儿79例（男18例，女61例）的临床资料，对男女儿童首发及主要临床症状、器官/系统损害情况、相关实验室指标、随访情况等进行对比研究。结果 首发症状及非首发症状中，男童组以发热发生率最高，女童组以面部红斑发生率最高。男童更易出现肾脏和血液系统的损害（P < 0.05），其中蛋白尿发生率较女童明显升高（P < 0.05）。女童更易出现关节痛（P < 0.05）。两组抗核抗体、抗双链DNA抗体、补体C3、红细胞沉降率的异常率均很高（>80%，P > 0.05）。男童组的疾病活动度在首次就诊及随访至第9年时高于女童组（P < 0.05）。随访结果显示（随访时间1个月至10年），在男童组中，3例失访，1例死亡，7例病情控制良好，但均需口服较大剂量激素或免疫抑制控制病情，2例发展为慢性肾功能衰竭，1例出现狼疮脑病。女童组中，3例失访，5例死亡，34例病情控制良好，其中口服醋酸泼尼松10 mg以下维持的有5例，停药1年的1例，停药2年的2例；4例出现狼疮脑病；1例在患病后第7年出现抑郁焦虑状态并有自杀倾向；2例出现视力下降、视物模糊、绿视；1例在服用激素第3年出现双侧股骨头缺血坏死。结论 不同性别SLE患儿的临床特征、部分实验室指标及预后存在差异。男性SLE患儿可能起病更重，更易发生肾脏及血液系统损害，且远期预后可能更差。女性SLE患儿可能更易累及关节。     

Patent ductus arteriosus and cerebral,cardiac, and gut hemodynamics in premature neonates

Patent ductus arteriosus is associated with multiple comorbidities in premature infants, however a causal link or strategy to decrease these morbidities has not been found. The association between the patent ductus arteriosus and morbidities has biologic plausibility as, like any cardiac mixing lesion, a significant systemic to pulmonic shunt may lead to pulmonary over-circulation and systemic hypoperfusion. Understanding the underlying pathophysiology of associated morbidities in the setting of a patent ductus arteriosus may aid in risk stratifying infants and offer a patient targeted approach to infants with a pathological ductal shunt. While the deleterious impact of increased pulmonary blood flow maybe easier to identify, the impact on end-organ perfusion is more challenging. In this review, we will discuss the pathophysiology of a hemodynamically significant patent ductus arteriosus in premature infants, impact on end-organ perfusion and associated morbidities, and novel modalities to assess shunt volume and effect on end-organ perfusion.     

Scarring alopecia in chronic cutaneous lupus erythematosus with neutrophils: A new scenario with therapeutic connotations

The relationship between autoinflammatory and autoimmune conditions has been demonstrated in recent decades. Several autoimmune conditions exhibit an autoinflammatory component, which can manifest in various ways. Neutrophilic dermatosis in the context of lupus erythematosus (LE) is one example. Otherwise, neutrophils are rare in LE, except for the bullous variant and nonbullous neutrophilic LE. In this paper, we describe a case of scarring alopecia due to LE that stopped responding to a treatment that had been effective for years. The biopsy specimen demonstrated the presence of neutrophils in the inflammatory infiltrate. A treatment with dapsone was prescribed and yielded rapid improvement. This first case of scarring alopecia in the context of nonbullous neutrophilic LE emphasizes the importance of the infiltrate in determining the optimal therapeutic choice.     

Update on vasculitis: an overview and dermatological clues for clinical and histopathological diagnosis – part I

The term vasculitis refers to the inflammation of vessel walls. It may range in severity from a self-limited disorder in one single organ to a life-threatening disease due to multiple organ failure. It has many causes, although they result in only a few histological patterns of vascular inflammation. Vessels of any type and in any organ can be affected, a fact that results in a broad variety of signs and symptoms. Different vasculitides with indistinguishable clinical presentations have quite different prognosis and treatments. This condition presents many challenges to physicians in terms of classification, diagnosis, appropriate laboratory workup, and treatment. Moreover, it compels a careful follow-up. This article reviews the Chapel-Hill 2012 classification, etiology, recent insights in pathophysiology, some important dermatological clues for the diagnosis and summarizes treatment of some of these complex vasculitis syndromes.