疑似皮肌炎的播散性嗜酸性细胞胶原病

报告1例疑似皮肌炎的播散性嗜酸性细胞胶原病。患者女,53岁。全身泛发暗褐色斑,干燥、皲裂伴瘙痒14个月,无肌肉症状。实验室及辅助检查提示,嗜酸性粒细胞增高、血清IgE升高,抗核抗体(ANA)阳性,CT示肺部多发斑片条索影,肺功能限制性通气功能障碍伴弥散功能减低,肌电图示肌源性损害,皮损组织病理提示符合结缔组织病。诊断:播散性嗜酸性细胞胶原病。     

Changes in cutaneous microcirculation, hemorrheology and platelet aggregation function in dermatomyositis.

Marked disturbance of microcirculation was observed in 85 cases of dermatomyositis: decrease in number and irregular pattern of the capillaries, many being tortuous and dilated and having a slow and granular blood stream, as well as an increase of vascular permeability. The changes were more marked in cases with visceral disorders, or in active disease; however, there was no correlation between these changes and the duration of illness. Blood hyperviscosity was found in an hemorrheological study. The cause is considered to be due to high plasma viscosity, high fibrinogen levels, and increase of aggregation of erythrocytes and platelets. Modulation of microcirculation is advisable in the treatment of dermatomyositis.     

Biomarkers in chronic kidney disease,from kidney function to kidney damage

Chronic kidney disease(CKD) typically evolves over many years, with a long latent period when the disease is clinically silent and therefore diagnosis, evaluation and treatment is based mainly on biomarkers that assess kidney function. Glomerular filtration rate(GFR) remains the ideal marker of kidney function. Unfortunately measuring GFR is time consuming and therefore GFR is usually estimated from equations that take into account endogenous filtration markers like serum creatinine(SCr) and cystatin C(Cys C). Other biomarkers such as albuminuria may precede kidney function decline and have demonstrated to have strong associationswith disease progression and outcomes. New potential biomarkers have arisen with the promise of detecting kidney damage prior to the currently used markers. The aim of this review is to discuss the utility of the GFR estimating equations and biomarkers in CKD and the different clinical settings where these should be applied. The CKD-Epidemiology Collaboration equation performs better than the modification of diet in renal disease equation, especially at GFR above 60 m L/min per 1.73 m2. Equations combining Cys C and SCr perform better than the equations using either Cys C or SCr alone and are recommended in situations where CKD needs to be confirmed. Combining creatinine, Cys C and urine albumin to creatinine ratio improves risk stratification for kidney disease progression and mortality. Kidney injury molecule and neutrophil gelatinase-associated lipocalin are considered reasonable biomarkers in urine and plasma to determine severity and prognosis of CKD.     

皮肌炎并发横纹肌溶解症及急性肾损伤一例

正皮肌炎是一种主要累及皮肤和横纹肌的自身免疫性结缔组织病,以红斑、水肿为皮损特点,伴有肌无力和肌肉炎症、变性的疾病,常伴有关节、心肌等多器官损害。皮肌炎本身所致的肾脏受损较少见,但皮肌炎可导致横纹肌溶解症,后者可并发急性肾损伤,肾功能衰竭一旦形成,病死率很高,预后极差。我院收治1例皮肌炎并发横纹肌溶解症及急性肾损伤的患者,现报告如下。临床资料患者,男,24岁。因全身皮损伴四肢无力10余     

SLE和皮肌炎患者下丘-垂体-甲状腺轴的研究

应用促甲状腺激素释放激素（ＴＲＨ）兴奋试验探讨３４例ＳＬＩ活动期患者、１３例ＳＬＥ静止期和８例皮肌炎患者下丘－垂体、甲状腺轴活动。结果显示,ＳＬＥ活动期血清促甲状腺激素（ＴＳＨ）和催乳素（ＰＲＬ）水平在０、１５、３０、６０分钟和１２０分钟与正常对照组及ＳＬＥ静止期比较显著升高,而静止校无显著性差异’;皮肌炎在０、１５、３０、６０分钟及１２０分钟与正常组比较有显著性差异,而皮肌炎各时点表ＴＳＨ水平与     

Long-term renal function,complications and life expectancy in living kidney donors

Living kidney transplantation is now a widely accepted treatment for end stage renal disease (ESRD) because it provides excellent outcomes for recipients. However, long-term outcomes of living kidney donors have not been well understood. Because securing the safety of the donor is essential to the continued success of this procedure, we reviewed articles discussing long-term outcomes of living kidney donors. Most studies found no decrease in long-term survival or progressive renal dysfunction in previous kidney donors. Moreover, the prevalence of hypertension was comparable to that expected in the general population, although some did report otherwise. Urinary protein showed small increases in this population and was associated with hypertension and a lower glomerular filtration rate. Quality of life following living kidney donation seems to be better than the national norm. We also encountered several reports of ESRD in previous living kidney donors. Regular follow-up of kidney donors is recommended and future controlled, prospective studies will better delineate risk factors which cause health problems following living kidney donation.     

儿童皮肌炎与成人皮肌炎患者临床和实验室资料对比分析

为了探讨儿童皮肌炎（JDMS）与成人皮肌炎（ADMS）临床表现上的差异，将35例JDMS和139例同期住院的ADMS患者的临床及实验室资料进行对照研究，结果显示JDMS男女比为1：1．3，而成人男女比为1：2．1，JDMS眼周紫红斑，Gottron's丘疹的高发生率及ADMS相似，但肌炎症状较轻，合并症较少，肌萎缩，吞咽困难，间质性肺炎，恶性肿瘤的发生率均明显低于成人患者（P＜0．05，P＜0．01，P＜0．01，P＜0．05），血清CPK，IgG升高率亦明显低于成人患者（P＜05，P＜0．01），而发热，上呼吸道感染的发生及抗“O”阳性率则明显高于成人患者（P＜0．01，P＜0．05，P＜0．01），结果提示JDMS与ADMS在发病机理及临床特点上均有差异，皮损特征对儿童皮肌炎的早期诊断更为重要。     

Acute kidney injury and the compensation of kidney function after nephrectomy in living donation

Acute kidney injury (AKI) incidence is growing rapidly, and AKI is one of the predictors of inpatient mortality. After nephrectomy, all the patients have decreased kidney function with AKI and recover from AKI. However, the characteristic and behavior of AKI is different from usual AKI and compensatory kidney function has been well known in the postoperative setting, especially in living donors. In this review, we have focused on the compensation of kidney function after nephrectomy in living donors. We discuss factors that have been identified as being associated with kidney recovery in donors including age, sex, body mass index, remnant kidney volume, estimated glomerular filtration rate, and various comorbidities.     

Clinical, serologic, and immunogenetic studies in patients with dermatomyositis.

Dermatomyositis is a disease of unknown etiology characterized by progressive, symmetrical, proximal muscle weakness with accompanying compatible cutaneous findings. Thirty-nine patients with dermatomyositis from the Louisville, Kentucky area were enrolled in this study. Patients were grouped into those with or without a malignancy. Ten patients (26%) either had or have had a malignancy. Twenty-five Caucasian patients were HLA typed for the A, B, DR and DQ locus antigens, of whom 5 had an associated malignancy and 20 did not have a malignancy. We found that no single antigen had a significantly increased or decreased frequency as compared with our control population for the entire group, or for any clinical subset we examined. Serologic testing revealed 4 patients with anti-Mi-2 antibodies and 1 patient with anti-PM-SCL antibodies. No patient had a positive anti-Jo-1 antibody in this group. The results of serologic tests in this group did not correlate with any clinical subset or HLA antigen. Our findings were in agreement with the previous reports in which approximately 25% of patients with DM have an associated malignancy. Our findings also support the notion that untargeted malignancy searches are not warranted. Contrary to previous reports we did not observe an inverse relationship between cancer and pulmonary disease in the dermatomyositis patient. This study does not indicate that there are any HLA associations or clinical associations, other than age, that distinguish patients with dermatomyositis as running a greater risk of developing malignancy.     

Circulating immune complexes in lupus erythematosus, scleroderma and dermatomyositis.

Circulating immune complexes (CIC) were measured by three different methods in serum from 17 patients with systemic lupus erythematosus (SLE), 3 patients with "hydralazine-induced" SLE-like syndromes, 14 patients with discoid lupus (DLE), 8 patients with systemic sclerosis and 5 patients with dermatomyositis. Immune complexes were detected in 13 of the 17 patients with SLE. All patients with lupus nephritis and typical exanthema had circulating immune complexes. The concentration of immune complexes was inversely correlated to serum complements C4 and C3. All 3 patients with "hydralazine-induced" SLE-like syndromes had circulating immune complexes that disappeared after withdrawal of the drug. Immune complexes were detected in 3 of the 14 patients with DLE; all 3 patients with CIC had wide-spread DLE. In systemic sclerosis, CIC were detected in only 1 of the 8 patients. Four of the 5 patients with dermatomyositis demonstrated CIC in serum. No complement consumption was detected in dermatomyositis and the immune complexes may have been secondary to tissue destruction.     

Terbinafine-induced dermatomyositis: a case report and literature review of drug-induced dermatomyositis

Dermatomyositis, a connective tissue disease syndrome where antibodies to the endothelium of the microvasculature of the skin, muscle and lung are implicated in lesional propagation, is characterized by photodistributed erythema, heliotrope rash, Gottron's papules, muscle weakness and interstitial pulmonary fibrosis. Endotheliotropic viruses and underlying neoplasia are among the inciting triggers. Uncommon drugs, namely the lipid-lowering agents, have been implicated in dermatomyositis. The patient, a 57-year-old man, developed a photodistributed rash and muscle weakness following treatment with the antifungal medication, terbinafine. A skin biopsy was performed, showing an atrophying interface dermatitis with pandermal mucinosis and striking vasculopathic changes including endothelial cell necrosis with denudement and basement membrane zone reduplication. Ultrastructural studies confirmed the presence of endothelial cell injury. Direct immunofluorescent testing showed prominent staining of C5b-9 along the dermal-epidermal junction and within the vasculature. Western blot studies showed strong seroreactivity of his serum to an endothelial-based protein weighing 45,000, a common target described in other microvascular injury-based syndromes. We have shown a temporal association between use of terbinafine and the development of dermatomyositis. The exact basis remains speculative. One potential hypothesis is based on the fact that terbinafine, the active agent in terbinafine, triggers apoptosis of human endothelial cells in culture. Enhanced endothelial cell apoptosis results in the displacement of various cellular antigens creating a state of neoantigenicity; its attendant sequelae is held to be one of anti-endothelial cell antibody formation, a defining pathogenetic event in the evolution of dermatomyositis. The second may be because of the effects of the drug on the promotion of an interferon-rich T-helper-1-dominant cytokine milieu.     

Pruritus in adult dermatomyositis

Dermatomyositis has a significant clinical component of pruritus that has not yet been studied. Pruritus can significantly affect the life of patients. The aim of the present work was to study the degree of pruritus experienced by patients. A four-question survey was sent to patients with documented dermatomyositis. The survey used a 100-mm Visual Analogue Scale (VAS) to describe current, worst and daily pruritus, and the effect this has on daily activities. Twenty-six subjects returned completed questionnaires: four had no pruritus; the majority had a significant amount with means above 50 on the VAS. A mean of 44.6 was found for the effect on daily life. Further studies should be performed to examine the prevalence and severity of pruritus in this population and it's effect on their quality of life. Clinicians must be aware of the significant pruritus and provide adequate therapy to improve quality of life.     

幼年型皮肌炎研究进展

幼年型皮肌炎(JDM)是一种发生于儿童、病因不明的自身免疫性疾病，其病因与遗传、自身免疫、感染及环境等因素相关。近来磁共振和高频超声波逐步用于该病的诊断和病情监测。治疗上应及早使用足量的糖皮质激素及免疫抑制剂，物理治疗亦是一种改善肢体功能的重要方法。JDM预后较成人皮肌炎好。     

皮肌炎自身抗体检测研究进展

一些自身抗体包括抗黑素瘤分化相关基因5抗体、抗转录中介因子1γ抗体、抗核基质蛋白2抗体、抗甘氨酰-tRNA合成酶抗体、抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体、抗组氨酰-tRNA合成酶抗体等与皮肌炎的临床表型和预后密切相关.抗黑素瘤分化相关基因5抗体、抗甘氨酰-tRNA合成酶抗体、抗组氨酰-tRNA合成酶抗体与皮肌炎并发间质性肺病有关,特别是抗黑素瘤分化相关基因5抗体与皮肌炎并发的间质性肺病的发生、病情活动和患者高死亡率显著相关.通常抗黑素瘤分化相关基因5抗体阳性的患者对常规的免疫抑制治疗疗效欠佳.抗转录中介因子1γ抗体、抗核基质蛋白2抗体与恶性肿瘤相关性皮肌炎有关.此外抗转录中介因子1γ抗体和抗核基质蛋白2抗体还与皮肌炎其他一些特征性临床表型相关,如抗转录中介因子1γ抗体阳性的患者发生银屑病样损害、手掌角化过度性丘疹明显增加,而抗核基质蛋白2抗体阳性的患者发生前臂、小腿和颈部无力更为常见.抗核小体重塑和组蛋白脱乙酰酶蛋白复合物抗体阳性患者发生间质性肺病风险和维持过长时间治疗的风险明显降低.因此临床上广泛地开展这些自身抗体的检测有助于指导皮肌炎的临床诊疗.