Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis

Introduction

Increased frequencies of hyperuricemia and gout have been associated with primary hyperparathyroidism, and recent clinical trials of parathyroid hormone (PTH) have reported hyperuricemic adverse events. We evaluated the potential population impact of PTH on serum uric acid (SUA) levels by using a nationally representative sample of United States adults.

Methods

By using data from 8,316 participants aged 18 years and older in the National Health and Nutrition Examination Survey 2003 to 2006, we examined the relation between serum PTH and SUA levels with weighted linear regression. Additionally, we examined the relation with hyperuricemia by using weighted logistic regression.

Results

SUA levels increased with increasing serum PTH concentration. After adjusting for age, sex, dietary factors, glomerular filtration rate (GFR), and other potentially related biomarkers (calcium, phosphorus, alkaline-phosphatase, 25-hydroxyvitamin D), the SUA level differences from the bottom (referent) to top quintiles of serum PTH levels were 0, 8, 13, 14, and 19 ??M (95% CI, 12 to 26; P for trend, < 0.001). These estimates were larger among renally impaired individuals (multivariate SUA difference between the extreme quintiles of PTH, 26 versus 15 ??M among those with GFR ?? 60 versus < 60 ml/min per 1.73 m², respectively) (P for interaction = 0.004). The odds of hyperuricemia by various definitions increased with increasing PTH levels as well (multivariate P values for trend, < 0.05).

Conclusions

These nationally representative data indicate that serum PTH levels are independently associated with serum uric acid levels and the frequency of hyperuricemia at the population level.     

Anti-MJ/NXP-2 autoantibody specificity in a cohort of adult Italian patients with polymyositis/dermatomyositis

Introduction

Autoantibodies in patients with polymyositis/dermatomyositis (PM/DM) are associated with unique subsets, clinical course and outcome. Anti-MJ antibodies, which recognize the nuclear protein NXP-2/MORC3, are reported in ~25% of juvenile DM. Prevalence and clinical significance of anti-MJ antibodies in adult Italian PM/DM patients were studied.

Methods

Sera from 58 consecutive adult Italian PM/DM patients were analyzed by immunoprecipitation of ³⁵S-labeled K562 cells extract, ELISA (anti-MJ, Jo-1), Western blot and indirect immunofluorescence. Clinical associations were analyzed using information from medical charts.

Results

Anti-MJ antibodies were the most prevalent specificity (17%) found mainly in DM (30%, 8 cases) vs 8% of PM (2 cases, P = 0.02). Comparing 10 anti-MJ (+) vs 48 anti-MJ (-) cases, DM was more common (P = 0.03), and age at onset was younger in anti-MJ (+) (P = 0.0006). In anti-MJ (+), heliotrope rash (P = 0.01) and calcinosis (P = 0.09) were more frequent. None of them had heart or lung involvement, or malignancy. Myopathy in anti-MJ (+) patients responded well to therapy and none of them had elevated CPK at last visit (0% vs 25% in anti-MJ (-)). Only 60% of anti-MJ (+) showed immunofluorescent nuclear dots staining, despite PML localization of NXP-2/MORC3.

Conclusions

Anti-MJ antibodies are the most frequent specificity in our cohort of adult Italian PM/DM. Anti-MJ (+) were associated with young onset DM, calcinosis, no internal organ involvement and good response of myopathy to therapy. Anti-MJ reported in juvenile DM is also found in adult PM/DM, and could be a new useful biomarker.     

Severe perturbations of the blood T cell repertoire in polymyositis, but not dermatomyositis patients.

Polymyositis and dermatomyositis are diseases characterized by muscle weakness and muscle inflammatory infiltrates. Their pathogenesis remains unclear. A central role for endomysial autoaggressive CD8(+) T cells is suspected in polymyositis and for perivascular B cells in dermatomyositis. We compared the T cell repertoire of 10 polymyositis and 10 dermatomyositis patients by immunoscope, a method providing a global assessment of the T cell repertoire and a sensitive detection of clonal T cell expansions. Samples were analyzed qualitatively and quantitatively in the blood (unsorted cells and CD4(+) and CD8(+) cells) and in muscle infiltrates. Dramatic perturbations of the T cell repertoire were observed in the blood of polymyositis but not dermatomyositis patients (p < 0.0005), the latter being undistinguishable from controls. These perturbations were due to oligoclonal expansions of CD8(+) T cells and most blood clonal expansions were also found in muscle. These results indicate that the pathogenesis of polymyositis and dermatomyositis is different and reinforce the view that polymyositis but not dermatomyositis is an autoimmune CD8(+) T cell-mediated disease. Moreover, this method may be helpful for the differential diagnosis of polymyositis and dermatomyositis and for noninvasive follow-up of polymyositis patients.     

Cancer risks of dermatomyositis and polymyositis: a nationwide cohort study in Taiwan

Introduction  

The association of idiopathic inflammatory myositis (IIM) and malignancies has been reported, but rarely in Asian countries. Our aim was to investigate the risk of cancer among IIM patients without a prior history of malignancies, in Taiwan.     

Safety and possible effects of low-intensity resistance training associated with partial blood flow restriction in polymyositis and dermatomyositis

Introduction

Our aim was to evaluate the safety and efficacy of a low-intensity resistance training program combined with partial blow flow restriction (BFR training) in a cohort of patients with polymyositis (PM) and dermatomyositis (DM).

Methods

In total, 13 patients with PM and DM completed a 12-week twice a week low-intensity (that is, 30% one-repetition-maximum (1RM)) resistance exercise training program combined with partial blood flow restriction (BFR). Assessments of muscle strength, physical function, quadriceps cross sectional (CSA) area, health-related quality of life, and clinical and laboratory parameters were assessed at baseline and after the intervention.

Results

The BFR training program was effective in increasing the maximal dynamic strength in both the leg-press (19.6%, P <0.001) and knee-extension exercises (25.2% P <0.001), as well as in the timed-stands (15.1%, P <0.001) and timed-up-and-go test (−4.5%, P =0.002). Quadriceps CSA was also significantly increased after the intervention (4.57%, P =0.01). Similarly, all of the components of the Short Form-36 Health Survey, the Health Assessment Questionnaire scores, and the patient- and physician reported Visual Analogue Scale were significantly improved after training (P <0.05). Importantly, no clinical evidence or any other self-reported adverse event were found. Laboratory parameters (creatine kinase and aldolase) were also unchanged (P >0.05) after the intervention.

Conclusions

We demonstrated that a 12-week supervised low-intensity resistance training program associated with partial blood flow restriction may be safe and effective in improving muscle strength and function as well as muscle mass and health-related quality of life in patients with PM and DM.

Trial registration

Clinicaltrials.gov {"type":"clinical-trial","attrs":{"text":"NCT01501019","term_id":"NCT01501019"}}NCT01501019. Registered November 29, 2011.     

Serum level of soluble CX3CL1/fractalkine is elevated in patients with polymyositis and dermatomyositis, which is correlated with disease activity

Introduction

Polymyositis (PM) and dermatomyositis (DM) are chronic inflammatory muscle diseases, in which chemokines are thought to contribute to inflammatory cell migration into muscle. In this study, we retrospectively analyzed the expressions of CX3CL1/fractalkine and its corresponding receptor, CX3CR1, in muscle and lung with interstitial lung disease (ILD) of PM patients and DM patients, and determined the correlation between serum soluble CX3CL1 level and disease activity.

Methods

Expressions of CX3CL1 and CX3CR1 in muscle and lung tissue were analyzed by immunohistochemistry. Serum CX3CL1 concentrations were measured by ELISA. For evaluation of patients' disease activity, serum creatinine kinase, manual muscle testing, and the alveolar-arterial oxygen pressure difference were used independently.

Results

CX3CL1 was expressed on infiltrated mononuclear cells and endothelial cells in muscle affected by PM and DM and in lung with ILD, whereas CX3CR1 was expressed on some CD4⁺ T cells, a majority of CD8⁺ T cells, and most macrophages in muscle, and on infiltrated mononuclear cells in the lung. Serum soluble CX3CL1 was significantly higher in PM patients and DM patients than in healthy controls. The CX3CL1 level was correlated with serum creatinine kinase and manual muscle testing score. In patients with PM and DM with ILD, serum CX3CL1 was also correlated with alveolar-arterial oxygen pressure difference. Furthermore, CX3CL1 was significantly decreased after conventional treatment.

Conclusions

The interaction between CX3CL1 and CX3CR1 might contribute to the inflammatory cell infiltration into affected muscle and lung with ILD in PM patients and DM patients. Serum CX3CL1 level could be a surrogate marker of disease activity.     

Clinical features of ocular toxoplasmosis in Korean patients

We report here the records of 10 consecutive Korean patients (10 eyes) with ocular toxoplasmosis which showed the typical clinical manifestations with seropositivity for Toxoplasma gondii specific IgG antibodies by micro-ELISA between 2006 and 2010. Nine patients were males and 1 was female; their age was 50.5 ± 13.8 years. The most common accompanying signs were vitritis (100%), anterior uveitis (70%), and scattered white deposit (80%). Pre-existing retinochoroidal scar was found in 1 (10%) patient. All patients received antiparasitic chemotherapy and systemic corticosteroid treatment, which resolved the presenting attack and recovered the visual acuity better than initial one in 9 patients and worse in 1. Optic atrophy, cataract, and retinal neovascularization were observed during the follow-up period and recurrence was detected in 3 eyes (30%) 6 to 20 months after the initial attack. In Korea, although rarely detected and reported, ocular toxoplasmosis needs more attention in clinical field of retinal diseases.     

52例HIV／AIDS患者口腔念珠菌病的临床表型及病原学鉴定

目的：口腔念珠菌病（ oral candidiasis,OC）是HIV／AIDS患者中最常见的口腔疾病,研究其临床表型及病原学特点对AIDS相关性口腔念珠菌病的诊断和临床用药有重要的指导意义。方法将70例确诊为HIV／AIDS且初步诊断为AIDS相关性口腔念珠菌病患者使用棉拭子在其病损区取材,接种至科玛嘉念珠菌显色培养基检验。结果 HIV／AIDS患者念珠菌感染的病例为52例,临床表型以假膜型和红斑型为主,其中假膜型最为常见（56％）。 HIV／AIDS患者口腔念珠菌病各致病病原菌以白念珠菌的检出率最高（60．32％）,热带念珠菌次之（19．05％）,光滑念珠菌为（12．70％）,克柔念珠菌（7．94％）。有9例HIV／AIDS伴口腔念珠菌病患者,检测出病原菌的混合感染,其中2例为3种病原菌感染。结论 HIV／AIDS患者口腔念珠菌病临床表型复杂且可以伴发,单纯通过临床表现鉴别诊断常不准确,且病原菌的准确分型可以指导临床用药,降低耐药菌的产生。     

五步蛇伤患者早期伤口切开的临床特点及预后

目的 探讨早期伤口切开的五步蛇伤患者的临床特点及预后.方法 选择22例已行早期伤口切开(观察组)及35例未行早期伤口切开(对照组)的五步蛇伤患者的临床资料进行回顾性分析总结,并比较其临床特点及预后.结果 观察组患者伤口的肿胀及溃疡程度均轻于对照组(P<0.05);观察组患者行早期伤口切开常引起伤口严重出血,血红蛋白浓度较对照组显著降低(P<0.05);观察组患者的平均住院天数显著少于对照组(P<0.05).结论 早期伤口切开将加重五步蛇伤患者的出血,但对伤口状况有较好的改善作用.对五步蛇伤患者在使用足量抗蛇毒血清且凝血功能正常后或可行小切口切开减压引流排毒,利于伤口愈合.     

Clinical and laboratory features of 14 young Chinese probable sCJD patients

Sporadic Creutzfeldt-Jakob disease (sCJD) occurs frequently in the relatively older population, mainly in the groups of 60–69 and 70–79 year-old. Since 2006 when China performed national CJD surveillance, 14 young probable sCJD patients below 40 year-old were identified, counting for 1.93% of all probable sCJD cases. The clinical features of young probable sCJD cases, including the onset feature, the presence of sCJD-associated signs and the clinical duration, are indistinguishable from those of older patients. Special sCJD-associated abnormalities on EEG and MRI were noticed in 7 and 10 cases. CSF 14–3–3 was positive in 7 cases. CSF RT-QuIC showed positive reactive curves in 9 cases, with short lag phases. PRNP sequencing did not find any mutation. Due to low rate of brain autopsy in China, performances of other CJD-associated examinations as much as possible are extremely important for the distinguish diagnosis of young probable sCJD patients.     

Characteristics of the serologic antibacterial response of patients with diphtheria and of bacterial carriers

The enzyme immunoassay system for the detection of specific antibacterial antibodies to Corynebacterium diphtheriae membrane protein (mol. wt. 64 KD) has been used. The physicochemical nature of these antibacterial antibodies has been established and their quantitative characteristics have been determined. The analysis of the dynamics of the formation of antimicrobial IgG- and IgM-antibodies in diphtheria patients (on days 3-28 of the disease) has shown that by the time of convalescence the antibody level increases about 200-fold (p less than 0.0001). The possibility of using the dynamics of the formation of specific antimicrobial IgM-antibodies for the serodiagnosis of carrier state is shown, even if the increase of the level of antimicrobial IgG-antibodies is insufficient for this purpose (not more than twofold).     

宫腔镜或宫腹腔镜联合手术治疗子宫纵隔69例疗效观察

目的:探讨宫腔镜或宫腹腔镜联合在治疗子宫纵隔中的临床疗效。方法:对69例子宫纵隔患者行宫腔镜子宫纵隔电切术(26例)或腹腔镜监视下宫腔镜纵隔切开术(43例)的临床效果进行回顾性分析。结果:69例患者均一次手术成功,手术时间为20～105 min,平均手术时间(42.3±6.8)min;术中出血10～45 ml,平均出血量为(18.2±3.2)mL;其中无子宫穿孔、水中毒、感染及大出血等并发症,随访6～33个月,自然流产率由术前的71.4%降到术后的14.8%,不孕症患者术前为39.1%明显下降至11.6%。结论:宫腔镜或宫腹腔镜联合电切术治疗子宫纵隔安全、有效,能显著改善患者的妊娠结局,提高患者的妊娠率。     

Paracoccidioidomycosis: A comparative study of the evolutionary serologic,clinical and radiologic results for patients treated with ketoconazole or amphotericin B plus sulfonamides

A comparative study of two groups of patients with paracoccidioidomycosis was carried out with the objective of comparing the evolutionary serologic, clinical and radiologic results after 6, 12, 15 and 18 months of treatment with ketoconazole (22 patients) or amphotericin B plus sulfonamides (32 patients). The serologic data analyzed as a whole showed a tendency to sharper drops in antibody titers in the patients treated with ketoconazole. Clinically patients treated with ketoconazole fared better but the differences were not statistically significant. No statistical difference was detected between groups in terms of the results of radiologic evolution.