Characteristics and prognostic implications of peripheral blood lymphocyte subsets in patients with anti-MDA5 antibody positive dermatomyositis-interstitial lung disease.

OBJECTIVES: To examine the characteristics of blood lymphocyte subsets in dermatomyositis-interstitial lung disease (DM-ILD) inflicted patients with positive anti-melanoma differentiation-associated gene 5 (anti-MDA5), as well as its prognosis value in this set of patients. METHODS: Data were retrospectively collected from 253 DM-ILD patients from three hospitals in China between January 2016 to January 2021. Patients were grouped into anti-MDA5 antibody positive group (MDA5+ DM-ILD) and anti-MDA5 antibody negative group (MDA5- DM-ILD) based on myositis-specific autoantibody test results. Demographic characteristics, lymphocyte subsets patterns and other clinical features were compared between the two groups. The association of lymphocyte subsets with 180-day mortality was investigated using survival analysis in MDA5+ DM-ILD. RESULTS: Out of 253 eligible patients with DM-ILD, 59 patients were anti-MDA5+ and 194 were anti-MDA5-. Peripheral blood lymphocyte count, CD3+ count, percentage of CD3+, CD3+CD4+ count, and CD3+CD8+ count was lower in MDA5+ DM-ILD than in MDA5- DM-ILD- (all P < 0.001) as well as CD3-CD19+ count (P = 0.04). In MDA5+ DM-ILD, CD3+CD8+ count ≤ 49.22 cell/µL (HR = 3.81, 95%CI [1.20,12.14]) and CD3-CD19+ count ≤ 137.64 cell/µL (HR = 3.43, 95%CI [1.15,10.24]) were independent predictors of mortality. CD3+CD8+ count ≤ 31.38 cell/µL was associated with a higher mortality risk in all DM-ILD patients (HR = 8.6, 95%CI [2.12,31.44]) after adjusting for anti-MDA5 and other clinical characteristics. CONCLUSION: Significant lymphocytes decrease was observed in MDA5+ DM-ILD patients. CD3+CD8+ cell count was associated with worse prognosis in both MDA5+ DM-ILD and all DM-ILD patients.

Endoscopic classification and pathological features of primary intestinal lymphangiectasia.

BACKGROUND: The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia (PIL). AIM: To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine the patients' imaging and pathological characteristics of the patients, and explain their causes. METHODS: We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1, 2007 and December 31, 2018. We compared and analyzed all endoscopic images, classified them into four types according to the endoscopic features of the intestinal mucosa, and analyzed the post-lymphographic computed tomography (PLCT) and pathological characteristics of each type. RESULTS: According to the endoscopic features of PIL in 123 patients observed during endoscopy, they were classified into four types: nodular-type, granular-type, vesicular-type, and edematous-type. PLCT showed diffuse thickening of the small intestinal wall, and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular types. Contrast agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous types. Analysis of the small intestinal mucosal pathology revealed that nodular-type and granular-type lymphangiectasia involved the small intestine mucosa in four layers, whereas ectasia of the vesicular- and edematous-type lymphatic vessels largely involved the lamina propria mucosae, submucosae, and muscular layers. CONCLUSION: Endoscopic classification, combined with the patients' clinical manifestations and pathological examination results, is significant and very useful to clinicians when scoping patients with suspected PIL.

Clinical and microbiological characteristics of Cryptococcus gattii isolated from 7 hospitals in China.

BACKGROUND: Infection, even outbreak, caused by Cryptococcus gattii (C. gattii) has been reported in Canada and the United States, but there were sparsely-reported cases of C. gattii in China. Our interest in occurrence, clinical manifestation, laboratory identification and molecular characterization of Chinese C. gattii strains leads us to this research. RESULTS: Out of 254 clinical isolates, initially identified as Cryptococcus neoformans (C. neoformans), eight strains were re-identified as C. gattii. Multi-locus sequence typing (MLST) showed genotype VGI accounted for the most (6 / 8), the other two strains were genotype VGII (VGIIa and VGIIb respectively) with 3 specific spectra of molecular weight about 4342, 8686, 9611 Da by MALDI-TOF MS. The minimal inhibitory concentrations (MICs) of Fluconazole with Yeast one was 2~4 times higher than that with ATB fungus 3 and MICs of antifungal agents against VGII strains were higher than against VGI strains. Comparative proteome analysis showed that 329 and 180 proteins were highly expressed by C. gattii VGI and VGII respectively. The enrichment of differentially expressed proteins was directed to Golgi complex. CONCLUSIONS: Infection by C. gattii in China occurred sparsely. Genotype VGI was predominant but VGII was more resistant to antifungal agents. There was significant difference in protein expression profile between isolates of VGI and VGII C. gattii.

The mechanism of formation of thin-walled cystic lung cancer.

Thin-wall cystic lung cancer is becoming of increasing interest in the study of pulmonary medicine. Consequently, more and more different images and pathologic manifestations have been found. The purpose of this article is to find pathologic characteristics and try to explain the formation mechanism of thin-walled cystic lung cancer.Sixty-five patients with this special lung cancer were analyzed retrospectively based on the review of medical records, radiologic findings, and pathologic changes.We found 3 pathologic types: adenocarcinoma, squamous cell carcinoma, and lymphoma. There were 60 cases of adenocarcinoma, 4 cases were squamous cell carcinoma, and only 1 lymphoma. Tumor cells, pulmonary vessels, fibrous tissues, and residual bronchi are the pathologic basis of different image findings.Thin-walled cystic lung cancers are mostly adenocarcinoma, but other pathologic types can also appear, such as squamous cell carcinoma and lymphoma. We can see that a large amount of fibrous tissues were generated by tumors around the bronchus, resulting in airway stenosis and degeneration. Tumor cells also can invade the bronchial wall and cause structural damage. All these lesions are similar to 1-way valves which can cause gas accumulation in the tumor area and result in thin-walled cystic lung cancer.

Computed tomography for the diagnosis of solitary thin-walled cavity lung cancer.

BACKGROUND AND AIM: Lung cancer is the most commonly diagnosed neoplasm and the leading cause of cancer-related death worldwide. Despite the high incidence of lung cancer, the diagnosis of solitary thin-walled cavity lung cancer is rare. The aim of this review is to explore the potentials of computed tomography (CT) as diagnostic tool for solitary thin-walled cavity lung cancer. METHOD: The literature search was made in electronic databases including PudMed, Ovid SP, Embase, Web of Sciences, EBSCO and Wiley online by using relevant key terms. Because of the rarity of the subject, no precise exclusion or inclusion criteria were used for article selection and the outcome dissemination was decided to be more descriptive rather than quantitative. RESULTS: The detection of cavitation in lungs is frequently done utilizing chest radiographs CT scans. However, the diagnostic challenge remains the accurate detection of solitary thin-walled cavity lung cancer among the prevalence of cavitary lung lesions in multiple thoracic disorders including benign disorders, infectious disease and malignant tumors. Moreover, an accurate diagnosis of solitary thin-walled cavity lung cancer is further complicated by its subjective classification within the literature. In order to facilitate early diagnosis of this disease and circumvent the need for more invasive tests that may not be warranted, the overarching goal is to establish definitive radiological features of lung cavities that are indicative of malignancy. Herein, we describe the benefits of using CT to identify and diagnose solitary thin-walled cavity lung cancer, as well as explore the underlying mechanisms that contribute to thin-walled cavity formation in oncology patients. CONCLUSION: CT is the best modality for the noninvasive differentiation between malignant and nonmalignant cavities as it provides reliable information regarding the morphology and density of lesions. Besides, CT densitometry can efficiently detect the calcifications in lesions.

Pulmonary arterial hypertension and microRNAs--an ever-growing partnership.

Pulmonary arterial hypertension (PAH) is a debilitating condition with progressive remodeling of the pulmonary resistance vessels. PAH is characterized by multifocal, polyclonal lesions inhabited by cells that underwent phenotypic transition, resulting in altered cell proliferation and contractility, ultimately resulting in increased vascular resistance. Diagnosis of PAH is confounded by the fact that it is largely asymptomatic in the initial stages. In fact, idiopathic PAH patients >65 years of age cannot be diagnosed hemodynamically due to high pulmonary capillary wedge pressure. This highlights the need for defining more robust molecular biomarkers for PAH diagnosis and progression. Recent studies have indicated that microRNAs (miRNAs), a class of small noncoding RNAs that regulate gene expression, play a discrete role in vascular inflammation and in the etiology of cardiovascular pathologies inclusive of PAH and can potentially serve as diagnostic biomarkers. However, a cohesive understanding of global miRNA-mediated molecular events that control pulmonary vasculature plasticity is lacking which, if addressed systematically, can lead to detailed elucidation of the downstream cellular pathways that are affected by activation/silencing of silenced cognate transcripts. In turn, this can lead to not only robust biomarkers, but also to novel therapeutic strategies targeting more upstream regulators than the existing ones targeting more downstream effectors. The current review aims to provide a summary understanding of PAH, its associated pathophysiology, current knowledge of the role of miRNAs in PAH, and identifies grey areas that need further research for successful bench-to-bedside transition of these exciting new discoveries.

Exogenous pulmonary surfactant for acute respiratory distress syndrome in adults: A systematic review and meta-analysis.

Acute respiratory distress syndrome (ARDS) is often characterized by reduced lung compliance, which suggests dysfunction of the endogenous surfactant system. The effectiveness of exogenous surfactants as replacements for the endogenous system in the treatment of ARDS in adults was assessed. Randomized controlled trials from Medline (1950-2011), Embase (1989-2011), the Cochrane Database of Systematic Reviews and the Cochrane Central Register of Controlled Trials (1994-2011) were analyzed. Two reviewers identified trials for inclusion and the results of included trials were quantitatively pooled with a fixed-effects model. Seven trials (2,144 patients) with good methodological quality were included in the analysis. Pulmonary surfactant treatment was not associated with reduced mortality [relative risk (RR), 1.00; 95% confidence interval (CI) 0.89-1.12]. Subgroup analysis revealed no reduced mortality for various surfactant types. Heterogeneity was not significant in the primary outcome analysis (I(2)=0%). There was no evidence of publication bias. Oxygenation, ventilation-free days, duration of ventilation and APACHE II scores did not undergo pooled analysis due to insufficient data. Exogenous surfactant did not reduce mortality in adults with ARDS in our meta-analysis, and we cannot accurately define whether exogenous surfactant has an effect on oxygenation from the included studies.

Mimicking pulmonary multiple metastatic tumors: A case of primary nodular parenchymal pulmonary amyloidosis with review of the literature.

Primary pulmonary amyloidosis is a relatively rare condition, characterized by amyloid deposition in the lungs and other associated structures. We report a case of primary nodular parenchymal pulmonary amyloidosis in a 44-year-old male. The patient was referred to our hospital for further evaluation of multiple lobulated nodules in both lungs. As the multiple lung nodules were suspected to be metastatic, (18)F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) was conducted, which revealed that the nodules had a mild uptake of (18)F-FCG. Amyloidosis was confirmed by conducting a percutaneous CT-guided fine-needle aspiration (FNA) biopsy in the left lung nodule. A literature review of previous studies on primary nodular parenchymal pulmonary amyloidosis from Medline (1970-October 2011) and Embase (1989-October 2011) was also included. Despite its rarity, primary nodular parenchymal pulmonary amyloidosis with a pattern of multiple nodules also forms part of the differential diagnosis of pulmonary metastases with high (18)F-FDG uptake on PET/CT.