[Clinical characteristics and prognostic factors of patients with anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis associated interstitial lung disease].

Objective: To analyze the clinical characteristics and prognostic factors of patients with anti-melanoma differentiation-associated gene 5 (anti-MDA5)-positive dermatomyositis associated interstitial lung disease (DM-ILD). Methods: The patients with MDA5+DM-ILD who were admitted to Department of Respiratory Medicine, Nanjing Drum Tower Hospital from January 2017 to March 2021 were enrolled. The clinical data and survival information were analyzed retrospectively. Patients were divided into survival group or death group, and rapid progressive ILD (RP-ILD) group or non-rapid progressive ILD group, according to their survival status and clinical progression. Results: A total of 105 patients with anti-MDA5+DM-ILD (median age of onset 54 years) were enrolled, 58% being female (61 cases). The main sub-type of dermatomyositis was amyopathic dermatomyositis (n=74, 70%), followed by dermatomyositis (n=31, 30%). The main extrapulmonary manifestations were skin lesions (n=60, 57.1%), muscle manifestations(n=20, 19%) and arthralgia/arthritis (n=20, 19%). 15.4% of the patients had positive ANA (antibody titer≥1∶320), and 61.9% of the patients had anti-RO-52 kDa antibody. A total of 66 patients (62.8%) developed RP-ILD, and 58 patients (56.3%) died. Lower oxygenation index (OR=0.974, 95%CI:0.954-0.994, P=0.012) and no joint pain (OR=0.032, 95%CI: 0.002-0.663 P=0.026) were independent risk factors for RP-ILD. Cox regression analysis showed that RP-ILD (HR=3.194, 95%CI:1.025-9.954, P=0.045), older than 53 years (HR=3.450, 95%CI: 1.388-8.577, P=0.008), ferritin level more than 1 330.5 ng/ml (HR=3.032, 95%CI 1.208-7.610, P=0.018) and C-reactive protein (CRP) above 16.95 mg/L (HR=2.794, 95%CI:1.102-7.084, P=0.030) were independent predictors of mortality. Conclusions: The clinical manifestations of patients with anti-MDA5+DM-ILD presenting to the respiratory department were heterogeneous, with most being amyopathic dermatomyositis, and both the incidence of RP-ILD and the risk of death were high. Even in the absence of associated rash, joint, or muscle manifestations, anti-MDA5 antibody screening should be considered in patients with rapidly progressive ILD who were negative on baseline autoantibody screening but positive for anti-RO52kDa antibody.

[Process and reflections on the development of expert consensus on diagnosis and treatment of idiopathic inflammatory myopathy associated interstitial lung disease].

This paper introduced the following information on the purpose of the consensus, process, writing format, formation of expert opinions, and the issues that remain to be solved and reflections, hoping to provide reference for readers to understand the content of this consensus and rational choice of application.

[Idiopathic pleuroparenehymal fibroelastosis: five case reports and review of literature].

Objective: To raise the awareness of idiopathic pleuroparenehymal fibroelastosis (iPPFE) through investigating the clinical, radiographic and pathological features. Methods: Five cases of iPPFE proved by pathology. The clinical data were studied respectively, and the relevant literature was reviewed. Results: All the cases of iPPFE were manifested by cough and dyspnea. The patients including 3 males and 2 females, aged from 30 to 70 years Chest CT scan showed pleural thickening, subpleural consolidation in both upper lungs complicated with tractive bronchiectasis.Computed tomography-guided percutaneous lung biopsy or surgical lung were performed and the same pathological showed pleura and subpleural dense elastic and collagen fibers. The elastic fibers stain was also positive,which was consistent with PPFE. One patient received low-dose corticosteroid, two received pirfenidone therapy, the others received no treatment. Three patients were stable during the follow-up. Conclusions: iPPFE has characteristic pathological features. However, the number of clinically reported cases is low due to missed diagnosis or misdiagnosed. Improving the understanding of features of iPPFE is helpful for the dianosis, therapy, and prognosis of this disease.

[Clinical value of myositis antibodies in patients with connective tissue disease-associated interstitial lung diseases].

Objective: To investigate the clinical significance of detection of myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) in patients with connective tissue disease-associated interstitial lung diseases (CTD-ILD). Methods: Serum samples of 120 patients with CTD-ILD admitted to the Department of Respiratory, Affiliated Drum Tower Hospital of Nanjing University Medical College from December 2016 to April 2018 were collected for analysis. The patients included 45 with polymyositis/dermatomyositis (PM/DM), 36 with Sjogren's syndrome (SS) and 39 with undifferentiated connective tissue disease (UCTD). There were 37 males and 83 females with an average age of (56±11) years. Thirty-two patients with non-CTD-ILD, 10 males and 22 females with an average age of (42±17) years, were enrolled as the control group. Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs and MAAs, and the positive rates of serum MSAs and MAAs were calculated. The antibody distribution and clinical characteristics of different groups were analyzed and compared. Results: Eighty-nine of the 120 patients with CTD-ILD were positive for MSA and/or MAA (74.2%), and the detection rates of MSAs and MAAs were 52.5% (63/120) and 61.7% (74/120) respectively. No myositis antibody was detected in the non-CTD-ILD group. The detection rates of MSAs in PM/DM-ILD group, SS-ILD group and UCTD-ILD group were 75.6% (34/45), 33.3%(12/36) and 43.6%(17/39) respectively. The total detection rate of MSAs in PM/DM group was significantly higher than that in SS group and UCTD group (χ(2)=14.53, 8.95, 0.01). The anti-ARS was the most frequent (50/120, 41.7%). The positive rates of MAAs in the three groups were 64.4%(29/45), 77.8%(28/36), 43.6%(17/39) respectively, and anti-Ro-52 accounted for 60%(72/120), and were highly correlated with MSAs such as anti-Jo-1 antibodies. Conclusion: Myositis antibody profiling should be performed in patients with ILD who were negative for conventional autoimmune antibody testing and had no CTD. In patients with SS-ILD and UCTD-ILD, the myositis antibody spectrum could detect the presence of myositis-specific antibodies and myositis-related antibodies in some patients, and its role in clinical diagnosis and treatment needed further observation.

[The clinical significance of myositis-specific antibodies in polymyositis/dermatomyositis associated interstitial lung diseases].

Objective: To investigate the profile and clinical significance of myositis-specific antibody spectrum (MSAs) in patients with polymyositis/dermatomyositis-associated interstitial lung disease (PM/DM-ILD). Methods: Sera from 74 patients with PM/DM-ILD, 29 patients with SLE and 32 healthy controls were collected and Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs . The clinical data of all patients were collected from medical records. Statistical analysis was performed using One-way ANOVA, t-test, rank sum test, χ(2) test or Fisher's exact test. Results: The overall detection rate of MSAs in 74 patients with PM/DM-ILD was 86.5%, significantly higher than that in patients with SLE and healthy controls (χ(2)=66.24, 69.85, P<0.01). According to the diagnostic criteria of PM/DM, 18 of 74 patients were definitely diagnosed, 11 were preliminarily diagnosed and 45 were suspected, in which the detection rate of MSA was 83.3%, 90.9% and 86.7%, respectively .The detection rates of MSAs in 17 PM-ILD and 57 DM-ILD were 82.4% and 87.7% respectively. The anti-ARS and anti-MDA5 were the two most common subtypes of MSAs in patients with PM/DM-ILD, the positive rates being 59.5% and 25.7%, respectively . The incidence of CADM, acute/subacute ILD and 90-day mortality in the anti-MDA5 positive group (χ(2)=12.945, 23.203, 26.434, P<0.05) was significantly higher than those of the anti-ARS group and the MSA-negative group, while the incidence of helitrope rash, V-rash, fever was significantly higher than the anti-ARS positive group (χ(2)=11.462, 5.895, 10.609, P<0.05). The incidence of muscle weakness in anti-Jo-1 group was significantly higher than that in the non-Jo-1 antibody group (χ(2)=3.991, P<0.05), while other clinical features were not statistically significant between the anti-Jo-1 and the non-Jo-1 anti-ARS positive groups (P>0.05). Conclusion: The detection rate and accuracy of MSAs in polymyositis/dermatomyositis with ILD was very high, which was useful for early diagnosis of the disease, and severity and prognosis assessment. It is strongly recommended that MSAs should be detected in patients with clinical suspicion of PM/DM-associated interstitial lung diseases.

[Follicular bronchiolitis: report of 3 cases and literature review].

Objective: To improve understanding of the characteristics of follicular bronchiolitis(FB). Methods: The clinical data of 3 patients with FB confirmed by thoracoscopic lung biopsy were retrospectively analyzed. A literature search was performed with "follicular bronchiolitis" as the key word in China Knowledge Resource Integrated Database, Wanfang and PubMed, Ovid Database. The time interval was from January 1947 to December 2015. Related articles of FB were retrieved and the clinical, radiographic characteristics and prognosis were analyzed. Results: Among the 3 patients, 1 was male and 2 were female, aging 32-55 years. Two patients were asymptomatic, and 1 patient presented with fever, cough and dyspnea. Two patients showed normal pulmonary ventilatory function with decreased diffusive function, and 1 patient showed normal pulmonary function. The predominant HRCT findings were bilateral multiple small nodules and cystic opacities, patchy ground-glass opacities, reticular opacities and traction bronchiectasis. The pathological examination by thoracoscopic biopsy revealed bronchiolar and peribronchiolar lymphoid follicles. All patients were treated with corticosteroids, with 2 patients receiving immunosuppressants. Follow-up HRCT after 1-2 months showed no improvement, and further follow-up HRCT after 2-4 years revealed no change in 2 patients while the other patient had increased pulmonary nodules and cystic opacities. Seventeen articles concerning FB with complete records were included in the literature review. A total of 64 patients were reported in these articles. The typical images were bilateral multiple small nodules and ground-glass opacities, reticular opacities, and cystic opacities. The majority of patients improved after treatment of corticosteroids and (or) immunosuppressants. But our 3 cases showed no improvement. Conclusions: FB is a rare small airway disease which has non-specific clinical manifestations and pulmonary function. The most common imaging findings are bilateral multiple small nodules, with cystic opacities, ground-glass opacities, and reticular opacities. Surgical thoracoscopic biopsy can get ideal specimen which is useful for diagnosis. The curative effects of corticosteroids or immunosuppressants on FB need to be further clinically investigated.

[Pulmonary intravascular large B-cell lymphomamanifesting as interstitial pneumonias: report of 2 cases and review of literature].

Objective: To investigate the clinical, radiographic characteristics and prognosis of pulmonary intravascular large B-cell lymphoma(IVLBCL) manifesting as interstitial pneumonias on HRCT. Methods: A retrospective analysis was carried out on clinical data of 2 patients with pulmonary IVLBCL admitted to the Affiliated Drum Tower Hospital of Nanjing University from March 2010 to May 2012. A literature research was performed with "pulmonary intravascular lymphoma" as the key word in Wanfang Database, China Knowledge Resource Integrated Database and Pubmed. The time interval was from January 1980 to June 2015. Related articles of pulmonary IVLBCL were retrieved and the clinical, radiographic characteristics and prognosis were analyzed. Results: The first patient was a 69 year-old female presenting with ground-glass opacities, interlobular septal thickening and patchy consolidation on HRCT, for whom the diagnosis was confirmed by transbronchial lung biopsy (TBLB). The second patient was a 70 year-old male presenting with diffuse ground-glass opacities on HRCT, and the diagnosis was made by pathology on video-assisted thoracoscopic biopsy. The 2 patients all presented with dyspnoea, cough, fever and elevated lactate dehydrogenase(LDH). The pathological study of lung biopsy specimen demonstrated invasion of atypical lymphocytes into small vessels and capillaries. The tumor cells were positive for CD(20).Literature review found 19 articles, all case reports with a total of 22 patients. Conclusions: The clinical manifestation of pulmonary IVLBCL was nonspecific and the disease progressed rapidly. For patients with interstitial pneumonias on HRCT, pulmonary IVLBCL needed to be considered as a differential diagnosis and pathological studies should be obtained as soon as possible, so that better prognosis could be archived through early intervention.

[Unilateral pleural effusion as first manifestation in Takayasu arteritis: a case report and review of literature].

Objective: To highlight the characteristics of pulmonary arterial involvement in Takayasu arteritis. Methods: The clinical and radiological data of a patient with Takayasu arteritis presenting with unilateral pleural effusion were studied and relevant literature was reviewed. The key words, "Takayasu arteritis" and "pleural effusion" were analyzed through literature retrieval in databases. Results: This 58 year-old female patient presented with shortness of breath. The chest CT scan showed bilateral hilar enlargement and pleural effusion on the left side. The blood pressure was not measurable in the course of the disease. After the aorticopulmonary-arteriography, we found that the pulmonary artery and the subclavian artery were involved. The diagnosis of Takayasu arteritis was made, and glucocorticoid therapy was initiated, with significant clinical and radiological improvement after therapy. Literature review found 4 cases of Takayasu arteritis with unilateral pleura effusion, ranging from 32 to 35 years of age, with a female predominance(Female∶Male=3∶1). The chief complaints were fever, chest pain and hemoptysis. All the patients recovered after the treatment of glucocorticoids. Conclusions: Takayasu arteritis presenting with unilateral pleural effusion was easily misdiagnosed as primary pulmonary diseases. Careful physical examination and timely angiography can be used to make the diagnosis.

[Diagnosis of pulmonary hypertension secondary to obstructive lung disease by M-mode echocardiography contrasting with right cardiac catheterization].

Fourty five patients with COPD underwent right heart hemodynamics and M-mode echocardiographic study. 5 echocardiographic parameters were screened for diagnosing pulmonary hypertension. Whichever two or more parameters positive were regarded as diagnostic criteria, the sensitivity, specificity and correct rate of diagnosis were 71.43%, 85.00%, and 78.05% respectively. The hemodynamics basis of M-mode echocardiography used for diagnosis of pulmonary hypertension and cor pulmonale was provided in this paper.

[Clinical significance of soluble interleukin-2 receptor in patients with pulmonary tuberculosis].

The levels of soluble interleukin-2 receptor (sIL-2R) among patients with various pulmonary diseases were measured by using sandwich ELISA. The levels of sIL-2R were significantly elevated in patients with active pulmonary tuberculosis and acute pneumonia when compared with healthy controls. The concentrations of sIL-2R in patients with active pulmonary tuberculosis decreased in accordance with improvement of other laboratory parameters. The clinical significance and possible mechanism of increasing of sIL-2R in patients with pulmonary tuberculosis were discussed.

Both genes and lncRNAs can be used as biomarkers of prostate cancer by using high throughput sequencing data.

OBJECTIVE: To investigate prostate cancer-related genes and lncRNAs by using a high throughput sequencing dataset. MATERIALS AND METHODS: RNA-seq data were obtained from the sequencing read archive database, including both benign and malignant tumor samples. After aligning the RNA-seq reads to human genome reference, gene expression profile as well as lncRNA expression profile was obtained. Next, student's t-test was used to screen both the differentially expressed genes (DEGs) and lncRNAs (DELs) between benign and malignant samples. Finally, goseq was used to conduct the functional annotation of DEGs. RESULTS: A total of 7112 DEGs were screened, such as ZNF512B, UCKL1, STMN3, GMEB2, and PTK6. The top 10 enriched functions of DEGs were mainly related to organism development, including multi-cellular development, system development and anatomical structure development. Also, we discovered 26 differentially expressed lncRNAs. CONCLUSIONS: The analysis used in this study is reliable in screening prostate cancer markers including both genes and lncRNAs by using RNA-seq data, which provides new insight into the understanding of molecular mechanism of prostate cancer.

Effects of some Ca2+ blockers on receptor potentials of crayfish retina.

One drug known to inactivate calmodulin and two others to block Ca2+ channels were applied to isolated superfused retinae of the crayfish Astacus leptodactylus and their influence on the receptor potentials (RP) was studied. Trifluoperazine causes an increase of the amplitude of maximum (h max) of RP and a prolongation of the time of half decrease (t2). Furthermore, trifluoperazine could antagonise the effect of raised calcium concentration of superfusate, which decreases h max and shortens t2 of the RP. Iproveratril and D-600 cause a transient increase and thereafter a progressive decrease of h max of the RP. There is a difference between effects of both drugs on t2 of the RP. The mechanisms of effects of these drugs on the sensitivity of the isolated retina are discussed.