Objective To investigate independent prognostic factors influencing the prognosis of connective tissue disease-associated interstitial lung disease with pulmonary hypertension (CTD-ILD-PH), and construct a nomogram model using machine learning to predict 1-, 3- and 5-year mortality risks, providing evidence for clinical diagnosis and treatment. Methods Patients diagnosed with CTD-ILD-PH and treated at the First Affiliated Hospital of Zhengzhou University from February 2011 to June 2021 were screened. The least absolute shrinkage and selection operator (Lasso), univariate Cox regression, and multivariate Cox regression analyses were combined to identify independent prognostic factors for CTD-ILD-PH patients. A novel nomogram prognostic model was constructed and internally validated using 1000 bootstrap resamples. The receiver operating characteristic (ROC) curve and Harrell's C-index assessed the predictive performance of the model. Calibration curves evaluated the model fit. Decision curve analysis (DCA) assessed the clinical utility of the model, and external validation was conducted using a separate test set. Results The study included 313 patients, with 108 deaths observed during the follow-up. Using the Lasso-Cox method, albumin, alanine aminotransferase (ALT), red cell volume distribution width (RDW), age, smoking history, rural residence, and pulmonary artery systolic pressure were identified as independent prognostic factors. The Harrell's C-index in the training set was 0.802, and the area under ROC curve was 0.880 (95%CI 0.833 - 0.928). Internal validation showed an average Harrell's C-index of 0.791. Calibration curves indicated high consistency between predicted and observed results. DCA confirmed the model's good clinical utility. External validation results demonstrated the model's favorable predictive performance and clinical utility. Conclusions Our research suggest that lower albumin, elevated ALT, elevated RDW, advanced age, smoking history, rural areas and higher pulmonary artery systolic blood pressure are independent prognostic risk factors for patients with CTD-ILD-PH. In this study, a prognostic model was developed for the first time to predict 1-, 3- and 5-year mortality in CTD-ILD-PH patients, which provides some reference value for future mortality risk assessment in CTD-ILD-PH.
Objective To investigate the effect of prone position ventilation (PPV) on hemodynamics in patients with interstitial lung disease (ILD) concurrent with acute respiratory distress syndrome (ARDS) or not. Methods Severe ARDS patients received PPV treatment in intensive care unit from 2013 to 2015 were retrospectively analyzed. Pulse index continuous cardiac output (PiCCO) monitoring indices were compared 2h before PPV (PPV-2), in-PPV (PPVmean) and 2h after turn back to supine position (PPV+2), including mean arterial pressure (MAP), cardiac index (CI), intrathoracic blood volume index (ITBVI), etc. Heart function, liver function, renal function, coagulative function were compared before and after all the PPV treatment. Results Thirty-six severe pulmonary ARDS patients were recruited. Norepinephrine (NE) dose was (0.14±0.10) μg·kg-1·min-1 and lactate concentration was (1.70±0.57) mmol/L before PPV. CI in all the patients was increased [(4.06±0.95) L·min-1·m-2 vs. (3.98±1.05) L·min-1·m-2, P > 0.05) and central venous oxygen saturation, ITBVI [(76.94±8.94)% vs. (70.67±8.23)%, (982.17±245.87) mL/m2 vs. (912.97±177.65) mL/m2, P < 0.05)] were significantly increased compared with PPV-2, while heart rate (HR), MAP, urine volume were decreased (P > 0.05). The patients were divided into an ILD group (n=17) andanon-ILD group (n=19),and no significant differences were found in baseline data between two groups. Compared with PPV-2, MAP was significant decreased at PPV+2 [(85.44±10.84) mm Hg vs. (89.21±10.92) mm Hg, P < 0.05) in the ILD group. Compared with PPVmean, CI was obviously declined at PPV+2 in the ILD group. Activated partial thromboplastin time was increased [(67.04±97.52)s vs. (41.24±8.72) s, P < 0.05] and BUN was higher [(10.64±4.95) μmol/L vs. (8.18±3.88) μmol/L, P < 0.05] in the ILD group, while no significant difference was found in the non-ILD group. Conclusion PPV will not affect cardiac output in severe ARDS patients with ILD. It can increase venous return and improve tissue perfusion, but the perfusion of the abdomen organ need to be monitored cautiously.
ObjectiveTo translate the King’s Brief Interstitial Lung Disease (K-BILD) to Chinese, so as to provide an well reliability and validity assessment instrument for health status of patients with interstitial lung disease.MethodsBrislin’s transition model, six expert’s panel and pre-survey were used for initial Chinese version of K-BILD. Items analysis, exploratory factor analysis (EFA), confirmatory factor analysis (CFA), internal consistency reliability and test-retest reliability were used for validity and reliability test with 122 respondents.ResultsTen-item Chinese version of K-BILD were proved to have great psychometric qualities, two factors were extracted by EFA, which could explain 63.35% of the total variance. Furthermore, the CFA demonstrates the fit indices of two-factors mode: χ2/df=0.797, RMSEA=0.000, NFI=0.848, IFI=1.048, CFI=1.000, TLI=1.071. Cronbach’s α and Guttman Split-half were 0.893 and 0.861, respectively. Besides, the test-retest reliability of the scale was 0.805.ConclusionThe Chinese version of K-BILD scale has good validity and reliability, which is applicable for health status assessment in patient with interstitial lung disease.
Objective To summarize the clinical characteristics of pneumocystis pneumonia (PCP) secondary to interstitial lung disease (ILD) to improve the prophylaxis and management level of clinicians. Methods The clinical data of 50 patients with PCP secondary to ILD in the Department of Respiratory and Critical Care Medicine of Nanjing Drum Tower Hospital from January 2015 to December 2022 were collected. SPSS 26.0 software was used for statistical analysis. Results A total of 50 patients with PCP secondary to ILD were screened. Among the 50 patients, there were 23 males and 27 females, with a median age of 64 years old. Forty-eight cases (96%) had a history of glucocorticoid therapy with the median duration of 3 months; 31 (77.5%, 31/40) cases developed PCP in the first 6 months after glucocorticoid therapy; 34 cases had a history of glucocorticoid and immunosuppressants at the same time. None of the 50 ILD patients used drugs for PCP prophylaxis before developing PCP. The major clinical manifestations of PCP secondary to ILD were worse cough and shortness of breath or fever. Laboratory results showed 38 cases (76.0%) had peripheral blood total lymphocyte count <200/µL, 27 cases (54.0%) had CD4+ T cell count <200/µL, 34 cases (68.0%) had CD4+ T cell count <300/µL, 37 cases (74.0%) had CD3+ T cell count <750/µL, 34 cases (68.0%) had β-D-glucan test >200 pg/mL, 35 cases (70.0%) had lactic dehydrogenase > 350 U/L and 41 cases (82.0%) had type Ⅰ respiratory failure. High resolution computed tomography showed added ground-glass opacity and consolidation on the basis of the original ILD. Thirty-six cases were detected the Pneumocystis jirovecii by metagenomic next-generation sequencing with broncho-alveolar lavage fluid as the main source, and 2 cases by smear microscopy. All patients were treated with trimethoprim-sulfamethoxazole. After treatment, 29 cases were discharged with a better health condition, 10 cased died, and 11 cases left hospital voluntarily because of treatment failure or disease deterioration. Conclusions After the use of glucocorticoid and immunosuppressants, ILD patients are susceptible to life-threatening PCP. It is particularly important to make an early diagnosis. Attention should be paid to integrate the symptoms, levels of peripheral blood lymphocyte count, β-D-glucan test, lactic dehydrogenase and imaging findings to make an overall consideration. It is suggested to perform next-generation sequencing with broncho-alveolar lavage fluid at an early stage when patients can tolerate fiberoptic bronchoscopy to avoid misdiagnosis and missed diagnosis. ILD patients often develop PCP in the first 6 months after using glucocorticoid and immunosuppressants. During follow-up, peripheral blood CD4+ and CD3+ T cell count should regularly be monitored so as to timely prevent PCP.
Objective To investigate the constituent ratio and clinical features of diffuse interstitial lung disease（DILD） in Chongqing city.Methods Data was collected from all patients diagnosed as DILD in five comprehensive teaching hospitals in Chongqing from 2002 to 2006.The disease constituent ratio of DILD in the respiratory department or in the whole hospital was analysised.Meanwhile the clinical manifestation and the laboratory examination such as lung function and biopsy were also analysised.Results Mean age of DILD patients is （61.65±13.31）years with a ratio male to female of 1.83.The constituent ratio of DILD were 2.83‰ in respiratory department and 0.30‰ in hospital in 2002，and increased to 8.29‰ and 0.48‰ respectively in 2006.Graticule（62.20%）and honeycombing（24.50%） were predominant imaging manifestations.47.55% patients had restrictive lung function impairment，and 51.05% had mixed lung function impairment.Pathologic examination revealed UIP as the most common type，however，81.82% cases could not be classified to any category pathologically.Conclusions The prevalence of DILD increased progressively from 2002 to 2006.A definite diagnosis of DILD demands cooperation of physicians，radiologists and pathologists.
Objective To study the risk factors of developing progressive pulmonary fibrosis (PPF) within one year in patients diagnosed with rheumatoid arthritis-associated interstitial lung disease (RA-ILD), and develop a nomogram. Methods A retrospective study was conducted in 145 cases of RA-ILD patients diagnosed and followed up in the Affiliated Hospital of Qingdao University from January 2010 to October 2022. Among them, 106 patients and 39 patients were randomly assigned to a training group and a verification group. The independent predictors of PPF in patients with RA-ILD within one year were determined by univariate and multivariate logistic regression analysis. Then a nomogram is established through these independent predictive variables. Calibration curve, Hosmer-Lemeshow test, receiver operating characteristic (ROC) curve and area under ROC curve (AUC) and clinical decision curve were used to evaluate the predictive efficiency of the nomogram model for PPF in RA-ILD patients within one year. Finally, internal validation was used to test the stability of the model. Results Of the 145 patients with RA-ILD, 62 (42.76%) developed PPF within one year, including 40 (37.7%) in the training group and 22 (56.41%) in the verification group. The PPF patients had higher proportion of subpleural abnormalities, higher visual score of fibrosis and shorter duration of RA. Logistic regression analysis showed that the duration of rheumatoid arthritis (RA), visual score of fibrosis and subpleural abnormality were independent risk factors for the occurrence of PPF within one year after diagnosis of RA-ILD. A nomogram was constructed based on these independent risk factors. The AUC values of the training group and the verification group were 0.798 (95%CI 0.713 - 0.882) and 0.822 (95%CI 0.678 - 0.967) respectively, indicating that the model had a good ability to distinguish. The clinical decision curve showed that the clinical benefit of PPF risk prediction model was greater when the risk threshold was between 0.06 and 0.71. Conclusion According to the duration of RA, the visual score of fibrosis and the presence of subpleural abnormalities, the predictive model of PPF was drawn to provide reference for the clinical prediction of PPF in patients with RA-ILD within one year after diagnosis.