ObjectiveTo analyze the benefits of lung transplantation in the treatment of interstitial lung disease (ILD) and investigate its prognostic factors.MethodsThe clinical data of patients diagnosed with ILD and meet the lung transplantation criteria were retrospectively analyzed from January 2012 to December 2017 in the First Affiliated Hospital of Guangzhou Medical University. A total of 111 patients, 88 males and 23 females, aged (58.3±11.4) years old, were divided into lung transplantation group and non-lung transplantation group. Clinical data and prognosis of the two groups were compared and the factors affecting the prognosis of lung transplantation were analyzed with relevant literatures. Results There were 56 patients in lung transplantation group and 55 patients in non-lung transplantation group. The mainly underlying disease of both groups were idiopathic pulmonary fibrosis (IPF). There was no significant difference in age, body mass index, arterial partial pressure of oxygen, percentage of forced vital capacity in the estimated value, percentage of diffusing capacity of the lung for carbon monoxide in the estimated value, six-minute walk distance between the two groups (P>0.05). The pulmonary arterial hypertension and arterial partial pressure of carbondioxide were higher in lung transplantation group than non-transplantation group (P<0.05). The 1-year survival rate in the lung transplantation group was significantly higher than that in the non-lung transplantation group: 77.4% vs. 32.7% (P<0.01). COX regression analysis showed that preoperative ventilator dependence, serum creatinine, bilirubin, pulmonary artery pressure, and procedures (single lung vs. double lung) had no significant effect on the prognosis of lung transplantation; age and preoperative diabetes mellitus were risk factors for the prognosis of lung transplantation.ConclusionsLung transplantation can significantly improve the prognosis of patients with ILD who are refractory to medicine therapy. IPF patients should be advised to consider lung transplantation as soon as possible. Age and preoperative diabetes mellitus are risk factors for the prognosis of lung transplantation.
Objective To evaluate the clinical relationship between serum carcinoembryonic antigen (CEA) and mortality of anti-melanoma differentiation associated gene 5 (MDA5) antibody positive dermatomyositis with interstitial lung disease (ILD). MethodsThe consecutive clinical data of 214 patients with anti MDA5 antibody positive dermatomyositis from West China Hospital of Sichuan University from February 2017 to September 2019 were collected retrospectively, including demographic, laboratory examination and imaging examination data. Patients were divided into CEA elevated group (CEA≥4.63 ng/mL) and CEA normal group (CEA<4.63 ng/mL) according to CEA level. R4.1.2 software was used for statistical analysis of all data, and Kaplan Meier method was used to draw the survival curve. Cox proportional hazard model was used to analyze the survival of patients with ILD, and to explore the risk factors associated with the survival of patients with anti-MDA5 antibody positive dermatomyositis with ILD. Results There were 180 patients with ILD who met the inclusion and exclusion criteria, 57 patients with rapidly progressive pulmonary interstitial fibrosis (RPILD), and 123 patients without RPILD; 121 women and 59 men, with an average age of 50.2±10.7 years; The average follow-up was 23.5 months, and 52 patients died. Univariable analysis suggested that CEA≥4.63 ng/mL, smoking, RPILD, lactate dehydrogenase (LDH) ≥321 IU/L, albumin<30 g/L and dyspnea were risk factors associated with death in patients with anti MDA5 dermatomyositis combined with ILD. Multivariable Cox regression analysis showed that CEA≥4.63 ng/mL [hazard ratio (HR) =3.01, 95% confidence interval (CI) 1.23 - 7.32, P=0.015], RPILD (HR=3.87, 95%CI 2.09 - 7.19, P<0.001), smoking (HR=2.37, 95%CI 1.25 - 4.47, P=0.008), LDH≥321 IU/L (HR=2.47, 95%CI 1.23 - 4.96, P=0.011), albumin<30 g/L (HR=2.57, 95%CI 1.38 - 4.78, P=0.003) were independent predictors for mortality. ConclusionsSerum CEA level can be used as a clinical prognostic predictor in patients with anti-MDA5 positive dermatomyositis and ILD. RPILD, smoking, LDH≥321 IU/L, and albumin<30 g/L are independent predictors for mortality.
Objective To investigate the levels and clinical significance of cold-inducible RNA-binding protein (CIRBP) and mucin 5AC (MUC5AC) in serum and bronchoalveolar lavage fluid (BALF) of patients with interstitial lung disease (ILD). Methods A total of 63 ILD patients who were hospitalized in the Department of Pulmonary and Critical Care Medicine, The First Hospital of Lanzhou University from January 2022 to February 2024 were collected, including 22 patients with idiopathic pulmonary fibrosis (IPF), 20 patients with connective tissue disease-related interstitial lung disease (CTD-ILD), and 21 patients with other types of ILD. Thirty healthy individuals with matching demographic characteristics during the same time period were selected as the healthy group. Enzyme linked immunosorbent assay was used to detect the levels of CIRBP and MUC5AC in serum of healthy group, the levels of CIRBP and MUC5AC in serum and BLAF of ILD patients. The levels of CIRBP and MUC5AC in serum were compared between the healthy group and the ILD group, and the levels of CIRBP and MUC5AC in serum and BALF were compared among the patients with different types of ILD. The correlations were analyzed between the levels of CIRBP and MUC5AC and the lung function, blood gas analysis, and 6-minute walk distance (6MWD) in the patients with different types of ILD. The receiver operating characteristic curve was drawn according to the levels of CIRBP and MUC5AC in serum. The optimal cut-off value, sensitivity and specificity of serum CIRBP and MUC5AC for diagnosis of ILD were determined. Results The serum CIRBP level in the three groups of ILD patients was higher than that of the healthy group (P<0.01), but there was no significant difference in the serum CIRBP level among the three groups of ILD patients (P>0.05). There was no significant difference in serum MUC5AC levels between the three groups of ILD patients and the healthy group, as well as among the three groups of ILD patients (P>0.05). There was no significant difference in the levels of CIRBP and MUC5AC in BALF among the three groups of ILD patients (P>0.05). CIRBP in BALF of the IPF patients was negatively correlated with 6MWD, while CIRBP in serum of the CTD-ILD patients was negatively correlated with partial pressure of oxygen and arterial oxygen saturation. CIRBP in serum of other ILD patients was negatively correlated with total lung capacity as a percentage of predicted value (TLC%pred), forced vital capacity as a percentage of predicted value (FVC%pred), diffusion capacity of carbon monoxide of lung as a percentage of predicted value (DLCO%pred), and 6MWD, while CIRBP in BALF was negatively correlated with DLCO%pred. The MUC5AC in BALF of IPF patients was negatively correlated with DLCO%pred, while the MUC5AC in BALF of CTD-ILD patients was negatively correlated with TLC%pred, DLCO%pred, and 6MWD. The serum MUC5AC in other ILD patients was negatively correlated with FVC%pred, DLCO% pred, and 6MWD. The sensitivity was 77.6%, and the specificity was 96.7% for diagnosing ILD with serum CIRBP. Sensitivity was 55.1%, and specificity was 76.7% for diagnosing ILD with serum MUC5AC. The combination of the two indicators resulted in a sensitivity of 87.8% and a specificity of 86.7% for the diagnosis of ILD. Conclusion The high expression levels of CIRBP and MUC5AC have certain diagnostic efficacy and disease assessment effects on ILD, and may serve as potential biomarkers for ILD.
ObjectiveTo explore the expression of periostin in bronchoalveolar lavage fluid (BALF) of patients with dermatomyositis-related interstitial lung disease (DM-ILD) and rheumatoid arthritis-related interstitial lung disease (RA-ILD).MethodsA total of 44 patients with DM-ILD and 28 patients with RA-ILD were underwent bronchoalveolar lavage. Cells in BALF were collected and analyzed by absolute different cell counts. The level of periostin and Krebs von den Lungen-6 (KL-6) were tested by enzyme linked immunosorbent assay. Results of high resolution CT of patients were scored. Thirty patients without interstitial lung disease (ILD) served as a control group.ResultsLevels of periostin and KL-6 were significantly increased in BALF of patients with DM-ILD and RA-ILD compared with control group (all P<0.05). Levels of periostin were positively correlated with lymphocyte counts and levels of KL-6 in BALF (allP<0.05). Furthermore, levels of periostin were significantly correlated with high resolution CT scores (P<0.05).ConclusionsLevels of periostin are increased in patients with DM-ILD and RA-ILD. Periostin might be served as an indicator of CTD-ILD.
Objective To evaluate the efficacy and safety of glucocorticoids (GC) monotherapy and GC combined with tacrolimus (TAC) therapy in patients with anti-synthetase syndrome-associated interstitial lung disease (ASS-ILD). Methods Through retrospective analysis and propensity score matching (PSM) analysis, the 2-year progression-free survival (PFS) and related side effects of ASS-ILD patients in TAC+GC group and GC monotherapy group were compared. Predictors associated with PFS were analyzed with COX. Results The 2-year PFS rate of TAC+GC group was better than that of GC group [P=0.0163; hazard ratio (HR) 0.347]; Univariate and multivariate analysis of the COX regression model for 2-year PFS in the two groups suggested that creatine kinase level (P=0.0019, HR 1.002) and initial treatment selection [(TAC+GC) vs. GC, P=0.0197, HR 0.207] were independent predictors of PFS; PSM analysis showed that the 2-year PFS rate of TAC+GC group (54.5%) was higher than that of GC group (18.2%) (P=0.0157, HR 0.275). In terms of adverse effect, there was no significant increase in GC+TAC group compared with GC group. Conclusion Compared with GC monotherapy, initial TAC+GC treatment significantly prolonged PFS in ASS-ILD patients and did not increase the incidence of drug-related complications.
ObjectiveTo improve clinicians' knowledge of hypersensitivity pneumonitis (HP). MethodsWe retrospectively analyzed the clinical data of 24 HP patients who were diagnosed in the Affiliated Drum Tower Hospital of Nanjing University Medical School during February 2005 to February 2013. The clinical,radiological and pathological features of those patients were summarized. ResultsAmong those 24 patients,15 were male and 9 were female,with mean age of (48±13) years. All patients had a history of environmental exposure. Two patients showed acute clinical manifestations,and there were 17 subacute and 5 chronic cases. The main clinical manifestations were dyspnea,cough,sputum,fever and weight loss with hypoxemia via blood gas analysis. Restrictive ventilatory impairment was the most frequent functional pattern,and the carbon monoxide diffusing capacity was decreased. Pulmonary function test showed restrictive ventilatory defect and gas interchange disturbance. The features of chest HRCT included diffuse ground-glass attenuation and/or patchy consolidation,centrilobular micronodules,mosaic sign,reticular and/or honeycombing lesions. Bronchoalveolar lavage fluid (BALF) demonstrated an increase of total cell counts with predominant lymphocytosis. The transbronchoscopic lung biopsy (TBLB) pathological examination revealed lymphocytic alveolitis,noncaseating granuloma,and interstial pneumonia. All patients were treated by corticosteroid and avoided antigen exposure and showed significant clinical and radiological improvement. ConclusionThe diagnosis of HP is difficult. In most cases (acute and subacute HP),a diagnosis can be made by combination history of exposure,chest HRCT manifestations,cell classification of BALF and pathological examination of TBLB. For atypical cases (chronic HP),a surgery lung biopsy is needed for multi-disciplinary diagnosis including pathologist,radiologist and pulmonologists.
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 lung involvement in Chinese patients with systemic sclerosis ( SSc) and its functional impact. Methods 68 patients with SSc were enrolled in Scleroderma study of PUMCH ( Peking Union Medical College Hospital) . All the patients underwent high resolution computed tomography ( HRCT) , pulmonary function testing, 6-minute walk testing, and echocardiography. And 36 patients filled in the Saint George’s Respiratory Questionnaire ( SGRQ) for assessment of healthrelated quality of life. Results HRCT revealed interstitial lung disease ( ILD) in 52 ( 76. 5% ) patients, 20 out of them without respiratory symptoms. Reticular opacification, ground-glass opacity ( GGO) , traction bronchiectasis, and honeycomb were presented respectively in 80.8% , 73.1% , 59.6% , and 30.8% of patients with SSc-ILD. Fibrosis was the predominant HRCT finding, and pure GGO ( in the absence of reticulation or architectural distortion) was only present in 8 ( 15. 7% ) patients. 57 (83.8% ) patients presented with diffusion defect, with most of them having moderate to severe impairent. Reduced FVC or TLC presented in 20 ( 29.4% ) and 28 ( 41.2% ) of patients respectively. The significant correlation was observed between the DLCO and the extent of ILD on HRCT ( rs = -0.476, P =0.000) . DLCO showed significant correlations with all the four components of the SGRQ ( Plt;0.05) . Significant correlations were also observed among the SGRQ scores and SpO2 ( maximum desaturation) or Borg index. Stepwise multiple regression analysis confirmed that the DLCO, SpO2 , and Borg index contributed to the SGRQ. Conclusions Lung involvement in Chinese SSc patients is common and insidious. The HRCT features of SSc-ILD are predominant fibrosis plus GGO, indicating little reversibility. Thus HRCT should be performed routinely in newly diagnosed SSc patients for early screening of lung involvement. The lung function defect of SSc is characterized by reduced diffusing capacity, and DLCO show better correlations with HRCT abnormities or SGRQ than FVC or TLC. Thus DLCO is of great value for early detection or severity assessment of SSc-ILD. SGRQ can be used to examine the health related quality of life of SSc patients and may reflect severity of lung involvement.