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 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.
Objective To highlight the characteristics of giant cell interstitial pneumonia ( GIP) . Methods The clinical, radiological, and pathological data of two patients with GIP pathologically proven by open lung and TBLB biopsy were presented respectively, and relevant literatures were reviewed. Results Patients with GIP usually had a history of exposure to metal dust. Clinical presentations included cough and dyspnea on exertion, and pulmonary function testing showed a restrictive abnormality. On chest radiography and high-resolution CT scans, it presented as bilateral areas of ground-glass attenuation, areas of consolidation, diffuse small nodules, extensive reticular opacities, and traction bronchiectasis. The main pathological findings included a desquamative interstitial pneumonia ( DIP) -like reaction with intra alveolar macrophages and numerous large multinucleated histiocytes that ingested inflammatory cells were admixed with macrophages. The finding of GIP was almost pathognomonic for hard metal pneumoconiosis. Conclusions GIP is a very rare chronic interstitial pneumonia, and has no characteristic clinical manifestations. Radiographic findings are similar to other idiopathic interstitial pneumonias. Careful collection of the occupational history can help to minimize misdiagnosis.
Idiopathic inflammatory myopathies are a group of connective tissue diseases characterized by nonsuppurative inflammation of the striated muscle. At present, the diagnostic criteria for polymyositis/dermatomyositis classification proposed by Bohan and Peter in 1976 is mainly used clinically. In clinical observations, it is found that myopathy involves not only skin and muscle but also affects many systems of the body. Interstitial lung disease occupies an important part, and it is an important cause of death of patients with inflammatory myopathy. Patients with idiopathic myositis should be examined as early as possible by high-resolution CT to improve the detection rate of myositis-associated interstitial lung disease and start treatment as soon as possible. At the same time, the patients with myositis have different clinical manifestations due to specific antibodies in the serum; some specific antibodies may indicate poor prognosis and poor treatment response. Timely screening of patients with positive myositis-specific antibodies in patients with the pulmonary interstitial disease can help the patient’s diagnosis and treatment process.
Objective To analyze the clinical symptoms, imaging, laboratory tests, efficacy and other indicators of patients diagnosed as diffuse parenchymal lung disease ( DPLD) , in order to provide a reference for differential diagnosis of secondary DPLD and idiopathic interstitial pneumonia ( IIP) .Methods Sixtytwo patients diagnosed as DPLD were retrospectively analyzed. Results In 62 patients with DPLD, 19 patients ( 30. 6% ) were diagnosed as secondary DPLD, 42 cases ( 67. 7% ) as IIP, 1 case ( 1. 6% ) as Langerhans cell histiocytosis. The smoking rate of the DPLD patient was 33. 8% , which was higher than general population ( 29. 7% ) . 94. 7% of the secondary DPLD patients had cough, which was higher than the IIP patients ( 71. 4%) . The average age of onset of the secondary DPLD and IIP was ( 45. 9 ±16. 8) years and ( 60. 5 ±7. 7) years respectively, without significant difference ( P gt; 0. 05) . Etiological factors of secondary DPLD were dust, pets, drugs, pesticides, decoration material, etc. The secondary DPLD patients had higher response rate to steroid therapy, but had no statistical difference compared with the IIP patients ( 46. 2% vs. 37. 5% , P gt;0. 05) . Conclusions As a group of diseases of known causes, history taking is very important for DPLD diagnosis and differential diagnosis. Clinical symptoms, imaging, and laboratory tests may provide reference for differential diagnosis of secondary DPLD and IIP.
ObjectiveTo evaluate the prevalence of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA),and explore the association of ILD with RA. MethodsPatients who were diagnosed as RA were recruited in the study,and were categorized into a RA-ILD group and a non-ILD group according to high-resolution computed tomography (HRCT).The data of clinical characteristics,serum biomarkers measurement,and pulmonary function test were collected. Logistic regression analysis was performed to evaluate the factors associated with the incidence of ILD in RA. ResultsA total of 59 patients with RA were enrolled. The incidence of ILD in RA was 42.3%. The rheumatoid factor (RF),carbohydrate antigen 15-3 (CA15-3),carbohydrate antigen12-5 (CA125),inspiratory capacity (IC) and diffusion capacity for carbon monoxide of the lung (DLCO) were significantly different between two groups (all P<0.05). Multivariate logistic regression analysis revealed that the increased CA15-3 and decreased DLCO were independent risk factors for ILD in the patients with RA. ConclusionsThe occurrence of ILD with RA seems very high. CA15-3 and DLCO may be important risk factors for the incidence of ILD in patients with RA.
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.