Objective To evaluate the diagnostic value and safety of surgical lung biopsies ( SLB)in patients with interstitial lung disease ( ILD) . Methods A retrospective analysis was performed on patients undergoing SLB, who were obtained from Chinese literatures through searching PubMed, CBM,Wanfang database, VIP information and CHKD from 2000 to 2010. The data from Affiliated Drum Tower Hospital, Nanjing University Medical College from2000 to 2010 was also reviewed. Results A total of 398cases underwent SLB, including open lung biopsy ( OLB) in 221 cases and video-assisted thoracoscopic lung biopsy ( VATLB) in 177 cases. SLB yielded a specific diagnosis for 386 cases. The total postoperative complication rate was 12. 1% and mortality rate was 2. 0% . The diagnostic yield, post-operative complication rate, and mortality rate between VATLB and OLB had no significant difference. Conclusion SLB is a useful and relatively safe procedure for diagnosis of ILD.
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.
Objective To explore the effects of bone marrow mesenchymal stem cells ( BMMSCs) on pulmonary fibroblasts of patients with nonspecific interstitial pneumonitis ( NSIP) , and investigate the therapeutic mechanism of BMMSCs for interstitial pulmonary fibrosis. Methods Human BMMSCs, human pulmonry fibroblasts ( HPFs) from NSIP patients, and normal HPFs were primary cultured in vitro. Then HPFs fromNSIP patients were co-cultured with BMMSCs or normal HPFs using Transwell co-culture system. After 24 hours, levels of transforming growth factor β1 (TGF-β1) and interferon inducible protein 10 ( IP-10) in culture supernatants were detected by ELISA method. Meanwhile, interleukin-6 ( IL-6) , IL-8, and monocyte chemotactic protein-1 ( MCP-1) in co-culture supernatants were detected by liquid chip. After co-cultured for 48 hours, total protein of HPFs was extracted and the expression level of alpha smooth muscle actin ( α-SMA) secreted by HPFs were detected by Western blot.Results HPFs from NSIP patients secreted higher level of IL-6, IL-8, and MCP-1 than normal HPFs, and secreted high level of α-SMA. In the Transwell co-culture system, human BMMSCs significantly reduced the levels of IL-6, IL-8, and MCP-1 secreted from HPFs of NSIP patients, and reduced the high expression of α-SMA in HPFs of NSIP patients. Conclusion Human BMMSCs can significantly reduce the secretion of IL-6, IL-8, MCP-1, and the expression of α-SMA in HPFs from NSIP patients.
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 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.
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.
ObjectiveTo explore the clinical, radiological and pathological characteristics of acute fibrinous and organizing pneumonia (AFOP). MethodsA case pathologically diagnosed with AFOP in September 2013 in the Second Affiliated Hospital of Nanjing Medical University was reported, and the related literature was reviewed. ResultsA 50-year-old woman with fever, chills, cough with sputum and chest pain was admitted to this hospital. The chest CT showed the nodules and patching infiltrates of the right middle lung. The pathological examination revealed the focally exudation of fibrin, lymphocyte infiltration and the presence of foam cells within the alveolar spaces, which is different from other well-known acute lung injures such as diffuse alveolar damage, cryptogenic organizing pneumonitis, and acute eosinophilic pneumonia. Coticosteroid therapy was induced and the patient showed significantly clinical and radiological improvement. ConclusionAFOP has no specific clinical, laboratory tests and radiology features, and it's diagnosis depends on pathological examination. Treatment with coticosteroids is proved to be effective.
Objective This study aims to analyze the clinical characteristics of idiopathic inflammatory myopathy (IIM) associated interstitial lung disease (ILD) with positive anti melanoma differentiation-associated gene 5 (MDA5) antibody. Methods The clinical data of 91 patients with IIM in Shanxi Bethune Hospital from Jan 2019 to Mar 2022 were collected. Then various comparisons and analyzed retrospectively in the clinical symptoms, laboratory indicators and imaging characteristics between the negative MDA5-IIM and the positive anti-MDA5-IIM. ResultsCompared with the negative anti-MDA5-IIM patients, the significantly higher incidences of Gottron sign (71.0% vs. 38.3%), ulcerative rash (45.2% vs.16.7%), dyspnea (64.5% vs. 36.7%), and ILD (71.0% vs. 43.3%), in the positive anti-MDA5-IIM patients (P<0.05). Compared with the negative anti-MDA5-IIM patients, the more ground-glass opacification (GGO) in the positive anti-MDA5-IIM patients on high resolution computed tomography (81.8% vs.53.8%, P<0.05). Compared with the positive anti-MDA5-IIM patients without ILD, the positive anti-MDA5-IIM patients with ILD had significantly higher incidence of Gottron sign (77.3% vs. 33.3%), the increased levels of lactate dehydrogenase [(525.20±203.09) IU/L vs. (321.73±188.88) IU/L], Krebs von den Lungen-6 [(1399.55±869.64) U/mL vs. (371.56±128.92) U/mL], and D-dimer [(1226.65±902.31) U/L vs. (703.73±160.11) U/L], as well as higher positive rate of anti-Ro-52 antibody (68.2% vs. 22.2%) and the higher case fatality rate (54.5% vs. 0.0%), with all P<0.05. The triple treatment of high-dose glucocorticoid (GC) combined with intravenous cyclophosphamide (IVCY) and another immunosuppressive agent (tacrolimus or tofacitinib) was found to be effective in the MDA5-IIM associated ILD group, with a lower case fatality rate (28.6%) than high-dose GC combined with IVCY group (47.1%). Conclusions The positive anti-MDA5-IIM with ILD of patients have the higher incidence of rash and dyspnea, with GGO as the most common imaging manifestation. When rapidly progressive interstitial lung disease (RPILD) is combined, the case fatality rate higher. Meanwhile, early intensive immunosuppressive therapy may improve the survival rate of RPILD patients with positive anti-MDA5 antibody.
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 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.