Objective To analyze the clinical presentations and radiological characteristics of acute exacerbation of idiopathic pulmonary fibrosis ( IPF) . Methods Clinical and radiological data of 2 patients with acute exacerbation of IPF from April 2006 to July 2008 were retrospectively analyzed and literatures were reviewed. Results Both patients were senior male patients over 60 years old. Dyspnea, cough and inspiratory crackles were the major symptoms and signs. Two patients were experiencing an exacerbation of dyspnea for one week and half of month, respectively. PaO2 /FiO2 of both patients was less than225 mm Hg. In both patients, high-resolution computed tomography ( HRCT) scans at the exacerbation showed typical signs of IPF including peripheral predominant, basal predominant reticular abnormality, with honeycombing and traction bronchiectasis and bronchiolectasis, and newly developing alveolar opacity. HRCT scan showed peripheral area of ground-glass attenuation adjacent to subpleural honeycombing in one patient, and diffusely distributed ground-glass opacity in another patient. Two patients had received corticosteroid treatment. For one patient, the symptoms improved, and ground-glass attenuation adjacent to subpleural honeycombing had almostly resolved. The other patient died of respiratory failure. Conclusions Some acute exacerbation in idiopatic pulmonary fibrosis can be idiopathic. The clinical presentations mainly include the worsening of dyspnea within short time. HRCT generally demonstrates new bilateral ground-glass abnormality with or without areas of consolidation, superimposed on typical changes of IPF.
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 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.
ObjectiveTo evaluate the role of rapid on-site evaluation (ROSE) in the diagnosis of lung space-occupying lesions.MethodsFrom June 1, 2017 to December 30, 2017, transbronchial biopsies were performed in patients with lung space-occupying lesions on chest CT, and biopsies were taken for ROSE and subsequent routine pathological examination. ROSE interpretation was performed by a pulmonologist who had been trained in cytopathology for 3 months. The interpretation was correlated with the follow-up routine pathological examination. The contemporary cases with lung space-occupying lesions who underwent transbronchial biopsies without ROSE were retrospectively reviewed. The quality assessment of biopsy specimens recorded in pathological reports were compared between cases with and without ROSE.ResultsA total of 101 patients underwent transbronchial biopsies in parallel with ROSE. The interpretation results of the pulmonologist were compared with the follow-up routine pathology, which showed that the consistency rate of malignant lesions was 84.1% and the consistency rate of benign lesions was 93.8%. Consistency test showed good agreement between the ROSE conducted by the pulmonologist and the routine pathological examination by pathologists (κ=0.66, P<0.01). The quality assessment of biopsy specimens showed that there was no significant difference on rate of satisfied biopsy specimens between cases with and without ROSE (98.0%vs 94.5%, P=0.14).ConclusionsThe use of ROSE combining with bronchoscopy allows good preliminary assessment of lung space-occupying lesions. Pulmonologists trained in short-term formal cytopathology are fully capable of performing ROSE, thereby obviating the need for cytopathologists to participate in on-site evaluation.
Objective To investigate the clinical characteristics of interstitial pneumonia patients with positive anti-signal recognition particle antibody (SRP-IP), and compare those with interstitial pneumonia patients with positive anti-Jo-1 antibody (Jo1-IP). Methods Clinical data of SRP-IP patients admitted to Department of Respiratory and Critical Care Medicine of Drum Tower Hospital affiliated to Nanjing University Medical School from May 2017 to May 2021, including clinical manifestations, laboratory examinations, pulmonary function tests and radiographic types, were retrospectively analyzed. The results were compared with those of Jo1-IP patients admitted during the same period. Results The SRP-IP patients were older than Jo1-IP patients (P=0.044). There were no significant differences in clinical manifestations or pulmonary function tests results between the two groups. The proportion of SRP-IP patients combined with positive anti-EJ antibody (P<0.001) or perinuclear anti-neutrophil cytoplasmic antibody (P=0.028) was significantly higher than that of Jo1-IP patients, while the proportion of SRP-IP patients combined positive anti-Ro-52 antibody was significantly lower than that of Jo1-IP patients (P=0.009). The erythrocyte sedimentation rate (ESR) of SRP-IP patients was faster than that of Jo1-IP patients (P=0.026). The serum IgM level (P=0.039) and peripheral NK cell counts (P=0.013) of SRP-IP patients were significantly lower than those of Jo1-IP patients. The most common chest CT findings in SRP-IP patients were organizing pneumonia and the proportion of usual interstitial pneumonia in SRP-IP patients was higher than that of Jo1-IP patients (P=0.032). The levels of creatine kinase (P=0.010), creatine kinase myocardial brand (P=0.025) and alanine aminotransferase (P=0.045) in interstitial pneumonia patients with high titer (++~+++) SRP antibody were higher than those in interstitial pneumonia patients with low titer (+) SRP antibody. SRP-IP and Jo1-IP patients were mainly treated with glucocorticoids combined with or without immunosuppressants, and there was no significant difference in the choice of treatment between the two groups. The proportion of patients with Jo1-IP evaluated as improved was significantly higher than that of patients with SRP-IP (p=0.005), while the proportion of patients with SRP-IP evaluated as stable was significantly higher than that of patients with Jo1-IP (P=0.035). The mortality of SRP-IP patients within 3 months was significantly higher than that of Jo1-IP patients (P=0.028). Conclusion Compared with Jo1-IP patients, SRP-IP patients are older, have faster ESR, are more likely to be combined with other autoantibodies, have lower serum IgM level and peripheral blood NK cell count, have more UIP imaging manifestations, and have a worse short-term prognosis.
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 investigate the diagnostic value and safety of electromagnetic navigation bronchoscopy combined with radial endobronchial ultrasound in peripheral pulmonary nodules.MethodsThe clinical imaging, surgical and pathological data of 60 patients with 76 peripheral pulmonary nodules who underwent electromagnetic navigation bronchoscopy combined with radial endobronchial ultrasound guided biopsy in the Nanjing Drum Tower Hospital, The Affiliated Hospital of Nanjing University Medical School from June 2020 to June 2021 were retrospectively analyzed. The diagnosis rate and complications were analyzed and summarized. The 76 pulmonary nodules were divided into a small pulmonary nodules group (10 nodules, diameter≤1 cm) and a pulmonary nodules group (1 cm<diameter≤3 cm, 66 nodules) according to diameter. The two groups were compared in terms of operation and diagnosis rate.ResultsPulmonary nodules diameter was 1.8±0.6 cm, operation time 29.8±8.6 min, navigation 2.9±0.9 times, biopsy 9.5±1.9 pieces. In the 76 pulmonary nodules, 55 were confirmed by pathology, with a total diagnosis rate of 72.4%, including 32 of malignant lesions and 23 of benign lesions. In the 76 pulmonary nodules, 59 had grade 0 hemorrhage, 17 had grade 1 hemorrhage, and none had grade 2 or more serious hemorrhage. Eight patients developed pneumothorax after surgery, and the degree of lung compression was less than 30%, which was improved after symptomatic treatment with oxygen inhalation. The operation time in the small pulmonary nodules group was significantly longer than that in the pulmonary nodules group, and there was no significant difference in diagnosis rate or complications between the two groups.ConclusionElectromagnetic navigation bronchoscopy combined with radial endobronchial ultrasound is a safe and effective method for the diagnosis of periphery pulmonary nodules, and it also has a high diagnostic rate for small pulmonary nodules (≤1 cm), which is worthy of clinical promotion and application.