Background: The available data on cryptogenic chronic hypersensitivity pneumonitis (ccHP) indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the rol...Background: The available data on cryptogenic chronic hypersensitivity pneumonitis (ccHP) indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe and progressive disease. Patients and Methods: A total of 9 patients were included in the study. They had criteria for ccHP viz. 1) clinical features of cryptogenic progressive restrictive lung disease, 2) high-resolution computed tomographic pulmonary abnormalities, and 3) bronchoalveolar lavage lymphocytosis (>30%). The regimen consisted of an initial induction phase of 3-month Solumedrol 1 g IV daily for 3 days followed by 1 month of Prednisone (P) 60 mg/day to tapered down to discontinuation by 3rd month. They also had received Mycophenolate mofetil (MMF) 1 g twice daily for 3 months. This stage was followed by a maintenance phase of yearly Rituximab infusions (1 g followed by 1 g 2 weeks later). Results: compared to their previous 6 months deterioration;all patients showed significant improvement in their forced vital volume, diffusion capacity for carbon monoxide, 6-minutes-walk after the induction phase (at 3 months) which improved further at 15 months with Rituximab therapy. Conclusion: After 3-month induction therapy with P and MMF;yearly R treatment is a safe, practical and effective long-term therapy for ccHP.展开更多
BACKGROUND Hypersensitivity pneumonitis(HP)is an immune-mediated syndrome caused by allergen inhalation.High-resolution computed tomography(HRCT)of HP may show diffuse ground-glass opacity,centrilobular ground-glass n...BACKGROUND Hypersensitivity pneumonitis(HP)is an immune-mediated syndrome caused by allergen inhalation.High-resolution computed tomography(HRCT)of HP may show diffuse ground-glass opacity,centrilobular ground-glass nodules,areas of air-trapping,thin-walled cysts,or fibrotic changes.CASE SUMMARY A 47-year-old male patient went to the hospital complaining of cough and gradual aggravation of shortness of breath.HRCT of the lung showed that multiple nodules and ground-glass high-density shadows were present in both lungs.In addition,circular high-density shadows of various sizes were widely distributed in both lungs with relatively normal lung markings inside them.But other tests did not have a positive finding that can clarify the cause.Therefore,the patient underwent a lung biopsy.The pathological results showed that the lesions tended to be HP.After 4 mo of follow-up,the lesions in the patient's lungs were absorbed spontaneously,and the symptoms of cough and shortness of breath have disappeared.The review results suggested that the patient's disease was self-healing,which was consistent with the characteristics of HP.CONCLUSION For some patients with HP,abnormal HRCT findings,such as the lesions in the lungs,can be absorbed on their own,which is an important clue in the diagnosis of the disease.Early diagnosis by lung biopsy is necessary when antigen exposure is unknown.展开更多
BACKGROUND Fibrotic hypersensitivity pneumonitis(FHP)is an allergic and diffuse pneumonia caused by repeated inhalation of antigenic substances,and sometimes developed in people working in specific environments.While ...BACKGROUND Fibrotic hypersensitivity pneumonitis(FHP)is an allergic and diffuse pneumonia caused by repeated inhalation of antigenic substances,and sometimes developed in people working in specific environments.While novel antigens and exposures continued to be described,physicians should maintain a high suspicion of potential exposures.A detailed assessment of the patient's occupational exposures as well as living environment is necessary and complete allergen avoidance is the first and most important step in the management of FHP once the allergens are determined.CASE SUMMARY A 35-year-old female was admitted to the hospital with a cough and breathing difficulties for more than one year.She was a nonsmoker and a manufacturer of halogen dishes,which are characteristic Chinese foods,for 15 years without any protection.High resolution computed tomography of the chest demonstrated an interstitial pneumonia pattern.Pulmonary function examination showed restricted ventilation dysfunction and a significant reduction in dispersion ability.Cell differentiation in bronchoalveolar lavage fluid demonstrated lymphocytosis(70.4%)with an increased lymphocyte CD4/CD8 ratio(0.94).Transbronchial lung biopsy combined with lung puncture pathology showed diffuse uniform alveolar interval thickening,chronic inflammatory cell infiltration,a proliferation of tissue in the bronchial wall fiber and alveolar epithelial follicle degeneration,resulting in fibrosis.CONCLUSION Exposure to spices used for the production of halogen dishes may cause FHP.展开更多
Background:The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis(HP)guidelines.The present study aimed to summarize differences in clinical characteristic...Background:The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis(HP)guidelines.The present study aimed to summarize differences in clinical characteristics and prognosis of non-fibrotic hypersensitivity pneumonitis(NFHP)and fibrotic hypersensitivity pneumonitis(FHP)and explore factors associated with the presence of fibrosis.Methods:In this prospective cohort study,patients diagnosed with HP through a multidisciplinary discussion were enrolled.Collected data included demographic and clinical characteristics,laboratory findings,and radiologic and histopathological features.Logistic regression analyses were performed to explore factors related to the presence of fibrosis.Results:A total of 202 patients with HP were enrolled,including 87(43.1%)NFHP patients and 115(56.9%)FHP patients.Patients with FHP were older and more frequently presented with dyspnea,crackles,and digital clubbing than patients with NFHP.Serum levels of carcinoembryonic antigen,carbohydrate antigen 125,carbohydrate antigen 153,gastrin-releasing peptide precursor,squamous cell carcinoma antigen,and antigen cytokeratin 21-1,and count of bronchoalveolar lavage(BAL)eosinophils were higher in the FHP group than in the NFHP group.BAL lymphocytosis was present in both groups,but less pronounced in the FHP group.Multivariable regression analyses revealed that older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors for the development of FHP.Twelve patients developed adverse outcomes,with a median survival time of 12.5 months,all of whom had FHP.Conclusions:Older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors associated with the development of FHP.Prognosis of patients with NFHP was better than that of patients with FHP.These results may provide insights into the mechanisms of fibrosis in HP.展开更多
Backgrounds:Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) that develops in response to the inhalation of various antigens. The clinical pathologies are very complex and undete...Backgrounds:Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) that develops in response to the inhalation of various antigens. The clinical pathologies are very complex and undetermined. The clinical features and outcomes of HP have not been fully elucidated. The aim of this study was to analyze the incidence, clinical features, and outcomes of HP patients and construct a simple clinical model for diagnosing chronic HP (CHP). Methods: The cohort study included 101 patients with HP admitted to the Nanjing Drum Tower Hospital from January 2009 to December 2017. The patients were categorized into acute HP (AHP, n = 72) and CHP (n = 29) groups according to the updated international criteria. The clinical, imaging, treatment, and follow-up data were retrospectively reviewed. All patients were followed up until December 31,2017. Statistical analysis was performed, and a clinical scoring system for CHP was constructed by SPSS 20.0 software. Results: The incidence of HP was 2.4% in ILD inpatients in our center. Patients in the CHP group were older =-2.212, P = 0.029), had more smokers (x^2 = 8.428, P = 0.004), and longer duration of symptoms (t =-4.852, P < 0.001) than those in the AHP group. Weight loss, crackles, digital clubbing, and cyanosis were more common in the CHP group than those in the AHP group (x^2 = 5.862, P < 0.001;x^2 = 8.997, P = 0.003;x^2 = 11-939, P = 0.001;and x^2 = 4.025, P = 0.045, respectively). On chest high-resolution computed tomography (HRCT), reticular patterns, traction bronchiectasis, and accompanying honeycombing were more common in CHP cases than those in AHP cases (x^2 = 101.000, P < 0.001;x^2 = 32.048, P < 0.001;a n d /2 = 36.568,P < 0.001, respectively). The clinical scoring system for CHP was established based on the clinical variables (age [A], duration of symptoms [D], smoking history [S], unidentified exposure [U], and chest HRCT [C];ADSUC)(area under the curve 0.935, 95% confidence interval: 0.883-0.987, P < 0.001). Eleven patients (15.3%) in the AHP group developed CHP, and unidentified exposure was an independent risk factor for the progression of disease (P = 0.038). The survival of patients with CHP, smoking history, unidentified antigens and fibrosis on Chest HRCT were significantly worse (P = 0.011, P = 0.001, F = 0.005, and P = 0.011, respectively) by Kaplan-Meier analysis. Cox multivariate regression analysis revealed that unidentified exposure and total lung volume (TLC pred%) were independent prognostic predictors for HP patients (P = 0.017 and P = 0.017, respectively). Conclusions: The clinical features and outcomes of the CHP patients differ from those of the AHP patients. ADSUC is a simple and feasible clinical model for CHP. Unidentified exposure is an independent risk factor for the progression of AHP to CHP. Unidentified exposure and a low baseline TLC pred% are independent predictors for survival in HP patients.展开更多
文摘Background: The available data on cryptogenic chronic hypersensitivity pneumonitis (ccHP) indicate an inherited predisposition to disease with triggering autoimmune phenomena. Hence, we evaluated prospectively the role of a new autoimmune regimen in treatment of its severe and progressive disease. Patients and Methods: A total of 9 patients were included in the study. They had criteria for ccHP viz. 1) clinical features of cryptogenic progressive restrictive lung disease, 2) high-resolution computed tomographic pulmonary abnormalities, and 3) bronchoalveolar lavage lymphocytosis (>30%). The regimen consisted of an initial induction phase of 3-month Solumedrol 1 g IV daily for 3 days followed by 1 month of Prednisone (P) 60 mg/day to tapered down to discontinuation by 3rd month. They also had received Mycophenolate mofetil (MMF) 1 g twice daily for 3 months. This stage was followed by a maintenance phase of yearly Rituximab infusions (1 g followed by 1 g 2 weeks later). Results: compared to their previous 6 months deterioration;all patients showed significant improvement in their forced vital volume, diffusion capacity for carbon monoxide, 6-minutes-walk after the induction phase (at 3 months) which improved further at 15 months with Rituximab therapy. Conclusion: After 3-month induction therapy with P and MMF;yearly R treatment is a safe, practical and effective long-term therapy for ccHP.
基金the Scientific Research Project of Gansu Provincial People's Hospital,No.2019-290。
文摘BACKGROUND Hypersensitivity pneumonitis(HP)is an immune-mediated syndrome caused by allergen inhalation.High-resolution computed tomography(HRCT)of HP may show diffuse ground-glass opacity,centrilobular ground-glass nodules,areas of air-trapping,thin-walled cysts,or fibrotic changes.CASE SUMMARY A 47-year-old male patient went to the hospital complaining of cough and gradual aggravation of shortness of breath.HRCT of the lung showed that multiple nodules and ground-glass high-density shadows were present in both lungs.In addition,circular high-density shadows of various sizes were widely distributed in both lungs with relatively normal lung markings inside them.But other tests did not have a positive finding that can clarify the cause.Therefore,the patient underwent a lung biopsy.The pathological results showed that the lesions tended to be HP.After 4 mo of follow-up,the lesions in the patient's lungs were absorbed spontaneously,and the symptoms of cough and shortness of breath have disappeared.The review results suggested that the patient's disease was self-healing,which was consistent with the characteristics of HP.CONCLUSION For some patients with HP,abnormal HRCT findings,such as the lesions in the lungs,can be absorbed on their own,which is an important clue in the diagnosis of the disease.Early diagnosis by lung biopsy is necessary when antigen exposure is unknown.
文摘BACKGROUND Fibrotic hypersensitivity pneumonitis(FHP)is an allergic and diffuse pneumonia caused by repeated inhalation of antigenic substances,and sometimes developed in people working in specific environments.While novel antigens and exposures continued to be described,physicians should maintain a high suspicion of potential exposures.A detailed assessment of the patient's occupational exposures as well as living environment is necessary and complete allergen avoidance is the first and most important step in the management of FHP once the allergens are determined.CASE SUMMARY A 35-year-old female was admitted to the hospital with a cough and breathing difficulties for more than one year.She was a nonsmoker and a manufacturer of halogen dishes,which are characteristic Chinese foods,for 15 years without any protection.High resolution computed tomography of the chest demonstrated an interstitial pneumonia pattern.Pulmonary function examination showed restricted ventilation dysfunction and a significant reduction in dispersion ability.Cell differentiation in bronchoalveolar lavage fluid demonstrated lymphocytosis(70.4%)with an increased lymphocyte CD4/CD8 ratio(0.94).Transbronchial lung biopsy combined with lung puncture pathology showed diffuse uniform alveolar interval thickening,chronic inflammatory cell infiltration,a proliferation of tissue in the bronchial wall fiber and alveolar epithelial follicle degeneration,resulting in fibrosis.CONCLUSION Exposure to spices used for the production of halogen dishes may cause FHP.
基金National Key Technologies R&D Program of China(Nos.2021YFC2500700 and 2016YFC0901100)
文摘Background:The presence of fibrosis is a criterion for subtype classification in the newly updated hypersensitivity pneumonitis(HP)guidelines.The present study aimed to summarize differences in clinical characteristics and prognosis of non-fibrotic hypersensitivity pneumonitis(NFHP)and fibrotic hypersensitivity pneumonitis(FHP)and explore factors associated with the presence of fibrosis.Methods:In this prospective cohort study,patients diagnosed with HP through a multidisciplinary discussion were enrolled.Collected data included demographic and clinical characteristics,laboratory findings,and radiologic and histopathological features.Logistic regression analyses were performed to explore factors related to the presence of fibrosis.Results:A total of 202 patients with HP were enrolled,including 87(43.1%)NFHP patients and 115(56.9%)FHP patients.Patients with FHP were older and more frequently presented with dyspnea,crackles,and digital clubbing than patients with NFHP.Serum levels of carcinoembryonic antigen,carbohydrate antigen 125,carbohydrate antigen 153,gastrin-releasing peptide precursor,squamous cell carcinoma antigen,and antigen cytokeratin 21-1,and count of bronchoalveolar lavage(BAL)eosinophils were higher in the FHP group than in the NFHP group.BAL lymphocytosis was present in both groups,but less pronounced in the FHP group.Multivariable regression analyses revealed that older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors for the development of FHP.Twelve patients developed adverse outcomes,with a median survival time of 12.5 months,all of whom had FHP.Conclusions:Older age,<20%of lymphocyte in BAL,and≥1.75%of eosinophil in BAL were risk factors associated with the development of FHP.Prognosis of patients with NFHP was better than that of patients with FHP.These results may provide insights into the mechanisms of fibrosis in HP.
基金National Natural Science Foundation of China (No.81200049 andNo.81670059)Nanjing Medical Science and Technique Development Foundation (No.QRX17005).
文摘Backgrounds:Hypersensitivity pneumonitis (HP) is an immune-mediated interstitial lung disease (ILD) that develops in response to the inhalation of various antigens. The clinical pathologies are very complex and undetermined. The clinical features and outcomes of HP have not been fully elucidated. The aim of this study was to analyze the incidence, clinical features, and outcomes of HP patients and construct a simple clinical model for diagnosing chronic HP (CHP). Methods: The cohort study included 101 patients with HP admitted to the Nanjing Drum Tower Hospital from January 2009 to December 2017. The patients were categorized into acute HP (AHP, n = 72) and CHP (n = 29) groups according to the updated international criteria. The clinical, imaging, treatment, and follow-up data were retrospectively reviewed. All patients were followed up until December 31,2017. Statistical analysis was performed, and a clinical scoring system for CHP was constructed by SPSS 20.0 software. Results: The incidence of HP was 2.4% in ILD inpatients in our center. Patients in the CHP group were older =-2.212, P = 0.029), had more smokers (x^2 = 8.428, P = 0.004), and longer duration of symptoms (t =-4.852, P < 0.001) than those in the AHP group. Weight loss, crackles, digital clubbing, and cyanosis were more common in the CHP group than those in the AHP group (x^2 = 5.862, P < 0.001;x^2 = 8.997, P = 0.003;x^2 = 11-939, P = 0.001;and x^2 = 4.025, P = 0.045, respectively). On chest high-resolution computed tomography (HRCT), reticular patterns, traction bronchiectasis, and accompanying honeycombing were more common in CHP cases than those in AHP cases (x^2 = 101.000, P < 0.001;x^2 = 32.048, P < 0.001;a n d /2 = 36.568,P < 0.001, respectively). The clinical scoring system for CHP was established based on the clinical variables (age [A], duration of symptoms [D], smoking history [S], unidentified exposure [U], and chest HRCT [C];ADSUC)(area under the curve 0.935, 95% confidence interval: 0.883-0.987, P < 0.001). Eleven patients (15.3%) in the AHP group developed CHP, and unidentified exposure was an independent risk factor for the progression of disease (P = 0.038). The survival of patients with CHP, smoking history, unidentified antigens and fibrosis on Chest HRCT were significantly worse (P = 0.011, P = 0.001, F = 0.005, and P = 0.011, respectively) by Kaplan-Meier analysis. Cox multivariate regression analysis revealed that unidentified exposure and total lung volume (TLC pred%) were independent prognostic predictors for HP patients (P = 0.017 and P = 0.017, respectively). Conclusions: The clinical features and outcomes of the CHP patients differ from those of the AHP patients. ADSUC is a simple and feasible clinical model for CHP. Unidentified exposure is an independent risk factor for the progression of AHP to CHP. Unidentified exposure and a low baseline TLC pred% are independent predictors for survival in HP patients.
