Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Me...Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Methods We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review All patients underwent transbronchial lung biopsy （TBLB）. Six of them underwent surgical lung biopsy as well. Results The patients included 7 men and 9 women, aged from 14 to 61 years （median, 47.5 years）. Extrathoracic organs involved included nervous system （7/16） and skin （5/16）. Respiratory symptoms included cough （12/16）, exertional dyspnea （11/16）, hemoptysis （4/16）, and chest pain （3/16）. CT findings included bilateral ground-glass opacities （12 / 16）, bilateral patchy opacities （12 / 16）, and centrilobular nodules （6/16）. The pathological findings of TBLB demonstrated increased eosinophils （3/16）, vasculitis （3/16）, and interstitial pneumonia （16/16）. The pathological fmdings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period （range, 3 to 51 months; median, 15 months）. Conclusions Asthma may be present in CSS patient when there is bronchial involvement. Grotmd-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.展开更多
Objective To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis （EAA）. Methods Patients with EAA diagnosed at Peking Union Medical C...Objective To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis （EAA）. Methods Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases （acute, subacute, and chronic） according to clinical presentations. Follow-up data regarding treatment response, subsequent radio- logical and pulmonary function studies, and clinical outcomes were collected. Results A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds （6 cases, 28.6%）. The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity （12 cases, 63.2%）. The most common findings on high-resolution computed tomography （HRCT） were ground-glass opacities （13 cases, 68.4%） and centri- lobular nodules （8 cases, 42.1%）. Airway obstruction in pulmonary function test, emphysema, hmg cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid （BALF） showed lymphocytosis. The total＇cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients （P〈0.05）. Nonnecrotizing granulomas were seen in 8 （47.1%） cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with avail- able follow-up data. Five patients recurred. Conclusions The characteristic abnormalities of pulmonary function, findings on HRCT, and pa- thology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.展开更多
Background： lmmunoglobulin G4-related disease （IgG4-RD） is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different ty...Background： lmmunoglobulin G4-related disease （IgG4-RD） is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China. Methods： We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features oflgG4-RD. Patients were grouped into different types according to organ involvement regarding organ number and organ site. The constituent ratio in different types was also analyzed. Results： A total of 200 IgG4-RD patients, with a male：female ratio of 2.08：1, were grouped into different types, Cases having involvement of two or three organs were the most common whereas the fewest number of patients had multi-organ （≥4） involvement. Serum IgG4 and lgE levels, IgG4/IgG ratio, and percentage of eosinophils increased as the number of involved organs increased. In addition, constituent ratio analysis revealed that patients with salivary gland/lacrimal gland swelling, who also constituted the largest number of IgG4-RD patients, had higher serum IgG4 concentrations and IgG4/IgG values, had higher percentage of Eos, and were more likely to have had a history of allergies relative to patients with internal organ involvement. Conclusions： The characteristic feature of IgG4-RD is multiple organ involvement with various clinical manifestations and different types. Although serum IgG4 levels increased with the number of involved organs, serum IgG4 levels were higher for those patients with salivary gland/lacrimal gland swelling compared with those with internal organ involvement. Thus, valuable clues to the differential diagnosis of IgG4-RD could be obtained by examining the clinical patterns of organ involvement,展开更多
文摘Objective To investigate the Churg-Strauss syndrome （CSS） associated lung involvement, concentrating on clinical characteristics, pathological findings of tung involvements, response to treatment, and prognosis. Methods We retrospectively analyzed the characters of the clinical manifestations, thin-section CT and pathological findings of CSS. The study involved 16 patients. Clinical data were obtained by chart review All patients underwent transbronchial lung biopsy （TBLB）. Six of them underwent surgical lung biopsy as well. Results The patients included 7 men and 9 women, aged from 14 to 61 years （median, 47.5 years）. Extrathoracic organs involved included nervous system （7/16） and skin （5/16）. Respiratory symptoms included cough （12/16）, exertional dyspnea （11/16）, hemoptysis （4/16）, and chest pain （3/16）. CT findings included bilateral ground-glass opacities （12 / 16）, bilateral patchy opacities （12 / 16）, and centrilobular nodules （6/16）. The pathological findings of TBLB demonstrated increased eosinophils （3/16）, vasculitis （3/16）, and interstitial pneumonia （16/16）. The pathological fmdings of surgical lung biopsy of 6 cases showed necrotizing vasculitis in 4 cases, capillaries in 5, eosinophilic pneumonia in 3, granulomas in 2, and airway abnormalities in 3. All patients improved in symptoms after therapy during the study period （range, 3 to 51 months; median, 15 months）. Conclusions Asthma may be present in CSS patient when there is bronchial involvement. Grotmd-glass opacities and consolidation seen on high-resolution CT reflect the presence of eosinophilic pneumonia, vasculitis, and pulmonary alveolar hemorrhage. TBLB has significant limitations for the diagnosis of CSS. Early diagnosis and therapy can result in satisfactory prognosis.
文摘Objective To assess the spectrum of causes, clinical features, differences between disease phases, and prognosis of extrinsic allergic alveolitis （EAA）. Methods Patients with EAA diagnosed at Peking Union Medical College Hospital from August 1983 to May 2007 were analyzed retrospectively. Their medical records were examined to gather clinical, laboratorial, radiological, and histopathological data. Patients were divided to three phases （acute, subacute, and chronic） according to clinical presentations. Follow-up data regarding treatment response, subsequent radio- logical and pulmonary function studies, and clinical outcomes were collected. Results A total of 21 cases were enrolled. Among them, 11 were subacute, 10 were chronic. The most common exposure was pet birds （6 cases, 28.6%）. The primary abnormality of pulmonary function was restriction and/or reduction in diffusing capacity （12 cases, 63.2%）. The most common findings on high-resolution computed tomography （HRCT） were ground-glass opacities （13 cases, 68.4%） and centri- lobular nodules （8 cases, 42.1%）. Airway obstruction in pulmonary function test, emphysema, hmg cysts, and fibrosis on HRCT were more frequently seen in chronic than in subacute patients, though the differences were not statistically significant. Bronchoalveolar lavage fluid （BALF） showed lymphocytosis. The total＇cell count and the percentage of neutrophils were significantly higher in subacute than in chronic patients （P〈0.05）. Nonnecrotizing granulomas were seen in 8 （47.1%） cases. Improvement or normalization in symptoms, radiography, and pulmonary function test after treatment were seen in all 18 patients with avail- able follow-up data. Five patients recurred. Conclusions The characteristic abnormalities of pulmonary function, findings on HRCT, and pa- thology are essential for all phases of EAA, and the atypical manifestations such as obstruction and fibrosis can also be present frequently, particularly in chronic cases. Differential cell counts of BALF are related to the phase of the disease. The treatment response and prognosis of EAA are good.
基金grants from the National Natural Science Foundation of China
文摘Background： lmmunoglobulin G4-related disease （IgG4-RD） is a newly recognized systemic disease that can involve multiple organs and various clinical phenotypes. The purpose of this study was to analyze different types of organ involvement in IgG4-RD patients in China. Methods： We conducted a prospective cohort study on IgG4-RD patients to analyze the clinical manifestations and rare features oflgG4-RD. Patients were grouped into different types according to organ involvement regarding organ number and organ site. The constituent ratio in different types was also analyzed. Results： A total of 200 IgG4-RD patients, with a male：female ratio of 2.08：1, were grouped into different types, Cases having involvement of two or three organs were the most common whereas the fewest number of patients had multi-organ （≥4） involvement. Serum IgG4 and lgE levels, IgG4/IgG ratio, and percentage of eosinophils increased as the number of involved organs increased. In addition, constituent ratio analysis revealed that patients with salivary gland/lacrimal gland swelling, who also constituted the largest number of IgG4-RD patients, had higher serum IgG4 concentrations and IgG4/IgG values, had higher percentage of Eos, and were more likely to have had a history of allergies relative to patients with internal organ involvement. Conclusions： The characteristic feature of IgG4-RD is multiple organ involvement with various clinical manifestations and different types. Although serum IgG4 levels increased with the number of involved organs, serum IgG4 levels were higher for those patients with salivary gland/lacrimal gland swelling compared with those with internal organ involvement. Thus, valuable clues to the differential diagnosis of IgG4-RD could be obtained by examining the clinical patterns of organ involvement,
