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
BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient wi...BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.展开更多
In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, ...In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, four of thirty-one members of the fourth generation, and two of twenty members of the fifth generation;the clinical features of the two members of the first generation are unavailable. Of a total of 48 members, 26 had eosinophilia, with counts ranging from 1761 to 6045 cells/mm<sup>3</sup>, with apparent autosomal dominant propagation;this confirms the experience of the authors of previous studies on this condition. Genetic analysis on eight members, which we reported in an earlier paper [1], revealed a pericentric inversion of chromosome 10 in two members. The entity of Familial Eosinophilia has been generally considered benign. However, the clinical presentation of this kindred was unusual in that valvular and endocardial damage, was frequent findings, without apparent involvement of the other organs and tissues. Mitral valve damage leading to both stenosis and regurgitation and requiring mitral valve replacement was noted in the index patient. This unique presentation may suggest that in patients with mitral valve disease, if blood eosinophilia is noted, it could point to a non-rheumatic etiology, thus a possible opportunity for treatment to prevent further damage to the heart. This recommendation may be even more timely, as many effective treatments are now available to treat even high-grade hypereosinophilia.展开更多
BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thr...BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thrombosis can threaten the lives of patients with sustained eosinophilia.Cerebral venous sinus thrombosis(CVST)in the setting of eosinophil-related diseases has seldom been reported.Here,we review the literature on HE with CVST to increase knowledge and encourage early diagnosis.CASE SUMMARY A previously healthy 41-year-old man was admitted to hospital with diarrhea and abdominal pain.He was treated with antibiotics for suspected acute colitis.Three days later,he experienced headache and vomiting.Brain computed tomography(CT)revealed thrombosis of the left jugular vein to the left transverse sinus vein.Platelet(PLT)count decreased to 60×1012/L,and absolute eosinophil count(AEC)increased to 2.41×109/L.He was treated with low-molecular-weight heparin.PLT count progressively decreased to 14×109/L,and we terminated anticoagulation and performed PLT transfusion.Six days after admission,he complained of a worsening headache.Brain CT revealed right temporal lobe and left centrum semiovale intracerebral hemorrhage,and AEC increased to 7.65×109/L.We used prednisolone for HE.The level of consciousness decreased,so emergency hematoma removal and decompressive craniectomy for right cerebral hemorrhage were performed.The patient was alert 2 d after surgery.He was treated with anticoagulation again 2 wk after surgery.Corticosteroids were gradually tapered without any symptomatic recurrence or abnormal laboratory findings.CONCLUSION HE can induce CVST,and we need to focus on eosinophil counts in patients with CVST.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BACKGROUND Hypereosinophilia(HE)is a heterogeneous disease of unknown etiology in which tissue and organ injury is inflicted by excess numbers of circulating or infiltrating eosinophils.Herein,we describe a patient with rare organ damage due to HE and review the pertinent literature.CASE SUMMARY A 43 year-old Chinese man with a 13-year history of eosinophilia and shortness of breath for 7 d presented to our hospital.During the course of his illness,the patient variably presented with gastrointestinal symptoms,eczema,vitiligo,mastitis,joint symptoms,nephrotic syndrome,and interstitial pneumonia.The chronic mastitis proved burdensome,necessitating bilateral mastectomy.HE was diagnosed by repeat bone marrow biopsy,and a kidney biopsy showed focal segmental glomerulosclerosis.Intermittent steroidal therapy is typically initiated to relieve such symptoms,although relapse and organ involvement often ensue once treatment is withdrawn.We administered methylprednisolone sodium succinate(40 mg/d)intravenously for 3 d,followed by oral tablets at the same dose.Subsequent computed tomography(CT)of the chest CT showed relative improvement of the interstitial pneumonia.The patient is currently on a continuous regimen of oral steroid,and his condition is stable.CONCLUSION HE is heterogeneous condition.This is the first reported case of bilateral mastectomy in a male patient with longstanding HE.
文摘In this paper we present a five-generation kindred with familial eosinophilia, associated with valvular heart disease in one of seven members of the second generation, four of sixteen members of the third generation, four of thirty-one members of the fourth generation, and two of twenty members of the fifth generation;the clinical features of the two members of the first generation are unavailable. Of a total of 48 members, 26 had eosinophilia, with counts ranging from 1761 to 6045 cells/mm<sup>3</sup>, with apparent autosomal dominant propagation;this confirms the experience of the authors of previous studies on this condition. Genetic analysis on eight members, which we reported in an earlier paper [1], revealed a pericentric inversion of chromosome 10 in two members. The entity of Familial Eosinophilia has been generally considered benign. However, the clinical presentation of this kindred was unusual in that valvular and endocardial damage, was frequent findings, without apparent involvement of the other organs and tissues. Mitral valve damage leading to both stenosis and regurgitation and requiring mitral valve replacement was noted in the index patient. This unique presentation may suggest that in patients with mitral valve disease, if blood eosinophilia is noted, it could point to a non-rheumatic etiology, thus a possible opportunity for treatment to prevent further damage to the heart. This recommendation may be even more timely, as many effective treatments are now available to treat even high-grade hypereosinophilia.
基金Zhejiang Research Center of Stroke Diagnosis and Treatment Technology,No.JBZX-202002Zhejiang Province Medical Science and Technology Project,No.2020RC061 and No.2018KY872.
文摘BACKGROUND Hypereosinophilia(HE)is defined as a peripheral blood eosinophil count of>1.5×109/L and may be associated with tissue damage.The clinical presentations of HE vary;however,myocardial fibrosis and thrombosis can threaten the lives of patients with sustained eosinophilia.Cerebral venous sinus thrombosis(CVST)in the setting of eosinophil-related diseases has seldom been reported.Here,we review the literature on HE with CVST to increase knowledge and encourage early diagnosis.CASE SUMMARY A previously healthy 41-year-old man was admitted to hospital with diarrhea and abdominal pain.He was treated with antibiotics for suspected acute colitis.Three days later,he experienced headache and vomiting.Brain computed tomography(CT)revealed thrombosis of the left jugular vein to the left transverse sinus vein.Platelet(PLT)count decreased to 60×1012/L,and absolute eosinophil count(AEC)increased to 2.41×109/L.He was treated with low-molecular-weight heparin.PLT count progressively decreased to 14×109/L,and we terminated anticoagulation and performed PLT transfusion.Six days after admission,he complained of a worsening headache.Brain CT revealed right temporal lobe and left centrum semiovale intracerebral hemorrhage,and AEC increased to 7.65×109/L.We used prednisolone for HE.The level of consciousness decreased,so emergency hematoma removal and decompressive craniectomy for right cerebral hemorrhage were performed.The patient was alert 2 d after surgery.He was treated with anticoagulation again 2 wk after surgery.Corticosteroids were gradually tapered without any symptomatic recurrence or abnormal laboratory findings.CONCLUSION HE can induce CVST,and we need to focus on eosinophil counts in patients with CVST.
