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
Churg-Strauss syndrome(CSS) is a systemic vascular disorder characterized by severe bronchial asthma hypereosinophilia,and allergic rhinitis.Small intestina ulcers associated with CSS are a relatively rare manifestati...Churg-Strauss syndrome(CSS) is a systemic vascular disorder characterized by severe bronchial asthma hypereosinophilia,and allergic rhinitis.Small intestina ulcers associated with CSS are a relatively rare manifestation that causes gastrointestinal bleeding.Multiple deep ulcers with an irregular shape are characteristic of small intestinal involvement of CSS.Video-capsuleendoscopy(VCE),double-balloon endoscopy(DBE) and Spirus assisted enteroscopy have been developed recently and enabled observation of the small intestine In this case report,we have described a patient with CSS who had multiple deep ulcers in the jejunum detected by oral DBE.Since severe gastrointestinal(GI) involvement has been identified as an independent factor associated with poor outcome,the careful investigation of GI tract must be needed for CSS patients with GI symptoms.We describe the usefulness of DBE for diagnosis of small intestinal ulcers in patient with CSS.展开更多
We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal p...We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone,after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.展开更多
Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, co...Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A 52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypical asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment, unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was established considering that five criteria of the American College of Rheumatology were found. Prednisolone was prescribed at 1 mg/kg/day, which completely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy. These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac assessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a prognosis of CSS.展开更多
Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The ...Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.展开更多
BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,an...BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,and eosinophilia.CASE SUMMARY We report an atypical case of EGPA in a 20-year-old female patient.Unlike previously reported cases of EGPA,this patient’s initial symptom was asthma associated with a respiratory infection.This was followed by Loeffler endocarditis and cardiac insufficiency.She received treatment with methylprednisolone sodium succinate,low molecular weight heparin,recombinant human brain natriuretic peptide,furosemide,cefoperazone sodium/sulbactam sodium,and acyclovir.Despite prophylactic anticoagulation,she developed a large right ventricular thrombus.EGPA diagnosis was confirmed based on ancillary test results and specialty consultations.Subsequent treatment included mycophenolate mofetil.Her overall condition improved significantly after treatment,as evidenced by decreased peripheral blood eosinophils and cardiac markers.She was discharged after 17 d.Her most recent follow-up showed normal peripheral blood eosinophil levels,restored cardiac function,and a reduced cardiac mural thrombus size.CONCLUSION This case illustrates the swift progression of EGPA and underscores the significance of early detection and immediate intervention to ensure a favorable prognosis.展开更多
Eosinophilic granulomatosis with polyangiitis(Eg PA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We rep...Eosinophilic granulomatosis with polyangiitis(Eg PA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPgA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction. The clinical data of 14 additional patients with EgP A or Churg-Strauss syndrome complicated with gastrointestinal perforation, which were reported from 1996 to 2014, were also collected and compared. The formation of multiple perforations and fistulas following high dosage steroid administration can have a good outcome with appropriate management. Meticulous attention to abdominal symptoms and appropriate interventions can result in timely management. Corticosteroid administration remains a very important perioperative procedure for EPgA.展开更多
BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the...BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the arterioles and venules.When the coronary arteries are invaded,it can lead to acute myocardial infarction(AMI),acute heart failure,and other manifestations that often lead to death in the absence of timely treatment.CASE SUMMARY A 69-year-old man was admitted to the emergency department due to chest pain for more than 1 h.He had a past history of bronchial asthma and chronic obstructive pulmonary disease and was diagnosed with AMI and heart failure.Thrombus aspiration of the left circumflex artery and percutaneous transluminal coronary angioplasty were performed immediately.After surgery,the patient was admitted to the intensive care unit.The patient developed eosinophilia,and medical history taking revealed fatigue of both thighs 1 mo prior.Local skin numbness and manifestations of peripheral nerve involvement were found on the lateral side of the right thigh.Skin biopsy of the lower limbs pathologically confirmed EGPA.The patient was treated with methylprednisolone combined with intravenous immunoglobulin and was discharged after 21 d.On follow-up at 7 d after discharge,heart failure recurred.The condition improved after cardiotonic and diuretic treatment,and the patient was discharged.CONCLUSION Asthma,impaired cardiac function,and eosinophilia are indicative of EGPA.Delayed diagnosis often leads to heart involvement and death.展开更多
Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronc...Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases of EGPA and almost three-fourths of family members affected by asthma. We explored genetic basis of EGPA in this family and found that genes were mutated in four affected siblings suggesting genetic involvement in susceptibility to EGPA.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Churg-Strauss syndrome(CSS) is a systemic vascular disorder characterized by severe bronchial asthma hypereosinophilia,and allergic rhinitis.Small intestina ulcers associated with CSS are a relatively rare manifestation that causes gastrointestinal bleeding.Multiple deep ulcers with an irregular shape are characteristic of small intestinal involvement of CSS.Video-capsuleendoscopy(VCE),double-balloon endoscopy(DBE) and Spirus assisted enteroscopy have been developed recently and enabled observation of the small intestine In this case report,we have described a patient with CSS who had multiple deep ulcers in the jejunum detected by oral DBE.Since severe gastrointestinal(GI) involvement has been identified as an independent factor associated with poor outcome,the careful investigation of GI tract must be needed for CSS patients with GI symptoms.We describe the usefulness of DBE for diagnosis of small intestinal ulcers in patient with CSS.
文摘We report on a case of Churg-Strauss syndrome (CSS)with colon erosion, cholecystitis and liver abscesses. A 21-year-old woman with a history of bronchial asthma for 3 years was admitted with a complaint of abdominal pain. Laboratory findings included remarkable leukocytosis and eosinophilia, and a colonoscopy revealed erosion from the rectum to the ileocecal region. In addition, a colonic biopsy specimen showed necrotizing vasculitis and marked eosinophilic infiltration. On the basis of the clinical features and histopathological findings, she was diagnosed with CSS and subsequently treated with oral prednisolone,after which the eosinophilia and abdominal pain disappeared. However, on the 15th d in hospital she developed cholecystitis and liver abscesses. She was therefore treated with antibiotics and as a result went into clinical remission.
文摘Churg-Strauss syndrome (CSS) is a very rare small-vessel vasculitis. Clinical features include asthma, rhinitis and/or sinusitis, and peripheral eosinophilia. Although cardiac findings are observed in 50% of cases, coronary artery disease is rarely reported and even more rarely suggestive. The value of cardiac MRI for these patients is discussed here. A 52-year-old non-smoker male without family antecedents of cardiovascular disease presented with worsening of atypical asthma that developed 10 months earlier. A month before, he had been admitted to the ICU because of respiratory distress and cardiogenic shock with chest pain. The angiogram revealed stenosis of the three main coronary arteries requiring the placement of several stents. Follow-up cardiac assessments showed good results. General impairment, unstable asthma associated with rhinitis, and eosinophilia suggested a systemic disease. The diagnosis of CSS was established considering that five criteria of the American College of Rheumatology were found. Prednisolone was prescribed at 1 mg/kg/day, which completely suppressed all symptoms and eosinophilia. Cardiac MRI was performed two months later and revealed a good control of myocardial lesions characterized by fibrosis beneath the anterior endocardium and the median septum. Immunosuppressive treatment was then administered together with corticosteroid therapy. These results suggest that acute coronary artery disease can reveal CSS in some cases. Here, the patient’s cardiac assessment was normal apart from the acute episode, and cardiac MRI helped detect signs of myocarditis and establish a prognosis of CSS.
文摘Vasculitis is an inflammation of vessel walls,followed by alteration of the blood flow and damage to the dependent organ.Vasculitis can cause local or diffuse pathologic changes in the gastrointestinal (GI) tract.The variety of GI lesions includes ulcer,submucosal edema,hemorrhage,paralytic ileus,mesenteric ischemia,bowel obstruction,and life-threatening perforation.The endoscopic and radiographic features of GI involvement in vasculitisare reviewed with the emphasis on small-vessel vasculitis by presenting our typicalcases,including Churg-Strauss syndrome,HenochSch nlein purpura,systemic lupus erythematosus,and Beh et's disease.Important endoscopic features are ischemic enterocolitis and ulcer.Characteristic computed tomographic findings include bowel wall thickening with the target sign and engorgement of mesenteric vessels with comb sign.Knowledge of endoscopic and radiographic GI manifestations can help make an early diagnosis and establish treatment strategy.
基金Supported by the 2020 National Natural Science Foundation Cultivation Project of Guizhou Medical University Affiliated Hospital,No.gyfynsfc[2020]-14.
文摘BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA),formerly known as Churg-Strauss syndrome,is a rare form of anti-neutrophil cytoplasmic antibodyassociated vasculitis characterized by asthma,vasculitis,and eosinophilia.CASE SUMMARY We report an atypical case of EGPA in a 20-year-old female patient.Unlike previously reported cases of EGPA,this patient’s initial symptom was asthma associated with a respiratory infection.This was followed by Loeffler endocarditis and cardiac insufficiency.She received treatment with methylprednisolone sodium succinate,low molecular weight heparin,recombinant human brain natriuretic peptide,furosemide,cefoperazone sodium/sulbactam sodium,and acyclovir.Despite prophylactic anticoagulation,she developed a large right ventricular thrombus.EGPA diagnosis was confirmed based on ancillary test results and specialty consultations.Subsequent treatment included mycophenolate mofetil.Her overall condition improved significantly after treatment,as evidenced by decreased peripheral blood eosinophils and cardiac markers.She was discharged after 17 d.Her most recent follow-up showed normal peripheral blood eosinophil levels,restored cardiac function,and a reduced cardiac mural thrombus size.CONCLUSION This case illustrates the swift progression of EGPA and underscores the significance of early detection and immediate intervention to ensure a favorable prognosis.
文摘Eosinophilic granulomatosis with polyangiitis(Eg PA) is a rare systemic small- and medium-sized-vessel vasculitis. The literature contains only a few reports of gastrointestinal perforation with this condition. We report a patient with EPgA treated with high-dose steroid who underwent emergency surgery for intestinal perforations. We performed a simple repair of the 11 perforations. Intestinal fistulas developed 8 d postoperatively; they healed well after 60 d of continuous washing and negative pressure suction. The clinical data of 14 additional patients with EgP A or Churg-Strauss syndrome complicated with gastrointestinal perforation, which were reported from 1996 to 2014, were also collected and compared. The formation of multiple perforations and fistulas following high dosage steroid administration can have a good outcome with appropriate management. Meticulous attention to abdominal symptoms and appropriate interventions can result in timely management. Corticosteroid administration remains a very important perioperative procedure for EPgA.
文摘BACKGROUND Eosinophilic granulomatosis with polyangiitis(EGPA)is a multisystem disease characterized by allergic rhinitis,asthma,and a significantly high eosinophil count in the peripheral blood.It mainly involves the arterioles and venules.When the coronary arteries are invaded,it can lead to acute myocardial infarction(AMI),acute heart failure,and other manifestations that often lead to death in the absence of timely treatment.CASE SUMMARY A 69-year-old man was admitted to the emergency department due to chest pain for more than 1 h.He had a past history of bronchial asthma and chronic obstructive pulmonary disease and was diagnosed with AMI and heart failure.Thrombus aspiration of the left circumflex artery and percutaneous transluminal coronary angioplasty were performed immediately.After surgery,the patient was admitted to the intensive care unit.The patient developed eosinophilia,and medical history taking revealed fatigue of both thighs 1 mo prior.Local skin numbness and manifestations of peripheral nerve involvement were found on the lateral side of the right thigh.Skin biopsy of the lower limbs pathologically confirmed EGPA.The patient was treated with methylprednisolone combined with intravenous immunoglobulin and was discharged after 21 d.On follow-up at 7 d after discharge,heart failure recurred.The condition improved after cardiotonic and diuretic treatment,and the patient was discharged.CONCLUSION Asthma,impaired cardiac function,and eosinophilia are indicative of EGPA.Delayed diagnosis often leads to heart involvement and death.
文摘Eosinophilic granulomatosis with polyangiitis (EGPA) is an uncommon ANCA associated vasculitic disorder characterized by systemic necrotizing vasculitis of small vessels occurring exclusively among patients with bronchial asthma and tissue eosinophilia. Familial EGPA is extremely rare. Only two case reports have been published so far. We present a Saudi family with 3 cases of EGPA and almost three-fourths of family members affected by asthma. We explored genetic basis of EGPA in this family and found that genes were mutated in four affected siblings suggesting genetic involvement in susceptibility to EGPA.
