嗜酸细胞性肉芽肿性多血管炎13例临床分析
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摘要
目的总结嗜酸细胞性肉芽肿性多血管炎(EGPA)患者的临床表现、影像学、病理,以探讨其临床特点,提高临床诊断水平。方法回顾性分析2011年01月至2016年04月南京鼓楼医院收治的13例EGPA患者的临床资料。结果 13例患者中,男女比6:7,发病年龄17～76岁,平均年龄52. 9±17. 1岁。病变累及多个系统,以哮喘为首发症状的占76. 9%(10/13)。病程中出现哮喘症状的占92. 3%(12/13),鼻窦炎与外周血嗜酸性粒细胞增多者均占84. 6%(11/13)。EGPA的胸部CT可发现磨玻璃样到气腔实变阴影,主要呈双侧外周为主的多发斑片状非节段性实变阴影69. 2%(9/13),结节样阴影38. 4%(5/13),网状阴影23. 1%(3/13),细支气管炎30. 1%(4/13)。抗中性粒细胞抗体阳性率30. 7%;肌电图以腓神经受累为主。4例患者经病理明确,送检组织可见嗜酸性粒细胞浸润,局部区域中小肌性脉管可见嗜酸性粒细胞透壁性浸润; 9例患者为临床诊断。除1例因意识障碍为首发症状患者外,12例患者均给予激素治疗,7例患者合用环磷酰胺,随访1年以上,病情稳定。结论 EGPA临床表现复杂,对同时有哮喘,嗜酸性粒细胞增多,鼻窦炎,肺部影像学为多发斑片状非节段性实变阴影的患者需考虑EGPA的可能,必要时行肺内或肺外活检明确。
Objective To summarize the clinical manifestations,imaging and pathology of eosinophilic granulomatous polyangitis( EGPA),in order to explore its clinical features and improve the level of clinical diagnosis.Methods The clinical data of 13 cases of EGPA patients from January 2011 to April 2016 were retrospectively analyzed. Results Of all 13 cases,the ratio of male to female was 6: 7,the onset age was 17 ～ 76 years,and the average age was 52. 9 ± 17. 1 years old. The lesions involved multiple systems,and 76. 9%( 10/13) cases began with asthma as their initial symptom. Patients with asthma during the course accounted for 92. 3%( 12/13),and sinusitis and peripheral blood eosinophilia both accounted for 84. 6%( 11/13). In the chest CT of EGPA,the shadow of ground glass to air cavity consolidation was found,which mainly presented as multivariate patchy non-segmental consolidation shadow in bilateral peripheral dominance( 69. 2%,9/13),nodular shadow( 38. 4%,5/13),reticular shadow( 23. 1%,3/13),and bronchiolitis( 30. 1%,4/13). The positive rate of ANCA was 30. 7%. EMG mainly indicated peroneal nerve damage. Of all cases,4 were pathologically confirmed,eosinophil infiltration was observed in the submitted tissues,and eosinophil permeable infiltration was observed in small muscle vessels of local area. The others were diagnosed clinically. In addition to 1 case performed consciousness disorder as first symptom,all the 12 patients were treated with hormone therapy,and 7 patients were treated with cyclophosphamide. All of them were followed up for more than 1 year and in stable condition. Conclusion The clinical manifestations of EGPA are complex,and the possibility of EGPA should be considered in patients with asthma,eosinophilia,sinusitis,and multispot pattern non-segmental consolidation shadow of lung imaging. If necessary,lung or extrapulmonary biopsy should be performed.
Objective To summarize the clinical manifestations,imaging and pathology of eosinophilic granulomatous polyangitis( EGPA),in order to explore its clinical features and improve the level of clinical diagnosis.Methods The clinical data of 13 cases of EGPA patients from January 2011 to April 2016 were retrospectively analyzed. Results Of all 13 cases,the ratio of male to female was 6: 7,the onset age was 17 ～ 76 years,and the average age was 52. 9 ± 17. 1 years old. The lesions involved multiple systems,and 76. 9%( 10/13) cases began with asthma as their initial symptom. Patients with asthma during the course accounted for 92. 3%( 12/13),and sinusitis and peripheral blood eosinophilia both accounted for 84. 6%( 11/13). In the chest CT of EGPA,the shadow of ground glass to air cavity consolidation was found,which mainly presented as multivariate patchy non-segmental consolidation shadow in bilateral peripheral dominance( 69. 2%,9/13),nodular shadow( 38. 4%,5/13),reticular shadow( 23. 1%,3/13),and bronchiolitis( 30. 1%,4/13). The positive rate of ANCA was 30. 7%. EMG mainly indicated peroneal nerve damage. Of all cases,4 were pathologically confirmed,eosinophil infiltration was observed in the submitted tissues,and eosinophil permeable infiltration was observed in small muscle vessels of local area. The others were diagnosed clinically. In addition to 1 case performed consciousness disorder as first symptom,all the 12 patients were treated with hormone therapy,and 7 patients were treated with cyclophosphamide. All of them were followed up for more than 1 year and in stable condition. Conclusion The clinical manifestations of EGPA are complex,and the possibility of EGPA should be considered in patients with asthma,eosinophilia,sinusitis,and multispot pattern non-segmental consolidation shadow of lung imaging. If necessary,lung or extrapulmonary biopsy should be performed.
引文
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[4] GUILLEVIN L,COHEN P,GAYRAUD M,et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients[J]. Medicine(Baltimore),1999,78(1):26-37.
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[7] FURUIYE M,KONNO K,SUMI Y,et al. A case of Churg-Strauss syndrome in which positive rheumatoid factor and MPO-ANCA preceded the development of clinical symptoms of vasculitis[J]. Nihon Kokyuki Gakkai Zasshi,2001,39(6):410-414.
[8] MASI A T,HUNDER G G,LIE J T,et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome(allergic granulomatosis and angiitis)[J]. Arthritis Rheum,1990,33(8):1094-1100.
[9] LANHAM J G,ELKON K B,PUSEY C D,et al. Systemic vasculitis with asthma and eosinophilia:a clinical approach to the ChurgStrauss syndrome[J]. Medicine(Baltimore),1984,63(2):65-81.
[10]饶慧,刘跃华,蒋明.变应性肉芽肿性血管炎15例临床分析[J].中华风湿病学杂志,2005,9(9):552-553,577.
[11] GUILLEVIN L,LHOTE F,GHERARDI R. Polyarteritis nodosa,microscopic polyangiitis,and Churg-Strauss syndrome:clinical aspects,neurologic manifestations,and treatment[J]. Neurol Clin,1997,15(4):865-886.
[12] GUILLEVIN L,LHOTE F,GAYRAUD M,et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients[J]. Medicine(Baltimore),1996,75(1):17-28.
[13] KNOELL D L,LUCAS J,ALLEN J N. Churg-Strauss syndrome associated with zafirlukast[J]. Chest,1998,114(1):332-334.
[14] TATSIS E,SCHNABEL A,GROSS W L. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome[J]. Ann Intern Med,1998,129(5):370-374.
[15] BERLIOZ M,TRIOLO V,SIRVENT N,et al. Churg-Strauss syndrome revealed by acute abdominal pain[J]. Pediatr Pulmonol,2001,32(1):92-94.
[2] RAMAKRISHNA G,MIDTHUN D E. Churg-Strauss syndrome[J].Ann Allergy Asthma Immunol,2001,86(6):603-613.
[3]郭述良,罗永艾.变应性肉芽肿性血管炎[J].中国实用内科杂志,2002,22(6):327-329.
[4] GUILLEVIN L,COHEN P,GAYRAUD M,et al. Churg-Strauss syndrome. Clinical study and long-term follow-up of 96 patients[J]. Medicine(Baltimore),1999,78(1):26-37.
[5]刘冬舟,谭艳红,肖学吕,等.变应性肉芽肿性血管炎69例荟萃分析[J].临床和实验医学杂志,2008,7(7):59-60.
[6] HELLEMANS S,DENS J,KNOCKAERT D. Coronary involvement in the Churg-Strauss syndrome[J]. Heart,1997,77(6):576-578.
[7] FURUIYE M,KONNO K,SUMI Y,et al. A case of Churg-Strauss syndrome in which positive rheumatoid factor and MPO-ANCA preceded the development of clinical symptoms of vasculitis[J]. Nihon Kokyuki Gakkai Zasshi,2001,39(6):410-414.
[8] MASI A T,HUNDER G G,LIE J T,et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome(allergic granulomatosis and angiitis)[J]. Arthritis Rheum,1990,33(8):1094-1100.
[9] LANHAM J G,ELKON K B,PUSEY C D,et al. Systemic vasculitis with asthma and eosinophilia:a clinical approach to the ChurgStrauss syndrome[J]. Medicine(Baltimore),1984,63(2):65-81.
[10]饶慧,刘跃华,蒋明.变应性肉芽肿性血管炎15例临床分析[J].中华风湿病学杂志,2005,9(9):552-553,577.
[11] GUILLEVIN L,LHOTE F,GHERARDI R. Polyarteritis nodosa,microscopic polyangiitis,and Churg-Strauss syndrome:clinical aspects,neurologic manifestations,and treatment[J]. Neurol Clin,1997,15(4):865-886.
[12] GUILLEVIN L,LHOTE F,GAYRAUD M,et al. Prognostic factors in polyarteritis nodosa and Churg-Strauss syndrome. A prospective study in 342 patients[J]. Medicine(Baltimore),1996,75(1):17-28.
[13] KNOELL D L,LUCAS J,ALLEN J N. Churg-Strauss syndrome associated with zafirlukast[J]. Chest,1998,114(1):332-334.
[14] TATSIS E,SCHNABEL A,GROSS W L. Interferon-alpha treatment of four patients with the Churg-Strauss syndrome[J]. Ann Intern Med,1998,129(5):370-374.
[15] BERLIOZ M,TRIOLO V,SIRVENT N,et al. Churg-Strauss syndrome revealed by acute abdominal pain[J]. Pediatr Pulmonol,2001,32(1):92-94.