Lung disease in patients with common variable immunodeficiency
被引:9
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作者:
Lopez, A. L.
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机构:
Hosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, ArgentinaHosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, Argentina
Lopez, A. L.
[1
]
Paolini, M. V.
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机构:
Hosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, ArgentinaHosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, Argentina
Paolini, M. V.
[1
]
Fernandez Romero, D. S.
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Hosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, ArgentinaHosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, Argentina
Fernandez Romero, D. S.
[1
]
机构:
[1] Hosp Dr Carlos G Durand, Unidad Inmunol & Histocompatibilidad, Buenos Aires, DF, Argentina
Background: Common Variable Immunodeficiency (CVID) is characterized by an impaired antibody production and a higher susceptibility to encapsulated bacterial infections. Lung disease is considered to be the most important cause of morbidity and mortality. Methods: We analyzed clinical, radiological and functional characteristics in 80 patients with CVID assisted in the Unidad Inmunologia e Histocompatibilidad at Durand Hospital from 1982 to 2018. Results: Of the 80 patients, 55 showed pathologic lung Computed Tomography (CT). Twenty of them (36.4%) showed bronchiectasis; 26 (47.3%) interstitial involvement associated with nodules and adenopathies called GLILD (granulomatous-lymphocytic interstitial lung disease); and nine patients (16.3%) showed other lesions. Nine percent of patients with lung disease showed CT progression; none of them had spirometry worsening. GLILD patients had normal and restrictive patterns in lung function tests, in equal proportions. Two patients - one with GLILD and the other one with bronchiectasis - had an increase in spirometric pattern severity without CT progression. Lung biopsy was performed in 19% of GLILD patients, all of whom had histopathologic diagnosis of Lymphoid Interstitial Pneumonia (LIP). Conclusions: GLILD is the major cause of lung disease in CVID. Computed tomography is useful for diagnosis but not necessary in follow-up, in which functional tests should have better correlation with clinical evolution, reducing radiation exposure. Biopsy should be indicated when the clinical diagnosis is unclear. Treatment should be considered whenever there is clear evidence of disease progression. (C) 2020 SEICAP. Published by Elsevier Espana, S.L.U. All rights reserved.
机构:
UH Univ Hosp Rainbow Babies & Children, Dept Pediat, Div Allergy Immunol & Rheumatol, Cleveland, OH USA
Icahn Sch Med Mt Sinai, Dept Med, Div Clin Immunol, New York, NY 10029 USAUH Univ Hosp Rainbow Babies & Children, Dept Pediat, Div Allergy Immunol & Rheumatol, Cleveland, OH USA
Lopes, Joao Pedro
Ho, Hsi-en
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机构:
Icahn Sch Med Mt Sinai, Dept Med, Div Clin Immunol, New York, NY 10029 USAUH Univ Hosp Rainbow Babies & Children, Dept Pediat, Div Allergy Immunol & Rheumatol, Cleveland, OH USA
Ho, Hsi-en
Cunningham-Rundles, Charlotte
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Icahn Sch Med Mt Sinai, Dept Med, Div Clin Immunol, New York, NY 10029 USAUH Univ Hosp Rainbow Babies & Children, Dept Pediat, Div Allergy Immunol & Rheumatol, Cleveland, OH USA
机构:
Univ Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, FranceUniv Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, France
Hadjadj, J.
Malphettes, M.
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机构:
Hop St Louis, AP HP, Dept Immunohematol, F-75010 Paris, FranceUniv Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, France
Malphettes, M.
Fieschi, C.
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机构:
Hop St Louis, AP HP, Dept Immunohematol, F-75010 Paris, FranceUniv Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, France
Fieschi, C.
Oksenhendler, E.
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机构:
Hop St Louis, AP HP, Dept Immunohematol, F-75010 Paris, FranceUniv Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, France
Oksenhendler, E.
Tazi, A.
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机构:
Univ Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, FranceUniv Paris Diderot, Sorbonne Paris Cite, Biostat & Clin Epidemiol Res Team, Serv Pneumol,AP HP,Hop St Louis,CREBS U1153, F-75010 Paris, France