[Primary Immunodeficiency: Primary Antibody Disorders in Respiratory Medicine]. / Primäre Immundefekte: Antikörpermangelerkrankungen in der Pneumologie.
Pneumologie
; 73(2): 94-107, 2019 Feb.
Article
em De
| MEDLINE
| ID: mdl-30759496
ABSTRACT
Clinical manifestations of primary immunodeficiency are heterogeneous, and early diagnosis is challenging. Leading symptoms are recurrent upper and lower respiratory tract infections. Response to antibiotic therapy is often reduced. Beside infectious complications autoimmunity, autoinflammation and malignant diseases occur frequently. About 50â% of all PID patients are diagnosed after childhood, and the main group are patients with primary antibody deficiencies. Treatment of choice is the immunoglobulin substitution and the prophylactic or therapeutic use of antibiotics. In patients presenting with immunodysregulation, immunosuppression is additionally indicated. Especially due to recurrent lower airway infection and/or interstitial lung diseases PID patients have a decreased live expectancy. Hence, both early diagnosis and sufficient therapy are mandatory.
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Base de dados:
MEDLINE
Assunto principal:
Imunoglobulinas
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Síndromes de Imunodeficiência
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Infecções
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Inflamação
Idioma:
De
Ano de publicação:
2019
Tipo de documento:
Article