Hypersensitivity pneumonitis: lessons for diagnosis and treatment of a rare entity in children

Orphanet Journal of Rare Diseases

Frank Brasch²  Judith Glöckner-Pagel¹  Veronika Teusch¹  Dominik Hartl³  Melanie Haug¹  Matthias Griese¹ 

[1] Dr. von Haunersches Kinderspital, University of Munich, Member of the German Center for Lung Research, Lindwurmstr. 4a, D-80337 Munich, Germany;Institute for Pathology, Bielefeld, Germany;Department of Pediatrics, University of Tuebingen, Tuebingen, Germany

关键词: Steroid treatment;    Precipitins;    Exogenous allergic alveolitis = extrinsic allergic alveolitis;    Diffuse parenchymal lung diseases;    Children;    Bronchoalveolar lavage;    Biopsy;   

Others  :  863613
DOI  :  10.1186/1750-1172-8-121

 received in 2013-05-20, accepted in 2013-07-03,  发布年份 2013

【 摘 要 】

Hypersensitivity pneumonitis (HP) also called exogenous allergic alveolitis = extrinsic allergic alveolitis in children is an uncommon condition and may not be recognized and treated appropriately.

To assess current means of diagnosis and therapy and compare this to recommendations, we used the Surveillance Unit for Rare Paediatric Disorders (ESPED) to identify incident cases of HP in Germany during 2005/6. In addition, cases of HP reported for reference from all over Germany to our center in the consecutive year were included.

Twenty-three children with confirmed pediatric HP were identified. All (age 9.4 y (4.4-15.1) presented with dyspnoea at rest or with exercise, mean FVC was 39% of predicted, seven of the 23 children already had a chronic disease state at presentation. IgG against bird was elevated in 20, and against fungi in 15. Bronchoalveolar lavage was done in 18 subjects (41% lymphocytes, CD4/CD8 1.99), and lung biopsy in 6. Except 2, all children were treated with prolonged courses of systemic steroids. Outcome was not favourable in all cases.

Late diagnosis in up to a quarter of the children with HP and inappropriate steroid treatment must be overcome to improve management of HP. Inclusion of children with HP into international, web-based registry studies will help to study and follow up such rare lung diseases.

【 授权许可】

   
2013 Griese et al.; licensee BioMed Central Ltd.

【 图 表 】

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