Abstract
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.
Highlights
Hypersensitivity pneumonitis (HP) - in Europe called extrinsic or exogenous allergic alveolitis (EAA) - is a complex syndrome incited by numerous inhaled agents including agricultural dusts, bio-aerosols, fungal, bacterial- or protozoan microorganisms, and certain reactive chemicals. In children it is a relatively uncommon condition and the two major inciting allergens are bird allergens including down and inhaled particles derived from fungi, like thermophilic actinomycetes, or rarely fusarium [1], aureobasidium [2,3] and epicoccum [4]
We used a survey that we performed to determine the incidence of pediatric diffuse parenchymal lung diseases in Germany, to learn more about the current status of HP in children in Germany [13]
HP is the most frequent chronic interstitial lung disease in children [13], a quarter of the children with HP presented here and incident during a 3 y period in Germany, were diagnosed in chronic disease state. This together with the fact that lung biopsies were done at a relatively high frequency, suggests that some difficulties have to be overcome to diagnose this condition in childhood
Summary
Hypersensitivity pneumonitis (HP) - in Europe called extrinsic or exogenous allergic alveolitis (EAA) - is a complex syndrome incited by numerous inhaled agents including agricultural dusts, bio-aerosols, fungal-, bacterial- or protozoan microorganisms, and certain reactive chemicals In children it is a relatively uncommon condition and the two major inciting allergens are bird (avian) allergens including down and inhaled particles derived from fungi, like thermophilic actinomycetes, or rarely fusarium [1], aureobasidium [2,3] and epicoccum [4]. Many paediatricians and general practitioners are likely not to be very familiar with the clinical presentation and diagnosis of HP, and many pediatric pulmonologists may not use up to date treatment All these issues, including an unknown proportion of cases of interstitial lung disease in children which may represent undiagnosed HP, suggested that current information on the diagnosis and treatment of HP is warranted. The goal of this study was to assess current means of diagnosis and therapy of HP, to compare this to the recommendations and to propose ways to improve future management with the help innovative strategies for rare lung diseases
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