Pediatric hypersensitivity pneumonitis: literature update and proposal of a diagnostic algorithm

Abstract

Hypersensitivity pneumonitis (HP) is a rare disease in childhood with the prevalence of 4 cases per 1 million children and an incidence of 2 cases per year. The average age of diagnosis at pediatric age is approximately 10 years. The pathogenesis of HP is characterized by an immunological reaction caused by recurrent exposure to triggering environmental agents (mostly bird antigens in children). The clinical picture of HP is complex and variable in children, often presenting in subacute forms with cough and exertion dyspnea. A diagnosis of HP should be considered in patients with an identified exposure to a triggering antigen, respiratory symptoms, and radiologic signs of interstitial lung disease. Blood tests and pulmonary function tests (PFT) support the diagnosis. Bronchoscopy (with bronchoalveolar lavage and tissue biopsy) may be needed in unclear cases. Antigen provocation test is rarely required. Of note, the persistence of symptoms despite various treatment regimens may support HP diagnosis. The avoidance of single/multiple triggers is crucial for effective treatment. No evidence- based guidelines for treatment are available; in particular, the role of systemic glucocorticoids in children is unclear. With adequate antigen avoidance, the prognosis in children with HP is generally favorable.

Keywords: Children; Cough; Dyspnea; Extrinsic allergic alveolitis; Hypersensitivity pneumonitis; Interstitial pneumonia; Pediatric.

Fig. 1

Diagnostic algorithm of childhood HP. Comments:^*Respiratory symptoms: the clinical course can be chronic with dry cough and exertion dyspnoea in children. Usually, before diagnosis, children are treated as asthmatic without significant clinical improvement [7]. ^§Environmental history: bird breeding in almost all pediatric cases [41]. ^**in the presence of known exposure history to trigger typical HRCT and response to treatment, BAL is not necessary [29]