Hypersensitivity pneumonitis in children: clinical features, diagnosis, and treatment

Hypersensitivity pneumonitis (HP), or extrinsic allergic alveolitis, is a form of immune-mediated inflammatory lung disease involving the distal portions of the lungs associated with intense or repeated exposure to a variety of finely dispersed environmental antigens. Although once believed to be a disease of adults because of its frequent association with the occupational setting, HP exists in the pediatric population and often goes unrecognized. Childhood HP is often associated with exposure to antigens in the home environment as well as with certain hobbies. Patients present in any one of the three disease stages: acute, subacute, and chronic, all with unique clinical presentations. Histopathologic findings depend on the disease stage at the time of evaluation. The immuno-pathogenesis is complex, but immune-complex (type III hypersensitivity) and cell-mediated (type IV hypersensitivity) immune responses appear to be the primary immune mechanisms involved in the pathogenesis of HP. Diagnosis can be very challenging. Although no single diagnostic or clinical laboratory test is available to diagnose HP, the most significant diagnostic tool is a detailed environmental exposure history. Avoidance of the inciting antigen is the most important form of treatment. Acute HP is responsive to antigen removal alone. However, a short course of prednisone for 2-3 weeks can be useful in patients with severe attacks. Subacute and chronic HP may require higher doses of corticosteroids for a longer duration (i.e. months); however, the long-term efficacy of using corticosteroids is still not well defined. As with most hypersensitivity diseases, early diagnosis provides the best prognosis.