Interstitial Lung Disease in Children Older Than 2 Years

Abstract

The spectrum of childhood interstitial lung diseases (chILD) encompasses a group of heterogeneous, rare disorders in children characterized by diffuse pulmonary infi ltrates and disordered gas exchange. Whereas the disorders that present in early life are unique to children, those that present in older children are also seen in adults. This review will concentrate on chILD presenting in children older than 2 years of age with a focus on the idiopathic interstitial pneumonias, connective tissue diseases, alveolar hemorrhage, and hypersensitivity pneumonitis. A systematic approach to diagnosis that includes a careful history and physical, computed tomography of the chest, bronchoalveolar lavage, and lung biopsy can be very helpful in establishing the correct diagnosis. Treatment approaches are described, including general supportive measures, indications for a trial of systemic corticosteroids, or other immunomodulating therapies, and when lung transplantation reserved for those with end-stage lung disease should be considered.

Interstitial Lung Disease in Children Made Easier…Well, Almost

“…Hypersensitivity pneumonitis is uncommon in childhood but manifests in an identical manner to, and shares the CT features of, the adult form of the disease. Precipitating exposures include avian antigens, mold spores, and airborne compounds from paints, glues, and insecticides (49). A German study of 23 children (aged 4-15 years) with hypersensitivity pneumonitis demonstrated poorly defined centrilobular nodules in 96% and diffuse ground-glass opacification in 73% in the acute phase (50) (Figs 20, 21).…”

Bronchoalveolar lavage fluid cytokine profiles in neuroendocrine cell hyperplasia of infancy and follicular bronchiolitis

“…Recently, patients with NEHI syndrome have been diagnosed using clinical findings, CT scan, infant pulmonary functions and negative findings on bronchoscopy in some centers [19]. However, despite gains in recognizing the classic clinical phenotype of NEHI, some children present with atypical clinical phenotypes that may require lung biopsy.…”

Diffuse Lung Disease

“…Birds, pets, and molds are the most common precipitating agents in children. Other causes include highly reactive lowmolecular-weight compounds in spray paints, epoxy resins, glues, and insecticides, as well as drugs such as methotrexate (Vece and Fan 2010). There is a mean lag time of 11 months from symptom onset to diagnosis, and a meticulous environmental exposure history is a crucial step in the clinical evaluation.…”

“…CXRs are limited in sensitivity, being normal in over one-third of cases at initial presentation (Buchvald et al 2011). The absence of serum-precipitating antibodies does not exclude the diagnosis (Clement et al 2010;Vece and Fan 2010). A presumptive diagnosis without biopsy can be made in children with an exposure history and positive precipitins, lymphocytosis on BAL, and typical findings on CT (Vece and Fan 2011).…”