Histologic patterns and considerations for childhood interstitial and diffuse lung diseases

Histologic pattern	Specific types of DLD that may display this histologic pattern
Pulmonary alveolar proteinosis	Genetic disorders associated with surfactant dysfunction (eg, mutations in the SFTPB, SFTPC, ABCA3, or NKX2-1/TTF1 genes) Autoimmune mechanisms, including GM-CSF autoantibodies Lysinuric protein intolerance Genetic disorders that impact macrophage function or GM-CSF signaling (eg, mutations in the CSF2RA, CSF2RB, MARS1, GATA2, STAT5B, and OAS1 genes)
Cellular interstitial pneumonitis	Pulmonary interstitial glycogenosis
Chronic pneumonitis of infancy	Genetic disorders of surfactant dysfunction
Desquamative interstitial pneumonia	Genetic disorders of surfactant dysfunction Chronic pulmonary hemorrhage syndrome
Diffuse alveolar damage (DAD)/acute interstitial pneumonia (AIP)	The findings of AIP are identical to the organizing or proliferative state of DAD, which is the histologic lesion of acute respiratory distress syndrome
Lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and related disorders	Immunodeficiency disorders Connective tissue disease
Nonspecific interstitial pneumonia (NSIP)	Connective tissue disease Rarely, SFTPC gene mutations
Organizing pneumonitis	Cryptogenic organizing pneumonitis* This finding also may accompany other histologic patterns and may be seen as a complication of HIV infection, chemotherapy, or bone marrow transplantation
Usual interstitial pneumonitis (UIP)^¶	One case of UIP was reported in an adolescent with an ABCA3 gene mutation; otherwise, UIP has not been reported in children

DLD: diffuse lung disease; GM-CSF: granulocyte-macrophage colony-stimulating factor; DAD: diffuse alveolar damage; AIP: acute interstitial pneumonia; LIP: lymphocytic interstitial pneumonia; NSIP: nonspecific interstitial pneumonia; HIV: human immunodeficiency virus; UIP: usual interstitial pneumonitis.

* Cryptogenic organizing pneumonitis was previously called bronchiolitis obliterans with organizing pneumonia.

¶ UIP is associated with idiopathic pulmonary fibrosis in adults, but idiopathic pulmonary fibrosis does not occur in children. In adults, the UIP pattern may also be seen in individuals with connective tissue disease and hypersensitivity pneumonitis.

Courtesy of Lisa R Young, MD.

Graphic 90994 Version 8.0

خرید پکیج

Histologic patterns and considerations for childhood interstitial and diffuse lung diseases