ﺑﺎﺯﮔﺸﺖ ﺑﻪ ﺻﻔﺤﻪ ﻗﺒﻠﯽ
خرید پکیج
تعداد آیتم قابل مشاهده باقیمانده : -12 مورد

Histologic patterns and considerations for childhood interstitial and diffuse lung diseases

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)
  • Rheumatic disease (juvenile idiopathic arthritis)*
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
  • Rheumatic disease (juvenile polymyositis/dermatomyositis; systemic lupus erythematosus)*
Lymphocytic interstitial pneumonia (LIP), follicular bronchiolitis, and related disorders
  • Immunodeficiency disorders
  • Rheumatic disease (Sjögren disease; systemic lupus erythematosus)*
Nonspecific interstitial pneumonia (NSIP)
  • Rheumatic disease (NSIP is a common manifestation)*
  • Rarely, SFTPC gene mutations
Organizing pneumonitis (OP)
  • Cryptogenic organizing pneumonitis
  • This finding also may accompany other histologic patterns and may be seen as a complication of rheumatic diseases*, HIV infection, chemotherapy, or bone marrow transplantation
Usual interstitial pneumonitis (UIP)Δ
  • One case of UIP was reported in an adolescent with an ABCA3 gene mutation; otherwise, UIP has not been reported in children

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

* Rheumatic diseases that can manifest with DLD during childhood include systemic sclerosis (scleroderma), juvenile polymyositis/dermatomyositis, juvenile idiopathic arthritis, mixed connective tissue disease, systemic lupus erythematosus, and Sjögren disease. NSIP is a common pattern for all of these disorders; less common patterns include OP, LIP (in Sjögren disease or systemic lupus erythematosus), DAD (in juvenile polymyositis/dermatomyositis or systemic lupus erythematosus), and PAP (in juvenile idiopathic arthritis).

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

Δ 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 9.0