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Lymphoid interstitial pneumonia

Disease definition

A rare idiopathic interstitial pneumonia characterized by a diffuse, dense, polyclonal lymphoid cell infiltration of the pulmonary interstitium and air spaces, with high prevalence in patients with immune dysregulation. Presenting symptoms are non-specific and include dyspnea and cough. The clinical course is highly variable, ranging from spontaneous resolution to progressive, fatal respiratory failure.


Classification level: Disorder
  • Synonym(s):
    • Lymphocytic interstitial pneumonia
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: All ages
  • ICD-10: J84.1
  • ICD-11: CB03.5
  • OMIM: 247610
  • UMLS: C0264511
  • MeSH: -
  • GARD: -
  • MedDRA: 10062997
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