Successful treatment of endogenous lipoid pneumonia due to Niemann-Pick Type B disease with whole-lung lavage

Am J Respir Crit Care Med. 2002 Jan 1;165(1):128-31. doi: 10.1164/ajrccm.165.1.2103113.


In Type B Niemann-Pick disease, progressive pulmonary infiltration is a major cause of morbidity and mortality, although the disease is usually diagnosed before adulthood in other organ systems. To date, no successful treatment of pulmonary involvement by Niemann-Pick disease has been documented. We describe the case of a patient with Niemann-Pick Type B disease who presented with extensive endogenous lipoid pneumonia and life-threatening hypoxia following bypass grafting for severe coronary artery disease. A surgical lung biopsy at the time of grafting revealed characteristic histology and ultrastructural features of Niemann-Pick disease, with confirmatory findings in biochemical studies. Because of the severity of the patient's symptoms, bilateral whole-lung lavage was undertaken, leading to symptomatic improvement, lessening of parenchymal opacification on high-resolution computed tomographic scanning, and a marked improvement in resting arterial oxygen tension while breathing air to 10.3 kPa from 8.4 kPa. Whole-lung lavage may be a potentially useful modality of treatment for patients with pulmonary involvement by Niemann-Pick Type B disease.

Publication types

  • Case Reports

MeSH terms

  • Anti-Inflammatory Agents / therapeutic use
  • Biopsy
  • Blood Gas Analysis
  • Bronchoalveolar Lavage / instrumentation
  • Bronchoalveolar Lavage / methods*
  • Combined Modality Therapy
  • Dyspnea / etiology
  • Forced Expiratory Volume
  • Humans
  • Macrophages, Alveolar / pathology
  • Male
  • Middle Aged
  • Myocardial Infarction / etiology
  • Myocardial Infarction / surgery
  • Niemann-Pick Diseases / complications*
  • Pneumonia, Lipid / diagnosis
  • Pneumonia, Lipid / etiology*
  • Pneumonia, Lipid / metabolism
  • Pneumonia, Lipid / physiopathology
  • Pneumonia, Lipid / therapy*
  • Steroids
  • Tomography, X-Ray Computed
  • Treatment Outcome
  • Vital Capacity


  • Anti-Inflammatory Agents
  • Steroids