An integrated clinical approach to diffuse cystic lung diseases

Sergio Harari, Giuseppe Paciocco

Research output: Contribution to journalArticlepeer-review


Lymphangioleiomyomatosis and Langerhans cell Histiocytosis are two different diseases that can involve the lungs, with unknown etiology and origin. Both are rare, present a similar radiological pattern (multiple nodules and cysts in the lungs) and may have a similar clinical presentation, with progressive dyspnea, hemoptysis, pneumothorax and a variable course usually culminating in respiratory failure. Due to these similarities a differential diagnosis may be difficult in some instances. This review underlines differences in epidemiology, pathogenesis and prognosis that could assist clinicians in making a differential diagnosis of these rare and severe pulmonary diseases.

Original languageEnglish
JournalSarcoidosis Vasculitis and Diffuse Lung Diseases
Issue numberSUPPL. 1
Publication statusPublished - Dec 2005


  • Bronchoalveolar lavage
  • Cystic lung diseases
  • High-resolution computed tomography
  • Langerhans cell histiocytosis
  • Lymphangioleiomyomatosis
  • Pulmonary arterial hypertension
  • Pulmonary function tests
  • Six-minute walking test
  • Trans-bronchial biopsies

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine


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