title: An integrated clinical approach to diffuse cystic lung diseases.
abstract: 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.
authors.affiliation: Unità Operativa di Pneumologia, UTIR Servizio di Fisiopatologia Respiratoria e Laboratorio di Emodinamica Polmonare, Ospedale San Giuseppe, AFAR, Milano, Italy. sharari@fatebenefratelli.it
authors.forename: Sergio
authors.initials: S
authors.lastname: Harari
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Paciocco
mesh_terms: D001707:Biopsy, Needle; D001992:Bronchoalveolar Lavage Fluid / Q000166:cytology; D003560:Cysts / Q000175:diagnosis* / Q000453:epidemiology; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006646:Histiocytosis, Langerhans-Cell / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D007150:Immunohistochemistry; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000453:epidemiology; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000453:epidemiology; D008297:Male; D011379:Prognosis; D012129:Respiratory Function Tests; D018570:Risk Assessment; D015996:Survival Rate; D014057:Tomography, X-Ray Computed