An integrated clinical approach to diffuse cystic lung diseases.
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.
Harari
Sergio
S
Unità Operativa di Pneumologia, UTIR Servizio di Fisiopatologia Respiratoria e Laboratorio di Emodinamica Polmonare, Ospedale San Giuseppe, AFAR, Milano, Italy. sharari@fatebenefratelli.it
Paciocco
Giuseppe
G
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