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.
An integrated clinical approach to diffuse cystic lung diseases / S. Harari, G. Paciocco. - In: SARCOIDOSIS VASCULITIS AND DIFFUSE LUNG DISEASES. - ISSN 1124-0490. - 22:1(2005), pp. S31-S39. ((Intervento presentato al 1. convegno RIPID International Congress on Rare Pulmonary Diseases and Orphan Drugs in Respiratory Medicine tenutosi a Milano nel 2005.
An integrated clinical approach to diffuse cystic lung diseases
S. Harari;
2005
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.Pubblicazioni consigliate
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