Rare infiltrative lung diseases: a challenge for clinicians.
Rare diffuse infiltrative lung diseases are a challenge for clinicians, radiologists, and pathologists for at least three reasons: (a) their low incidence and prevalence hamper the acquisition of expertise and frequently the diagnosis is delayed; (b) therapeutic actions are mainly empirical and based on steroid use, and (c) pathogenetic events are difficult to explain and only recently new therapeutic measures taking advantage of innovative genetic and/or immunopathogenetic studies have been suggested. In this review rare diffuse lung disorders are briefly discussed (pulmonary alveolar proteinosis, inherited lipidoses, acute eosinophilic pneumonia, amyloidosis, pulmonary ossification, pulmonary alveolar microlithiasis). The list is obviously not exhaustive and arbitrarily chosen. The intent is, however, to emphasize that in this difficult field multidisciplinary expertise and the knowledge of the most recent pathogenetic mechanisms have the main role in diagnosis and treatment.
Dipartimento di Malattie dell'Apparato Respiratorio e del Torace, Ospedale GB Morgagni, IT-47100 Forlì, Italy. vepolet@tin.it
Venerino
V
Poletti
Ulrich
U
Costabel
Gian Luca
GL
Casoni
Caterina
C
Bigliazzi
Marjolein
M
Drent
Dario
D
Olivieri
D000208:Acute Disease; D000686:Amyloidosis / Q000175:diagnosis / Q000209:etiology; D022861:Hermanski-Pudlak Syndrome / Q000175:diagnosis / Q000209:etiology; D006801:Humans; D008064:Lipidoses / Q000175:diagnosis / Q000209:etiology / Q000235:genetics; D020347:Lithiasis / Q000175:diagnosis / Q000209:etiology; D008171:Lung Diseases / Q000175:diagnosis* / Q000209:etiology*; D009999:Ossification, Heterotopic / Q000175:diagnosis / Q000209:etiology; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000209:etiology; D011650:Pulmonary Alveoli; D011657:Pulmonary Eosinophilia / Q000175:diagnosis / Q000209:etiology; D035583:Rare Diseases / Q000175:diagnosis* / Q000209:etiology*