NDLI: Assessment and management of pulmonary alveolar proteinosis in a reference center

Content Provider	PubMed Central
Author	Campo, Ilaria Mariani, Francesca Rodi, Giuseppe Elena, Paracchini Eric, Tsana Piloni, Davide Nobili, Isabella Zamir, Kadija Corsico, Angelo Isa, Cerveri Chalk, Claudia Trapnell, Bruce C. Braschi, Antonio Tinelli, Carmine Luisetti, Maurizio
Copyright Year	2013
Abstract	Pulmonary alveolar proteinosis (PAP) is a term defining an ultra-rare group of disorders characterised by a perturbation in surfactant homeostasis, resulting in its accumulation within airspaces and impaired gas transfer. In this report we provide data from a cohort of PAP patients (n = 81) followed for more than two decades at the San Matteo University Hospital of Pavia, Italy. In agreement with other large series in PAP individuals, 90% of the study subjects were affected by autoimmune/idiopathic PAP, while the remaining subjects were divided as follow: congenital 1%, secondary 4% and PAP-like 5%. The disease affected males and females with a ratio of 2:1 and approximately one third of PAP patients were lifelong nonsmokers. Occupational exposure was reported in 35% of subjects in this series. With reference to the PAP clinical course, in 29 patients (7% with spontaneous remission) disease severity did not necessitate whole lung lavage (WLL) in the long-term follow up. On the other hand, 44 PAP patients underwent therapeutic WLL: in 31 subjects a single WLL was sufficient to provide long term, durable benefit, whereas 13 patients required multiple WLLs. The intra-patient mean interval between two consecutive WLLs was 15.7 ± 13.6 months. When baseline data among never lavaged and PAP patients lavaged at least once were compared, the need for lavage was significantly associated with serum biomarkers (CEA, Cyfra, LDH), lung function parameters forced vital capacity (FVC), and lung diffusing capacity (Dlco). We conclude that patient cohorts with an ultra-rare disease, such as PAP, referred to a single reference center, can provide useful information on the natural history and clinical course of the disease.
Related Links	http://dx.doi.org/10.1186/1750-1172-8-40
Starting Page	40
File Format	PDF
ISSN	17501172
e-ISSN	17501172
Journal	Orphanet Journal of Rare Diseases
Volume Number	8
Language	English
Publisher	BioMed Central
Publisher Date	2013-03-13
Access Restriction	Open
Rights Holder	BioMed Central
Subject Keyword	Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Pharmacology (medical) Genetics (clinical)

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