Pulmonary alveolar proteinosis (PAP) is a syndrome characterised by accumulation of proteinaceous, periodic acid-Schiff positive material within airspaces due to disruption of surfactant homeostasis [1]. In 95% of cases, PAP is associated with increased serum levels of auto-antibodies neutralising granulocyte-macrophage colony-stimulating factor and, therefore, this form is referred to as “auto-immune PAP” [2]. Irrespective of the underlying cause, the current standard of care for PAP is represented by whole lung lavage (WLL) and, to a lesser extent, bronchoscopic lobar/segmental lavage [3]. WLL has played such a critical role in the management of patients affected by PAP, whose natural history had been markedly influenced by its application, that PAP is now no longer considered a potentially lethal disease. Although WLL has been performed for 50 yrs, having been first described in 1963 [4], it is still a procedure performed in selected centres that is not standardised
Call for an international survey on therapeutic lavage for pulmonary alveolar proteinosis.
LUISETTI, MAURIZIO
2012-01-01
Abstract
Pulmonary alveolar proteinosis (PAP) is a syndrome characterised by accumulation of proteinaceous, periodic acid-Schiff positive material within airspaces due to disruption of surfactant homeostasis [1]. In 95% of cases, PAP is associated with increased serum levels of auto-antibodies neutralising granulocyte-macrophage colony-stimulating factor and, therefore, this form is referred to as “auto-immune PAP” [2]. Irrespective of the underlying cause, the current standard of care for PAP is represented by whole lung lavage (WLL) and, to a lesser extent, bronchoscopic lobar/segmental lavage [3]. WLL has played such a critical role in the management of patients affected by PAP, whose natural history had been markedly influenced by its application, that PAP is now no longer considered a potentially lethal disease. Although WLL has been performed for 50 yrs, having been first described in 1963 [4], it is still a procedure performed in selected centres that is not standardisedI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
