CHEST Guidelines-Pulmonary-Alveolar-Proteinosis-After-Allogeneic-He

Pulmonary-Alveolar-Proteinosis-After-Allogeneic-He

Pdf Summary

The document is a study conducted by the French Société Francophone de Greffe de Moelle et Thérapie Cellulaire, focusing on pulmonary alveolar proteinosis (PAP) in adults following allogeneic hematopoietic stem-cell transplantation (HSCT). PAP is marked by a dysfunction in lung surfactant clearance due to impaired activity of alveolar macrophages, leading to surfactant buildup in the alveoli. It can be autoimmune, hereditary, or secondary to other conditions. The study surveyed 43 HSCT centers in France, identifying five adult-onset secondary PAP cases post-transplantation.<br /><br />These cases, collected from 2016 to 2019, were classified into two clinical categories: early-onset acute PAP, occurring during the aplasia period, and late-onset progressive PAP linked to potential drug-induced macrophage dysfunction. Patients presented with varied symptoms, including respiratory distress and abnormal pulmonary function tests. Diagnosis generally involved bronchoalveolar lavage (BAL) and, in some cases, lung biopsy. Histology commonly featured periodic acid-Schiff (PAS) positive material, characteristic of PAP.<br /><br />The study found a significant association between PAP and certain medications such as ruxolitinib and sirolimus, which may disrupt macrophage functions. Management typically involved withdrawal of the suspected drug, and subsequent clinical improvement was observed in most cases. The study emphasizes using less invasive BAL with PAS staining for diagnosis to avoid risks associated with lung biopsies in immunocompromised patients undergoing HSCT. It also highlights the need to review patient medications and consider temporal relationships to PAP onset.<br /><br />Overall, the outcomes suggested that managing the underlying promoting factors could lead to favorable prognoses, and further underscores the role of medication review in PAP patients post-HSCT.

Keywords

pulmonary alveolar proteinosis

allogeneic hematopoietic stem-cell transplantation

alveolar macrophages

lung surfactant clearance

secondary PAP

bronchoalveolar lavage

ruxolitinib

sirolimus

immunocompromised patients

medication review