Abstract Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by accumulation of lipoproteinaceous surfactant within the alveoli due to impaired clearance by dysfunctional alveolar macrophages or disrupted granulocyte-macrophage colony-stimulating factor (GMCSF) signaling. Standard therapy for symptomatic disease is wholelung lavage (WLL), with adjunctive GMCSF therapies; lung transplantation is reserved for refractory or fibrotic disease. The incidence of recurrence and the optimal surveillance strategy remain undefined. We report a single complex case of a worsening dyspneic 73-year-old male that illustrates the clinical challenge of diagnosing and treating recurrent PAP three years after a bilateral sequential lung allograft. The initial Periodic Acid-Schiff (PAS) was negative in bronchoalveolar lavage (BAL) despite a crazypaving pattern on high-resolution computed tomography (HRCT) and positive anti-GMCSF antibody. Repeat BAL three years later showed abundant PASpositive lipoproteinaceous material, confirming recurrence of PAP. Our case was complicated by three concomitant opportunistic infections: Aspergillus spp., Mycobacterium avium complex, and Nocardia spp. Atypically for PAP, during this same interval, spirometry showed deterioration in the form of a worsening obstructive pattern (FEV1 110% predicted decreased gradually to 99% predicted and the FEV1/FVC ratio dropped from 91 % to 68 %). This highlights how infection can confound the clinical presentation in an immunocompromised host. PAP was treated with inhaled sargramostim (recombinant GMCSF) initiated when recurrence became evident, together with two sequential wholelung lavages. Both lavages resulted in dramatic clearance of alveolar material on followup HRCT, marked improvement in dyspnea, and partial recovery of spirometric parameters (FEV1 rose to 105 % predicted and FEV1/FVC improved to 74 % at four-months postlavage). This case demonstrates that negative BAL cytology should not exclude PAP in the appropriate clinical context and emphasizes the need for multimodal diagnostic integration (combining imaging, serology, cytology, and pulmonaryfunction testing) and expedited multidisciplinary coordination in posttransplant PAP surveillance. This abstract is funded by: None
Lopez et al. (Fri,) studied this question.