Updated severity and prognosis score of pulmonary alveolar proteinosis: A multi-center cohort study in China.

ABSTRACT:

Background

The high-resolution computed tomography (HRCT) score is an important component of the severity and prognosis score of pulmonary alveolar proteinosis (SPSP). However, the HRCT score in SPSP only considers the extent of opacity, which is insufficient.

Methods

We retrospectively evaluated HRCT scores for 231 patients with autoimmune pulmonary alveolar proteinosis (APAP) from three centers of the China Alliance for Rare Diseases. The SPSPII was created based on the overall density and extent, incorporating the SPSP. The severity of APAP patients was assessed using disease severity scores (DSS), SPSP, and SPSPII to determine the strengths and weaknesses of the different assessment methods. We then prospectively applied the SPSPII to patients before treatment, and the c

SUBMITTER: Bai JW 

PROVIDER: S-EPMC9941621 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature