Telemedicine-Based Functional Monitoring in Pulmonary Alveolar Proteinosis: A Pilot Experience of Post-Whole-Lung Lavage Follow-up

Abstract

Background: Pulmonary alveolar proteinosis (PAP) is a rare lung disorder characterized by intra-alveolar accumulation of surfactant material, resulting in impaired gas exchange and functional limitations. Whole-lung lavage (WLL) remains the standard therapy; however, objective assessment of functional status is important during longitudinal follow-up, particularly for patients who require monitoring outside specialized centers. Objectives: This study evaluated the association between home-based six-minute walk test (6MWT) parameters and the need for repeat WLL in patients with PAP. Methods: In this exploratory observational study, 21 patients with PAP who had undergone at least one WLL were followed using a telemedicine-based monitoring approach. Patients completed home-based 6MWT assessments, including walking distance, oxygen saturation, and heart rate response. Dyspnea severity was assessed using the modified Medical Research Council (mMRC) scale; oxygen dependence and symptom-related parameters were also recorded. Exploratory binary logistic regression analysis was performed to evaluate factors associated with repeat WLL. Results: Patients requiring repeat WLL had significantly shorter 6MWT distances (297.6 ± 109.5 m vs. 429.1 ± 97.4 m, P = 0.009) and higher mMRC dyspnea scores (1.91 ± 0.94 vs. 0.72 ± 0.89, P = 0.002) than those not requiring repeat WLL. In an exploratory multivariable logistic regression analysis, a lower 6MWT distance was associated with the requirement for repeat WLL; however, after adjustment, none of the evaluated predictors showed a statistically significant independent association. Other parameters, including oxygen desaturation and demographic characteristics, were not independently associated with repeat WLL. Conclusions: The home-based 6MWT provides clinically relevant information on functional impairment and may serve as an adjunctive marker for longitudinal monitoring of patients with PAP. Telemedicine-based assessment is a feasible approach for remote collection of functional data; however, its effectiveness in improving clinical outcomes or guiding WLL decisions requires confirmation in larger prospective studies using validated assessment protocols.