ADOPTING ORPHAN DISEASES RARE INTERSTITIAL LUNG DISEASES

adoptingorphandiseaseskapak

PULMONER REHABILITATION IN RARE INTERSTITIAL LUNG DISEASES

Ayşenur Yılmaz

Burdur Mehmet Akif Ersoy University, Faculty of Health Science, Department of Physiotherapy and Rehabilitation, Burdur, Türkiye

Yılmaz A. Pulmoner Rehabilitation in Rare Interstitial Lung Diseases. In: Altinisik G, McCormack FX, editors. Adopting Orphan Diseases: Rare Interstitial Lung Diseases. 1st ed. Ankara: Türkiye Klinikleri; 2025. p.257-268.

ABSTRACT

Interstitial lung diseases (ILDs) are a heterogeneous group of disorders characterized by inflammation and/or fibrosis of the lung parenchyma, leading to impaired gas exchange and progressive respiratory dysfunction. A common feature of these conditions is their chronic course, which significantly reduces patients’ quality of life. Pulmonary alveolar proteinosis (PAP) is a rare subtype of ILD characterized by the abnormal accumulation of surfactant within the alveoli. This accumulation disrupts normal gas exchange and results in respiratory symptoms such as hypoxemia, dyspnea, and ineffective cough. Whole lung lavage (WLL) is currently considered the gold standard treatment for PAP, offering temporary relief of symptoms. However, WLL is invasive, carries the risk of procedure-related complications, and often requires repeated applications. Therefore, the development of safer, non-invasive, and supportive treatment approaches has become an important necessity for both patient comfort and long-term disease management. Pulmonary rehabilitation (PR), particularly when structured in an individualized and disease-specific manner, has emerged as a promising complementary strategy in the comprehensive management of rare ILDs, including PAP. Among the core components of PR, inspiratory muscle training (IMT) and expiratory muscle training (EMT) have shown evidence-based effectiveness in chronic lung diseases by enhancing respiratory muscle strength, improving cough efficiency, increasing exercise tolerance, and elevating quality of life. PAP, although supported by limited clinical data, has gained more attention in the literature in recent years due to the growing number of reported cases, making it a suitable model for evaluating the clinical outcomes of these interventions. This chapter presents the outcomes of a patient with PAP who underwent a tailored physiotherapy program. The structured rehabilitation led not only to improvements in physiological parameters but also to significant gains in functional capacity and psychosocial well-being. These findings underscore the crucial role of physiotherapy within multidisciplinary, patient-centered care models. In the management of rare and complex ILDs, PR can be considered an effective non-pharmacological therapeutic option. Expanding the implementation of such programs and sharing their results in scientific platforms may provide valuable contributions not only for PAP but also for the management of other rare ILDs.

Keywords: Pulmonary alveolar proteinosis, interstitial lung disease; Rare interstitial lung disease; Pulmonary rehabilitation; Inspiratory muscle training

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