CLINICAL CHARACTERISTICS OF PROGRESSIVE PULMONARY FIBROSIS IN PATIENTS WITH AUTOIMMUNE ASSOCIATED ILD
Nagy Tamás
Clinical Medicine
Dr. Reusz György
SE Pulmonológiai Klinika, Könyvtár
2024-05-14 14:30:00
Pulmonology
Dr. Losonczy György
Dr. Müller Veronika
Dr. Németh Tamás
Dr. Bozsányi Szabolcs
Dr. Tamás László
Dr. Fent László
Dr. Rojkó Lívia
ILDs are a heterogeneous group of conditions with various etiologies; however, functional progression can worsen the prognosis and the outcome. IPF is regarded as a prototype disease for progression, but ILDs with autoimmune features, such as CTD-ILD and IPAF, can show progressive decline during the disease course, thus deteriorating the QoL and the survival. Progressive non-IPF ILD in PPF is characterized by clinical symptom worsening, lung function deterioration and increased fibrosis on HRCT scans.
The focus of this thesis and the retrospective studies conducted, was the analysis of clinical characteristics and the identification of possible predictors of progression and treatment options in different ILD populations. In the study population ILDs were diagnosed by MDD at the Department of Pulmonology, Semmelweis University. In the CTD-ILD Study, we included a total of 107 patients with CTD-ILD and IPAF, while a total of 84 IPF and SSc-ILD patients with physiologic lung function parameters were analyzed in SSc-ILD Study. At baseline patient characteristics, functional parameters and imaging were registered, while functional parameters were evaluated at every follow-up.
Our results showed that in the study populations most ILD patients receiving ISU and/or antifibrotic treatment - depending on the underlying condition - showed improved functional outcomes. Importantly, patient-reported symptoms, such as cough (especially dry cough), and the presence of PH as a lung-related comorbidity, should be taken into consideration in connection with the disease progression in SSc-ILD patients. Despite the age difference, exercise capacity was similar in IPF and SSc-ILD patients, emphasizing the involvement of additional factors in physical performance. Interestingly, regardless of the typical restrictive parameters of IPF, CO diffusion per alveolar unit - KLCO - was lower in the SSc-ILD population, indicating the presence of altered respiratory mechanisms.
There is emerging need for prospective studies to detect new progression markers of functional deterioration and to develop guidelines for the optimal timing of therapy introduction and treatment of choice in this special ILD population even in early stages with physiologic lung function remaining.
