ERİŞKİN KİSTİK FİBROZİS HASTALARIN İZLEMİNDE ASPERGİLLUS ENFEKSİYONUN SIKLIĞI, KLİNİK ÖZELLİKLERİ ve HASTALIĞIN PROGNOZUYLA İLİŞKİSİ

Abstract

MOKHTARI MOGHANJOGHI A., The Frequency and Clinical Features of Aspergillus Infection in Adult Patients with Cystic Fibrosis and its Relevance to Disease Prognosis. Department of Respiratory Diseases, Hacettepe University, Specialty Thesis, Ankara, 2024. Cystic Fibrosis is one of the most common lethal hereditary multisystemic diseases. The condition and function of the respiratory system are crucial factors for the patient's survival and quality of life. Pulmonary infections and exacerbations are the most significant factors leading to damage and advancing morbidity and mortality of the disease. The role and importance of fungal infections in this regard have not been investigated as thoroughly as bacteria, and their place in managing the disease process remains debatable. The aim of our study is to investigate the characteristics, frequency, and impact of Aspergillus infection on prognosis in patients with cystic fibrosis. Adults diagnosed with CF followed up at our center between January 2017 and January 2023 were included in the study. Basic demographic, clinical data, and laboratory results of the patients were retrospectively examined from the hospital electronic database. The impact of Aspergillus infection on respiratory function (FEV1) and survival (annual pulmonary exacerbation frequency, CF-ABLE (4-year), French (3-year), and US-CFF (5-year) prognostic score systems) was investigated using SPSS v.25. A total of 118 patients were included in the study, 44 (37%) of whom were female, with a mean age of 26.3±6.8 years, mean BMI of 21.05±4.10 kg/m^2, and mean FEV1 of 64.41±24.84%. Aspergillus infection did not have a negative effect on patients’ growth and development; however, one year after infection, there was a significant average decrease of 9.53% (95% CI 5.70-13.72) in FEV1 (p<0.001). The FEV1 value of the Aspergillus (+) cohort was significantly lower than that of the control cohort, with a mean difference of 7.42% (95% CI 0.06-14.75) after 2 years (p=0.04). Regarding the annual pulmonary exacerbation frequency, the Aspergillus (+) cohort had a significantly worse prognosis than the control cohort with a difference of 0.58 (95% CI 0.20-0.97) (p=0.003). A significant deterioration was observed in disease severity and prognostic scores one year after infection in the study cohort (CF-ABLE p=0.001; French and US-CFF p<0.001). There was no significant difference in the prognostic scores between the Asp (+) and Asp (-) groups over the 6-year study period. However, disease severity and prognosis deteriorated more rapidly in the Asp (+) cohort compared to the control cohort (CF-ABLE p=0.001; French p<0.001 and US-CFF p=0.009). In conclusion, Aspergillus infection did not have a significant negative effect on the development, clinical course, respiratory function, and prognosis of patients in the short term (<1 year), regardless of clinical phenotype. However, a negative impact was observed on respiratory function, pulmonary exacerbation frequency, and prognostic scores from the intermediate term (1-2 years) until the end of the study period.