Title Lung involvement in systemic sclerosis, literature review /
Translation of Title Lung Involvement in Systemic Sclerosis, Literature review.
Authors Kappes, Christopher Tobias
Full Text Download
Pages 24
Keywords [eng] Systemic sclerosis, Pulmonary involvement, Interstitial lung disease, Pulmonary arterial hypertension, Pathology, Biomarkers, Therapy, Krebs von den Lungen-6
Abstract [eng] Systemic sclerosis, a complex autoimmune disorder affecting multiple organ systems, progresses irreversibly if left untreated. Early treatment initiation is crucial to control systemic sclerosis, particularly for pulmonary involvement which carries the highest mortality rate. The most important factor for survival is time of treatment initiation thus late detection and the subsequent late treatment initiation worsens the prognosis. Symptoms in early stages are often non-specific and can occur without radiological evidence, whereas other patients are asymptomatic despite physical findings, necessitating diagnosis through a combination of symptoms, physical findings, pulmonary functional test and high-resolution computer tomography. To increase diagnostic accuracy investigations have been conducted exploring the additional use of imaging and laboratory biomarkers in combination with the aforementioned methods. Sonography stands out as a promising imaging modality due to its easy accessibility and affordability. In combination with the already established diagnosis methods, sonography shows a possibility of being added as fourth method. Amongst the most promising laboratory biomarkers, the inflammatory marker axis consisting of interleukin-1ß, interleukin 17 and interleukin 23 might be a possible addition to the already established diagnostic methods due to their correlation. Krebs von den Lungen-6 in combination with cytokeratin 19 fragments conveys information about lung function status and fibrosis extent. Matrix metalloproteinase 7 is an alternative to cytokeratin 19 and just like carbohydrate antigen 15.3 is more readily available as most hospitals have established protocols for tumor marker testing. Pneumoprotein surfactant protein D can be used in monitoring disease progression and to confirm treatment efficacy. The use of biomarkers could revolutionize the approach to treatment initiation, as currently guidelines dictate treatment initiation only upon meeting specific criteria. Immunosuppressive therapy stands as the cornerstone for treating interstitial lung disease, while the optimal treatment strategy for pulmonary artery hypertension remains a topic of debate.
Dissertation Institution Vilniaus universitetas.
Type Master thesis
Language English
Publication date 2024