• Sjögren
• lung
• ILD
• phenotypes
• outcome
• progression

Lung involvement is among the most frequent extraglandular manifestations of Primary Sjogren’s Syndrome (pSS). Interstitial Lung Disease (ILD) presence is clearly associated with increased mortality and usually drives therapeutic choices. Indeed, lung involvement in pSS patients is a very complex phenomenon resulting from the potential combination of at least 3 pathophysiological mechanisms: bronchial disease, interstitial lung changes and lymphoproliferative manifestations.
In this complex scenario, this thesis addresses the phenotypic and prognostic stratification of pSS-ILD patients, representing a very actual challenge for the modern rheumatologist. Chapter 1 provides a brief introduction and outline of the thesis.
In Chapter 2 an extensive review of the literature highlights the many open questions and discusses the most controversial aspects in the field of pSS-related ILD, providing the background and rationale for the following original works.
Chapter 3 describes a monocentric retrospective study investigating the phenotypic heterogeneity of pSS-ILD patients in our cohort, as well as baseline risk factors for the development of ILD in pSS patients. This chapter also includes a cross-sectional work focusing on the characterization of glandular involvement of pSS-ILD patients in terms of clinical symptoms, functional tests, ultrasound and histopathological findings.
In Chapter 4 a retrospective cohort study describes the outcome and the rate of clinical, functional and radiologic progression of ILD in pSS patients.
Finally, Chapter 5 describes a new PhD project aimed at the identification of novel biological and imaging biomarkers for the early diagnosis as well as the phenotypic and prognostic stratification of ILD related to autoimmune systemic diseases.