This correlation has been studied since the 1930s, and it is believed that exposure to silica can lead to various autoimmune diseases, such as systemic lupus erythematosus, rheumatoid arthritis, and systemic sclerosis. The exact mechanisms behind this link remain unclear. This study aimed to evaluate the prevalence of connective tissue diseases (CTD) and positive autoantibodies in pneumoconiosis patients and the association between radiological categorization and autoantibodies. The study included 317 subjects. Pneumoconiosis cases' clinical, radiographic classification, and laboratory results were compared to autoantibody-positive and autoantibody-negative cases, and CTD cases were examined. The prevalence of the CTD in pneumoconiosis was 6%. Sjögren syndrome (n = 5) was the most common disease associated with pneumoconiosis. Autoantibodies were found in 83 (26.2%) pneumoconiosis cases. Antinuclear antibody positivity was highest (n = 75
23.65%). More than 1 autoantibody was positive in 23 cases (7.25%), while rheumatoid factor was positive in 9 cases (2.84%). It has been shown that autoantibody positivity is 2.79 times higher in cases with category 3 and above pneumoconiosis. The prevalence of CTD and autoantibody positivity associated with pneumoconiosis is higher than in the general population. The presence of autoantibody positivity is associated with advanced pneumoconiosis disease.