Interstitial lung disease (ILD) is one of the most serious pulmonary complications associated with connective tissue diseases (CTDs), resulting in significant morbidity and mortality. Recently, lung ultrasound examination has appeared as a useful, feasible, non-invasive, radiation-free imaging technique with high sensitivity and specificity for the diagnosis of CTD-associated ILD. In this review, we discuss the literature concerning lung ultrasound findings (B-lines and pleural irregularities) in systemic sclerosis. We also examine the possible utility and potential limitations of lung ultrasound in other CTDs including: inflammatory myopathies, mixed connective tissue disease, and Sjögren's syndrome. Specifically, we compare the diagnostic accuracy of lung ultrasound with traditional imaging techniques and pulmonary functional tests.