Lower urinary tract symptoms (LUTS) have long been overlooked in systemic sclerosis (SSc). However, they are out of proportion of what would be expected based on age, sex, and presence of usual risk factors. Thus, there must be specific scleroderma-related mechanisms to result in LUTS. Fibrosis, nervous involvement (notably, dysautonomia), early signs of menopauses, and functional restriction play certainly a role, but available evidence shows inconsistent results. Thus, these factors are not sufficient to explain all aspect of LUTS in SSc. In vitro experiments point out a promising alternative mechanism, already observed in other rheumatologic diseases: an antibody-mediated etiology. However, more research is needed to better understand the pathophysiology of LUTS in SSc and develop specific treatment.