Background: Pneumatosis intestinalis, characterised by the presence of intramural bowel gas, is a radiological finding with varied clinical implications, ranging from incidental detection to severe underlying pathology, including bowel ischaemia or perforation. Its association with systemic sclerosis, a connective tissue disorder, remains rare and poorly understood, presenting unique diagnostic and therapeutic challenges.
Methods: A comprehensive literature review was conducted, analysing 34 documented cases of pneumatosis intestinalis associated with systemic sclerosis, examining demographic profiles, clinical features, diagnostic findings, and outcomes of various management strategies.