Pneumatosis intestinalis in systemic sclerosis: navigating diagnostic and therapeutic challenges

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.

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.

Pneumatosis intestinalis in systemic sclerosis predominantly occurs in older female patients and is associated with gastrointestinal dysmotility and microvascular damage. Symptoms range from asymptomatic presentations to severe abdominal pain with obstructive symptoms, with the latter increasing the likelihood of surgical intervention. Non-operative management, encompassing antibiotic therapy, oxygen supplementation, and dietary modifications, was associated with favourable outcomes and the lowest mortality. In contrast, surgical intervention, often necessitated by suspected ischemia or perforation, was associated with significantly higher morbidity and mortality.

Pneumatosis intestinalis in systemic sclerosis is a complex clinical entity demanding a nuanced, multi-disciplinary approach to management. Non-operative management should remain the cornerstone of treatment for clinically stable presentations, with surgery reserved for those with clear indications. The absence of standardised guidelines underscores the need for further research to refine diagnostic criteria and therapeutic protocols, ultimately improving long-term outcomes in this complex intersection of gastrointestinal and systemic disease.