Pneumatosis cystoides intestinalis (PCI) in a patient with undiagnosed systemic sclerosis

Abstract

Pneumatosis cystoides intestinalis (PCI) refers to the presence of gas within the wall of the small or large intestine. The pathophysiology is incompletely understood and is probably multifactorial in nature. PCI is a known but rare complication of systemic scleroderma, and the aetiology of PCI in patients with scleroderma is not fully understood. We present the case of a patient who was referred to gastroenterology clinic by her general practitioner for investigation of 8 months of weight loss, urgency, diarrhoea, bloating and crampy abdominal pain. Extensive investigations were performed to exclude infective, inflammatory or malignant aetiologies for these symptoms. She was diagnosed with PCI on her colonoscopy and was subsequently screened for secondary causes. Our patient was diagnosed with the limited cutaneous (CREST) variant of systemic scleroderma. This case report illustrates that PCI could be an uncommon presentation of systemic sclerosis, therefore clinicians should be aware of the association between these conditions.