Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis.

A woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence of parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare, but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improving quality of life.