The challenge of leading with multiple and severe gastrointestinal complications in a patient with systemic sclerosis

Systemic sclerosis (SSc) is a rare chronic autoimunne-mediated connective tissue disorder. Although the aetiology of the disease remains undetermined, systemic sclerosis is characterized by fibrosis and proliferative vascular lesions of the skin and internal organs. Prevalence of the disease in Europe and USA is estimated at 8-30 per 100.000 people with an annual incidence of 1-2 per 100.000 people1 . Involvement of the gastrointestinal tract can be found at any form of SSc, but is more frequent in patients with diffuse disease2 . Gastrointestinal symptoms are seen in 90% of all the patients with SSc, and are the presenting feature of disease in 10% of the individuals. The involvement can extend from the mouth to the anus, with the oesophagus and anorectum most frequently affected. Gastrointestinal involvement is the leading cause of morbidity and the third most commmon cause of mortality in patients with systemic sclerosis, with cardiopulmonary and renal involvement being the first and second most common causes, respectively3 . Severity of gastrointestinal disease has been shown to be a marker of worse prognosis and mortality in patients with systemic sclerosis4,5. Gastrointestinal disease can be present with esophageal dysmotility, gastroparesis, small bacterial intestinal overgrowth, colonic dysmotility and fecal incontinence. The symtoms have a profound impact on quality of life. Severe gastrointestinal disease is associated with pseudo-obstruction and malabsorption and may result in dependence on enteral or total parenteral nutrition6 . Given the heterogenicity of symptoms and the complexitie related to the management, these patients become routinely a challenge.