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Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis
  1. Ana Bento da Silva1,2,
  2. Maria Helena Lourenço1,2,3,
  3. Jaime Cunha-Branco1,3 and
  4. Maria João Gonçalves1,2,4
  1. 1Rheumatology, Centro Hospitalar de Lisboa Ocidental - Hospital de Egas Moniz, Lisbon, Portugal
  2. 2Rheumatology, Comprehensive Health Research Center, NOVA Medical School, Lisbon, Portugal
  3. 3Faculdade de Ciências Médicas, NOVA Medical School, Lisbon, Portugal
  4. 4Escola Superior de Tecnologia da Saúde de Lisboa, Lisbon, Portugal
  1. Correspondence to Dr Ana Bento da Silva; adbentosilva{at}


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.

  • Malabsorption
  • Parenteral / enteral feeding
  • Malnutrition
  • Connective tissue disease

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  • Contributors ABdS contributed actively for the diagnosis and treatment management of the patient presented in this case report and was also responsible for writing the first draft of the manuscript. MJG conceived the presented idea, was the principal physician of the patient and was the main reviewer of the first draft of the manuscript. MHL and JB contributed for the management of the patient and commented on previous versions of the manuscript. All authors read and approved the final manuscript and agreed with its submission.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.