TY - JOUR
T1 - Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis
AU - Bento da Silva, Ana
AU - Lourenço, Maria Helena
AU - Cunha-Branco, Jaime
AU - Gonçalves, Maria João
N1 - Publisher Copyright:
© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.
PY - 2023/4/4
Y1 - 2023/4/4
N2 - 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.
AB - 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.
KW - Connective tissue disease
KW - Malabsorption
KW - Malnutrition
KW - Parenteral / enteral feeding
UR - http://www.scopus.com/inward/record.url?scp=85151806720&partnerID=8YFLogxK
U2 - 10.1136/bcr-2022-254537
DO - 10.1136/bcr-2022-254537
M3 - Article
C2 - 37015763
AN - SCOPUS:85151806720
SN - 1757-790X
VL - 16
JO - BMJ case reports
JF - BMJ case reports
IS - 4
M1 - e254537
ER -