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2018 ; 8
(1
): ä Nephropedia Template TP
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Gastrointestinal Manifestations of Systemic Sclerosis
#MMPMID30057856
McFarlane IM
; Bhamra MS
; Kreps A
; Iqbal S
; Al-Ani F
; Saladini-Aponte C
; Grant C
; Singh S
; Awwal K
; Koci K
; Saperstein Y
; Arroyo-Mercado FM
; Laskar DB
; Atluri P
Rheumatology (Sunnyvale)
2018[]; 8
(1
): ä PMID30057856
show ga
Systemic sclerosis (SSc) is a rare autoimmune disease characterized by
fibroproliferative alterations of the microvasculature leading to fibrosis and
loss of function of the skin and internal organs. Gastrointestinal manifestations
of SSc are the most commonly encountered complications of the disease affecting
nearly 90% of the SSc population. Among these complications, the esophagus and
the anorectum are the most commonly affected. However, this devastating disorder
does not spare any part of the gastrointestinal tract (GIT), and includes the
oral cavity, esophagus, stomach, small and large bowels as well as the liver and
pancreas. In this review, we present the current understanding of the
pathophysiologic mechanisms of SSc including vasculopathy, endothelial to
mesenchymal transformation as well as the autoimmune pathogenetic pathways. We
also discuss the clinical presentation and diagnosis of each part of the GIT
affected by SSc. Finally, we highlight the latest developments in the management
of this disease, addressing the severe malnutrition that affects this vulnerable
patient population and ways to assess and improve the nutritional status of the
patients.