Gastrointestinal tract (GIT) involvement is a common cause of debilitating symptoms in patients with systemic sclerosis.
There are no disease modifying therapies for this condition and the treatment remains symptomatic, largely owing to the lack of a clear understanding of its pathogenesis.
Professor Cohen and colleagues from Philadelphia, USA investigated novel aspects of the pathogenesis of gastrointestinal involvement in systemic sclerosis.
The resesarch team summarized existing knowledge regarding the cardinal clinical gastrointestinal manifestations of systemic sclerosis, and its pathogenesis, emphasising recent investigations that may be valuable in identifying potentially novel therapeutic targets.
|Up to 8% of systemic sclerosis patients develop severe GIT symptoms|
|Alimentary Pharmacology & Therapeutics|
The team performed an electronic, and manual Google search.
The researchers noted that GIT is the most common internal organ involved in systemic sclerosis.
Any part of the GIT from the mouth to the anus can be affected.
The research team found that there is substantial variability in clinical manifestations and disease course and symptoms are nonspecific and overlapping for a particular anatomical site.
Gastrointestinal involvement can occur in the absence of cutaneous disease.
The team noted that up to 8% of systemic sclerosis patients develop severe GIT symptoms.
This subset of patients display increased mortality with only 15% survival at 9 years.
The researchers observed that dysmotiity of the GIT causes the majority of symptoms.
Recent investigations have identified a novel mechanism in the pathogenesis of GIT dysmotility mediated by functional anti-muscarinic receptor autoantibodies.
Professor Cohen's team comments, "Despite extensive investigation, the pathogenesis of gastrointestinal involvement in systemic sclerosis remains elusive."
"Although treatment currently remains symptomatic, an improved understanding of novel pathogenic mechanisms may allow the development of potentially highly effective approaches including intravenous immunoglobulin and microRNA based therapeutic interventions."