From the Guidelines
Scleroderma is characterized by gastrointestinal histologic findings of smooth muscle atrophy and gut wall fibrosis, resulting from microvasculature damage, collagen deposits, and inflammation, which ultimately lead to muscle dysfunction and fibrosis. The gastrointestinal involvement in scleroderma, as noted in a 2020 study published in the journal Gut 1, typically starts with microvasculature damage due to collagen deposits and inflammation, causing neural damage that progresses to muscle dysfunction and fibrosis. Key features of these histologic findings include:
- Smooth muscle atrophy
- Gut wall fibrosis
- Microvasculature damage
- Collagen deposits
- Inflammation
- Neural damage These changes can affect various parts of the gastrointestinal tract, including the esophagus, small intestine, and colon, leading to impaired motility and other complications. According to the study 1, the clinical outcome in elderly scleroderma patients is the poorest of all adult onset dysmotility patients, highlighting the importance of early recognition and management of gastrointestinal involvement in scleroderma. The study also notes that long-term parenteral nutrition can offer a safe and effective means of nutritional support in patients with severe small bowel involvement 1.
From the Research
Gastrointestinal Histologic Findings in Scleroderma
The gastrointestinal histologic findings in scleroderma are characterized by:
- Smooth muscle atrophy and fibrosis, leading to gastrointestinal motility disturbances 2, 3
- Collagen replacement of vascular and enteric smooth muscle, resulting in hypomotility, lumen dilatation, tensile rigidity, and eventual loss of organ functions 4
- Esophageal and intestinal manifestations, including gastroesophageal reflux disease (GERD), pseudo-obstruction, bacterial overgrowth, and defecatory disorders 5, 6, 2, 3
Specific Gastrointestinal Involvement
The gastrointestinal tract is commonly involved in scleroderma, with the esophagus being the most frequently affected, followed by the anorectal region, small bowel, stomach, and colon 2. The clinical presentation of these conditions ranges from asymptomatic to life-threatening 2.
Pathophysiology and Consequences
The pathophysiologic mechanism of gastrointestinal involvement in scleroderma appears to be one of smooth muscle atrophy and, to a lesser degree, fibrosis, resulting in gastrointestinal motility disturbances 2, 3. These changes may cause malnutrition, which can be a serious consequence of scleroderma 6, 3. Additionally, fecal incontinence is a common symptom of scleroderma that can lead to depression 6.