Symptoms in Patients with SSc may have small and large bowel involvement
Involvement of the small intestine (17%–57% patients) and colon (10%–50% patients) is common. The major manifestations are due to diminished peristalsis with resulting stasis and dilatation. The diminished peristalsis can lead to bacterial overgrowth (33%–40% of patients; positive hydrogen breath test, high folate, ≥10 5 organisms/mL of jejunal fluid). Later, malabsorption can be a major problem (low albumin, low B6/B12/folate/25-OH vit D, high fecal fat, low d -xylose absorption test, low β carotene, high international normalized ratio due to low vitamin K). Patients may report abdominal distention and pain due to dilated bowel, obstructive symptoms from intestinal pseudo-obstruction, or diarrhea from bacterial overgrowth or malabsorption. If the malabsorption becomes severe, the patient may have signs of vitamin deficiencies or electrolyte abnormalities.
Patients with large bowel involvement affecting the anorectum can suffer from debilitating fecal incontinence. This may be due to a neuropathy more than sphincter atrophy/fibrosis. Atrophy and thinning of the muscular wall in the colon can lead to “wide mouth” diverticulae. It should be emphasized that barium studies are relatively contraindicated in SSc patients with poor GI motility owing to the risk of barium impaction. Rectal prolapse has also been reported.
How are small and large bowel problems managed in these patients?
• Stimulation of gut motility with domperidone, metoclopramide, or erythromycin can be given 30 minutes before meals to stimulate gut motility. It is important to note that data for prokinetic agents outside of short-term use for gastric motility is extremely limited.
• Injectable octreotide may help in severe or refractory cases.
• Fiber may help colonic dysmotility. Some patients may experience symptoms of bloating, but dietary fiber intake through 100% whole grain products, fruits, and vegetables is generally safe and recommended.
• Maintaining exercise may help move food through the digestive tract.
• Diarrhea is commonly treated initially as if it were due to bacterial overgrowth. An antibiotic is given that can partially decrease the gut flora, such as rifaximin (550 mg thrice a day [TID]), ciprofloxacin (500 mg twice a day [BID]), amoxicillin-clavulanic acid (875 mg BID), or metronidazole (500 mg TID) for 10 days. In most cases, this stops the diarrhea. In patients with relapse, cyclic antibiotic courses can be used.
• Agents that slow intestinal motility, such as paregoric or loperamide, should be avoided.
• If the diarrhea persists, a malabsorption work-up should be pursued. Most patients with malabsorption can be treated with supplemental vitamins, minerals, and predigested liquid food supplements. A rare patient will need total parenteral nutrition.
• Dietary modification (for symptoms of bloating, alternating diarrhea, and constipation): patients may consider removing specific food that may be more difficult to digest (e.g., gluten and lactose). A food diary may allow patients to identify specific triggers. Some patient advocacy groups suggest a low FODMAP (fermentable, oligosaccharides, disaccharides, monosaccharides, and polyols) diet. Additional online data can be found at the end of this chapter. Despite the potential merits of eliminating some “problem foods,” patients should be advised to exercise caution with dietary restriction given the increased risk of malnutrition in SSc and the fact that many patients can be severely underweight. Consultation with a dietician should be considered.
• Fecal incontinence is treated with biofeedback, sacral nerve stimulation (limited data on tibial nerve stimulation), and/or surgical repair.
• Rectal prolapse includes management of constipation and possibly surgical correction.