What is the management of scleroderma of the small bowel?

Management of Scleroderma of Small Bowel

The management of small bowel scleroderma requires a comprehensive nutritional support strategy, with parenteral nutrition being essential for patients with severe disease who cannot maintain adequate nutrition through enteral routes. 1

Pathophysiology and Clinical Presentation

Scleroderma affecting the small bowel is characterized by:

• Initial microvasculature damage from collagen deposits and inflammation
• Neural damage progressing to muscle dysfunction
• Eventually smooth muscle atrophy and gut wall fibrosis
• Manifestations of intestinal dysmotility and pseudo-obstruction

Small bowel involvement in scleroderma typically presents with:

• Intestinal pseudo-obstruction
• Malabsorption
• Small intestinal bacterial overgrowth (SIBO)
• Progressive malnutrition

Treatment Approach

Symptom Management

1. Prokinetic Agents

   • Most effective in early neuropathic phase
   • Options include metoclopramide, cisapride, octreotide, and erythromycin 2
   • Less effective in late myopathic phase

2. Management of SIBO

   • Rotating antibiotics to treat bacterial overgrowth 2
   • Common options include ciprofloxacin, metronidazole

3. Pain Management

   • Avoid high doses of opioids which can worsen dysmotility 1
   • Consider pain specialist involvement if patient has been on long-term opioids

Nutritional Support (Critical Component)

1. Initial Approach

   • Regular monitoring of nutritional status and body mass index 1
   • Dietary modifications and oral supplements as first-line 1
   • Low-residue diet with vitamin supplementation 2

2. Enteral Nutrition

   • If oral intake is insufficient but patient is not vomiting, consider gastric feeding 1
   • If gastric feeding fails, trial jejunal feeding via nasojejunal tube 1
   • Consider jejunostomy for long-term feeding and medication administration 1

3. Parenteral Nutrition

   • Indicated when enteral feeding fails (often due to abdominal distension or pain) 1
   • Home central venous hyperalimentation has shown to improve quality of life in severe cases 3
   • Long-term parenteral nutrition is often necessary for severe small bowel involvement 1, 4

4. Venting Procedures

   • Venting gastrostomy may reduce vomiting but has potential complications 1

Surgical Interventions

• Avoid unnecessary surgery and optimize nutritional status before any surgical procedure 1
• Careful manometric and radiographic localization of affected segments may allow for judicious surgical resection or venting procedures in select cases 2
• Subtotal colectomy may be considered for colonic pseudo-obstruction 5

Special Considerations

• Elderly scleroderma patients with GI involvement have the poorest clinical outcomes 1
• Monitor for vitamin B12 deficiency, particularly important in small bowel involvement
• Assess for osteoporosis which is common in chronic intestinal disorders
• Consider psychological support as GI manifestations can significantly impact quality of life 6

Monitoring

• Regular weight measurements at each visit
• Nutritional assessments including micronutrient levels
• Monitoring for complications of parenteral nutrition (septicemia, superior vena cava obstruction) 3

Prognosis

The mortality rate in scleroderma patients with GI manifestations is significantly elevated, with malabsorption and malnutrition contributing to a reported 50% mortality rate 6. However, appropriate nutritional support can improve outcomes and quality of life 3.