Table 1.
Prevalence, complications, and diagnostic management of GI manifestations in patients with SSc.
| Organ involvement | Prevalence | Gastrointestinal manifestations/complications and their prevalence | Diagnosis/Management |
|---|---|---|---|
| Oropharyngeal involvement | 10–70%[114] | Microstomia: 43%–80%[19,115] | 1. Regular dental exams.[98] |
| 2. Panoramic radiographic exams to assess for osseous changes.[98,114] | |||
| Xerostomia and periodontal disease: 30%–73%[19,115] | |||
| Gingival inflammation/bleeding: 60%–73%[19,41,98,115] | 3. Good oral hygiene and artificial saliva/lubricants to manage dental and oral/perioral soft tissue pathology.[98,114] | ||
| Oropharyngeal dysphagia: 25%[98,116] | |||
| 4. Mechanical soft foods, small bolus size, mouth stretching, and even bilateral commissurotomy are treatment options for decreased mouth opening.[114] | |||
| Esophageal involvement | 90%; 30–50% can be asymptomatic[20,47,88,101] | GERD 90%[14,117] | 1. EGD is used for diagnosing esophagitis, Barrett’s, and adenocarcinoma.[85] |
| Lower esophageal sphincter laxity 37.8–55%[14,41,117,118] | |||
| 2. Esophageal manometry is used to evaluate esophageal dysmotility.[85,114] | |||
| Esophagitis 60%[47,101] | |||
| Esophageal strictures 41%[47,101] | |||
| 2. Barium swallow can be used to detect strictures and their severity.[85] | |||
| Barrett’s esophagus 12.7–13%[88,101] | |||
| 3. pH monitoring to assess for therapeutic efficacy of PPI in GERD.[85,114] | |||
| 4. PPI is the mainstem of acid suppression treatment required in scleroderma patients to relieve GERD symptoms and prevent complications. | |||
| 5. Lifestyle modification is also recommended (avoiding large and late-night meals).[98,114] | |||
| Gastric involvement | 50%[20,88] | Gastroparesis 50%[20,88] | 1. Gastric emptying study.[85,98,114] |
| GAVE 5.6–22.3%[20,88,98] | |||
| 2. EGD is used to diagnose GAVE if the patient has iron deficiency anemia and for therapeutic purposes like laser photocoagulation or endoscopic band ligation.[85,98,114] | |||
| Gastric Bleeding Ectasis 0.6–0.8%[10,119] | |||
| Upper GI Bleeding 3.2%[10,120] | |||
| 3. Pro-kinetics (metoclopramide, domperidone) are used for gastroparesis management.[98] | |||
| Small bowel involvement | 40%; 20% can be asymptomatic[98,105] | Diarrhea: 27.7–79%[98,120] | 1. Scintigraphy, capsule endoscopy, MRI/CT enterography may be performed to evaluate small bowel involvement and extent.[98] |
| Small Intestinal Bacterial Overgrowth 33–50%[88] | |||
| Malabsorption 10–25%[88] | |||
| Small Bowel Pseudo-obstruction: 5.4%[116] | |||
| 2. Hydrogen-breath test to assess for SIBO.[79,85,98] | |||
| Pneumatosis Cystoides Intestinalis Rare[79,98] | |||
| 3. C. Diff testing and stool studies may be indicated in patients with diarrhea.[85,98] | |||
| 4. Abdominal X-ray and CT abdomen for pseudo-obstruction evaluation.[85] | |||
| 5. Measurement of fat-soluble vitamins if malabsorption is suspected.[85] | |||
| 6. Diet modification, probiotics, and antibiotics (fluoroquinolones, metronidazole, tetracycline, | |||
| rifaximin) are treatment options for SIBO.[98,114] | |||
| Colon involvement | 20–50%[20,88,98] | Constipation: 9.2–38%[98,120] | 1.Colonoscopy is recommended for scleroderma patients with new-onset constipation.[79,85,98] |
| Megacolon: 1.5–3.8%[120] | |||
| Large intestine vascular ectasia 1.3–3.1%[98,120] | |||
| 2. Fiber supplementation, bowel training, stool softeners, laxatives, and prokinetics are recommended to manage constipation.[79,98,114] | |||
| Lower GI bleeding 2.9%[10,120] | |||
| Wide-mouth diverticula 1.3–8.6%[16,98,120,121] | |||
| Anorectal involvement | 50–70%[47,79,98,101] | Rectal Prolapse 20%[121,122] | 1. Anorectal manometry, MR defecography, and balloon expulsion test are used to assess fecal incontinence.[79,85] |
| Fecal Incontinence 20–38%[20,88,98,120,123] | |||
| Fecal Impaction 18%[98,120] | |||
| 2. Anti-diarrheal agents and diet changes are used to improve stool consistency[98] | |||
| 3. Sacral nerve stimulation was shown to be beneficial in the management of fecal incontinence.[79,98,114] | |||
| 4. Surgical intervention may be indicated for rectal prolapse.[79,114] | |||
| Liver involvement | 1.1–1.5%[98] | Primary biliary cirrhosis (PBC) 2–18%[119,123] | 1. Checking liver enzymes, bilirubin, and antimitochondrial antibodies.[98,114] |
| Autoimmune hepatitis[85,98] | |||
| 2. If PBC is suspected but AMA negative, anti-gp210 and anti-sp100 are highly specific.[98] | |||
| 3. Liver ultrasound if the tests are abnormal.[114] | |||
| 4. Avoidance of hepatotoxic medications. | |||
| 5. Hepatic dosing of medications metabolized by the liver[85] | |||
| 6. Ursodeoxycholic acid for PBC.[85,98,114] |
CT = computerized tomography, EGD = esophagogastroduodenoscopy, GAVE = gastric antral vascular ectasia, GERD = gastroesophageal reflux disease, GI = gastrointestinal tract, MRI = magnetic resonance imaging, PBC = primary biliary cirrhosis, PPI = Proton pump inhibitors, SIBO = small intestinal bacterial overgrowth.