Case Reports in Gastrointestinal Medicine / 2016 / Article / Tab 2

Case Report

Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases

Table 2

Demographic data for 39 Japanese cases of SSc with PCI.

CharacteristicsNumber of patients

Age (years), median (range)57 (24–83)
 Female36 (92.3%)
 Male3 (7.7%)
Types of scleroderma
 Diffuse11 (28.2%)
 Limited23 (59.0%)
 NA5 (12.8%)
Duration from onset of SSc to onset of PCI (years), median (range)6 (0–30)
Past corticosteroid therapy
 Present24 (61.5%)
Autoantibody positivity
 Antinuclear antibody33 (84.6%)
 Anticentromere antibody7 (18.0%)
 Anti-Scl-70 antibody3 (7.7%)
 Negative6 (15.4%)
Overlap syndrome
 Polymyositis/scleroderma8 (20.5%)
 Sjogren’s syndrome/scleroderma1 (2.6%)
Site of PCI
 Small intestine27 (69.2%)
 Small and large intestine2 (5.1%)
 Large intestine2 (5.1%)
 NA8 (20.5%)
Symptoms and signs
 Abdominal distention32 (82.1%)
 Abdominal pain13 (33.3%)
 Abdominal tenderness11 (28.2%)
 Nausea/vomiting9 (23.1%)
 Weight loss8 (20.5%)
 Anorexia8 (20.5%)
 Constipation5 (12.8%)
 Fatigue5 (12.8%)
 Diarrhea5 (12.8%)
 Peritoneal irritation sign2 (5.1%)
Diagnostic modalities
 Upper gastrointestinal series2 (5.1%)
 Plain X-ray film10 (25.6%)
 CT11 (28.2%)
 Plain X-ray film and CT11 (28.2%)
 Exploratory laparotomy5 (12.8%)
 Present34 (87.2%)
 Absent5 (12.8%)
 Antibiotics13 (33.3%)
 Oxygen therapy9 (23.1%)
 Octreotide2 (5.1%)
 Surgery2 (5.1%)
 Treatments other than the above-mentioned ones (symptomatic therapy combined with fluid therapy or elemental diet, etc.)15 (38.5%)

SSc: systemic sclerosis; PCI: pneumatosis cystoides intestinalis; NA: not available; CT: computed tomography.

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