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.

Characteristics	Number of patients
Age (years), median (range)	57 (24–83)
Sex
Female	36 (92.3%)
Male	3 (7.7%)
Types of scleroderma
Diffuse	11 (28.2%)
Limited	23 (59.0%)
NA	5 (12.8%)
Duration from onset of SSc to onset of PCI (years), median (range)	6 (0–30)
Past corticosteroid therapy
Present	24 (61.5%)
Autoantibody positivity
Antinuclear antibody	33 (84.6%)
Anticentromere antibody	7 (18.0%)
Anti-Scl-70 antibody	3 (7.7%)
Negative	6 (15.4%)
Overlap syndrome
Polymyositis/scleroderma	8 (20.5%)
Sjogren’s syndrome/scleroderma	1 (2.6%)
Site of PCI
Small intestine	27 (69.2%)
Small and large intestine	2 (5.1%)
Large intestine	2 (5.1%)
NA	8 (20.5%)
Symptoms and signs
Abdominal distention	32 (82.1%)
Abdominal pain	13 (33.3%)
Abdominal tenderness	11 (28.2%)
Nausea/vomiting	9 (23.1%)
Weight loss	8 (20.5%)
Anorexia	8 (20.5%)
Constipation	5 (12.8%)
Fatigue	5 (12.8%)
Diarrhea	5 (12.8%)
Peritoneal irritation sign	2 (5.1%)
Diagnostic modalities
Upper gastrointestinal series	2 (5.1%)
Plain X-ray film	10 (25.6%)
CT	11 (28.2%)
Plain X-ray film and CT	11 (28.2%)
Exploratory laparotomy	5 (12.8%)
Pneumoperitoneum
Present	34 (87.2%)
Absent	5 (12.8%)
Treatment
Antibiotics	13 (33.3%)
Oxygen therapy	9 (23.1%)
Octreotide	2 (5.1%)
Surgery	2 (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.