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.