title: Pneumatosis Cystoides Intestinalis in Patients with Systemic Sclerosis: A Case Report and Review of 39 Japanese Cases.
abstract: Pneumatosis cystoides intestinalis (PCI) is a rare gastrointestinal complication of systemic sclerosis (SSc) characterized by intramural accumulation of gas within thin-walled cysts. We report the case of an 82-year-old female patient with pneumoperitoneum due to PCI associated with SSc and review the features of the 39 Japanese cases. The median patient age was 57 years (range 24-83 years) and the male/female ratio was 1 : 12. In the recent decade, 14 out of 15 cases (93.3%) evaluated with CT scans were diagnosed with PCI. The results suggest that CT scan may be a useful diagnostic tool for detecting PCI. PCI in patients with SSc is usually benign and requires only conservative therapy. However, two patients (5.1%) with signs of peritoneal irritation required surgery. When peritoneal irritation secondary to additional pathology is observed, surgical treatment may be warranted; a precise diagnosis for this condition is therefore essential.
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Manabu
authors.identifier: 0000-0003-3122-1124
authors.initials: M
authors.lastname: Kaneko
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Shin
authors.identifier: 0000-0003-3489-6821
authors.initials: S
authors.lastname: Sasaki
authors.affiliation: Department of Internal Medicine, Division of Rheumatology, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Shuzo
authors.initials: S
authors.lastname: Teruya
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Kosuke
authors.initials: K
authors.lastname: Ozaki
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Ishimaru
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Emi
authors.initials: E
authors.lastname: Terai
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nakayama
authors.affiliation: Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Watanabe