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Case Rep Gastrointest Med. 2016;2016:2474515. doi: 10.1155/2016/2474515. Epub 2016 Aug 29.

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

Case reports in gastrointestinal medicine

Manabu Kaneko, Shin Sasaki, Shuzo Teruya, Kosuke Ozaki, Kazuhiro Ishimaru, Emi Terai, Hiroshi Nakayama, Toshiyuki Watanabe

Affiliations

  1. Department of Surgery, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.
  2. Department of Internal Medicine, Division of Rheumatology, Omori Red Cross Hospital, 4-30-1 Chuo, Ota-ku, Tokyo 143-8527, Japan.

PMID: 27651961 PMCID: PMC5019915 DOI: 10.1155/2016/2474515

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.

References

  1. AJR Am J Roentgenol. 2007 Jun;188(6):1604-13 - PubMed
  2. Arthritis Rheum. 2013 Nov;65(11):2737-47 - PubMed
  3. Eur J Med Res. 2009 Jun 18;14 (6):231-9 - PubMed
  4. Mod Rheumatol. 2012 Aug;22(4):610-5 - PubMed
  5. Gastroenterology. 1995 May;108(5):1560-5 - PubMed
  6. Lancet. 1995 May 13;345(8959):1220-2 - PubMed
  7. Rheum Dis Clin North Am. 2015 Aug;41(3):459-73 - PubMed
  8. J Clin Rheumatol. 2010 Dec;16(8):379-81 - PubMed
  9. Am J Clin Pathol. 1967 Nov;48(5):455-65 - PubMed
  10. Hum Pathol. 1985 Jul;16(7):683-8 - PubMed
  11. Am J Gastroenterol. 1997 May;92(5):763-71 - PubMed
  12. Arch Pathol Lab Med. 1989 Aug;113(8):898-901 - PubMed
  13. Arch Surg. 1974 Jul;109(1):89-94 - PubMed
  14. Intern Med. 1993 Jul;32(7):607-9 - PubMed
  15. Gut. 1984 Aug;25(8):839-45 - PubMed
  16. Am J Med Sci. 2006 Aug;332(2):100-2 - PubMed
  17. Transplant Proc. 1984 Dec;16(6):1666-8 - PubMed
  18. Semin Arthritis Rheum. 1990 Apr;19(5):269-77 - PubMed
  19. Arch Surg. 2003 Jan;138(1):68-75 - PubMed
  20. Intern Med J. 2012 Mar;42(3):323-9 - PubMed
  21. J Clin Gastroenterol. 2008 Jan;42(1):5-12 - PubMed
  22. Am J Surg. 1973 Jun;125(6):667-71 - PubMed

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