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Scleroderma renal crisis with coexisting segmental arterial mediolysis presenting as intraperitoneal bleeding: a case report.
J Med Case Rep. 2019 Mar 20; 13(1):74.JM

Abstract

BACKGROUND

Segmental arterial mediolysis is a rare nonarteriosclerotic and noninflammatory vascular disease that may cause intraperitoneal bleeding. Scleroderma renal crisis is a rare complication of systemic sclerosis, leading to severe hypertension and renal dysfunction. To the best of our knowledge, this is the first reported case of a patient with concurrent systemic sclerosis with scleroderma renal crisis and pathologically confirmed segmental arterial mediolysis.

CASE PRESENTATION

We report a case of a 68-year-old Chinese woman diagnosed with systemic sclerosis who was found to have coexisting segmental arterial mediolysis. She presented with back pain, and massive intraperitoneal bleeding was detected by computed tomography. She underwent laparotomy, and the bleeding was found to originate from the gastroepiploic artery. The pathological examination demonstrated gastroepiploic arterial dissection caused by segmental arterial mediolysis. After surgery, she developed severe hypertension with hyperreninemia and progressive renal dysfunction. Given the risk factors of corticosteroid administration and the presence of anti-ribonucleic acid polymerase III antibody, she was diagnosed with scleroderma renal crisis. The patient was proved to have a very rare case of coexisting scleroderma renal crisis and segmental arterial mediolysis.

CONCLUSIONS

There is no known etiological connection between segmental arterial mediolysis and systemic sclerosis or scleroderma renal crisis, but it is possible that coexisting segmental arterial mediolysis and scleroderma renal crisis may have interacted to trigger the development of the other in our patient.

Authors+Show Affiliations

Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan. Department of Nephrology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan. eriw@jichi.ac.jp.Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Surgery, Sainokuni Higashiomiya Medical Center, 1522 Toro-cho, Kita-ku, Saitama, 331-8577, Japan.Department of Rheumatology, Sainokuni Higashiomiya Medical Center, 1522 Toro-cho, Kita-ku, Saitama, 331-8577, Japan.Department of Nephrology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Nephrology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.Department of Rheumatology, Saitama Medical Center, Jichi Medical University, 1-847 Amanuma-cho, Omiya-ku, Saitama, 330-8503, Japan.

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

30890184

Citation

Kaneko, Shohei, et al. "Scleroderma Renal Crisis With Coexisting Segmental Arterial Mediolysis Presenting as Intraperitoneal Bleeding: a Case Report." Journal of Medical Case Reports, vol. 13, no. 1, 2019, p. 74.
Kaneko S, Watanabe E, Abe M, et al. Scleroderma renal crisis with coexisting segmental arterial mediolysis presenting as intraperitoneal bleeding: a case report. J Med Case Rep. 2019;13(1):74.
Kaneko, S., Watanabe, E., Abe, M., Watanabe, S., Yabe, H., Kojima, S., Takagi, K., Hirai, K., Morishita, Y., & Terai, C. (2019). Scleroderma renal crisis with coexisting segmental arterial mediolysis presenting as intraperitoneal bleeding: a case report. Journal of Medical Case Reports, 13(1), 74. https://doi.org/10.1186/s13256-019-1993-z
Kaneko S, et al. Scleroderma Renal Crisis With Coexisting Segmental Arterial Mediolysis Presenting as Intraperitoneal Bleeding: a Case Report. J Med Case Rep. 2019 Mar 20;13(1):74. PubMed PMID: 30890184.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Scleroderma renal crisis with coexisting segmental arterial mediolysis presenting as intraperitoneal bleeding: a case report. AU - Kaneko,Shohei, AU - Watanabe,Eri, AU - Abe,Mai, AU - Watanabe,Shinji, AU - Yabe,Hiroki, AU - Kojima,Shigehiro, AU - Takagi,Kenji, AU - Hirai,Keiji, AU - Morishita,Yoshiyuki, AU - Terai,Chihiro, Y1 - 2019/03/20/ PY - 2018/09/30/received PY - 2019/01/23/accepted PY - 2019/3/21/entrez PY - 2019/3/21/pubmed PY - 2019/7/23/medline KW - Gastroepiploic artery aneurysm KW - Hypertension KW - Prednisolone KW - Renal dysfunction KW - Systemic sclerosis KW - Vascular disease SP - 74 EP - 74 JF - Journal of medical case reports JO - J Med Case Rep VL - 13 IS - 1 N2 - BACKGROUND: Segmental arterial mediolysis is a rare nonarteriosclerotic and noninflammatory vascular disease that may cause intraperitoneal bleeding. Scleroderma renal crisis is a rare complication of systemic sclerosis, leading to severe hypertension and renal dysfunction. To the best of our knowledge, this is the first reported case of a patient with concurrent systemic sclerosis with scleroderma renal crisis and pathologically confirmed segmental arterial mediolysis. CASE PRESENTATION: We report a case of a 68-year-old Chinese woman diagnosed with systemic sclerosis who was found to have coexisting segmental arterial mediolysis. She presented with back pain, and massive intraperitoneal bleeding was detected by computed tomography. She underwent laparotomy, and the bleeding was found to originate from the gastroepiploic artery. The pathological examination demonstrated gastroepiploic arterial dissection caused by segmental arterial mediolysis. After surgery, she developed severe hypertension with hyperreninemia and progressive renal dysfunction. Given the risk factors of corticosteroid administration and the presence of anti-ribonucleic acid polymerase III antibody, she was diagnosed with scleroderma renal crisis. The patient was proved to have a very rare case of coexisting scleroderma renal crisis and segmental arterial mediolysis. CONCLUSIONS: There is no known etiological connection between segmental arterial mediolysis and systemic sclerosis or scleroderma renal crisis, but it is possible that coexisting segmental arterial mediolysis and scleroderma renal crisis may have interacted to trigger the development of the other in our patient. SN - 1752-1947 UR - https://www.unboundmedicine.com/medline/citation/30890184/Scleroderma_renal_crisis_with_coexisting_segmental_arterial_mediolysis_presenting_as_intraperitoneal_bleeding:_a_case_report_ L2 - https://jmedicalcasereports.biomedcentral.com/articles/10.1186/s13256-019-1993-z DB - PRIME DP - Unbound Medicine ER -