Wegener's Granulomatosis Complicated by Intestinal Ulcer due to Cytomegalovirus Infection and by Thrombotic Thrombocytopenic Purpura

Mayuko Yamazaki; Takashi Takei; Shigeru Otsubo; Yuko Iwasa; Yasuko Yabuki; Keiko Suzuki; Minako Koike; Keiko Uchida; Ken Tsuchiya; Wako Yumura; Shigeru Horita; Kazuho Honda; Takashi Akiba; Kosaku Nitta

doi:10.2169/internalmedicine.46.0050

CASE REPORTS

Wegener's Granulomatosis Complicated by Intestinal Ulcer due to Cytomegalovirus Infection and by Thrombotic Thrombocytopenic Purpura

Mayuko Yamazaki, Takashi Takei, Shigeru Otsubo, Yuko Iwasa, Yasuko Yabuki, Keiko Suzuki, Minako Koike, Keiko Uchida, Ken Tsuchiya, Wako Yumura, Shigeru Horita, Kazuho Honda, Takashi Akiba, Kosaku Nitta

Author information

Mayuko Yamazaki
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Takashi Takei
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Shigeru Otsubo
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Yuko Iwasa
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Yasuko Yabuki
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Keiko Suzuki
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Minako Koike
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Keiko Uchida
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Ken Tsuchiya
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Wako Yumura
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Shigeru Horita
Department of Medicine, Kidney Center, Tokyo Women's Medical University
Kazuho Honda
Department of Pathology, Tokyo Women's Medical University
Takashi Akiba
Department of Blood Purification, Kidney Center, Tokyo Women's Medical University
Kosaku Nitta
Department of Medicine, Kidney Center, Tokyo Women's Medical University

Keywords: Wegener's granulomatosis, intestinal ulcer, cytomegalovrius infection, thrombotic thrombocytopenic purpura, progressive glomerulonephritis, plasma exchange

JOURNAL OPEN ACCESS

2007 Volume 46 Issue 17 Pages 1435-1440

DOI https://doi.org/10.2169/internalmedicine.46.0050

Details

Abstract

A 61-year-old woman was admitted to our hospital because of acute kidney injury. She complained of general fatigue, appetite loss, and a high fever. Nodular lesions were observed on chest X-rays and there were >100 erythrocytes per high power field in her urinary sediment. A renal biopsy revealed necrotizing granulomatous glomerulonephritis, and her serum proteinase 3-antineutrophil cytoplasmic anitibody (PR3-ANCA) titer was elevated (55 EU). Based on these findings we made a diagnosis of Wegener's granulomatosis (WG). Hemodialysis was started immediately after admission. Steroid therapy was administered and her symptoms were relieved, but her renal function did not improve. On the 50th hospital day her condition suddenly became complicated by hemoperitoneum and massive intestinal bleeding, and the descending, transverse, ascending colon and part of the ileum were surgically resected. The cytomegalovirus (CMV) antigen titer was elevated, and histologic examination of the bowel specimen showed positive staining for CMV in the ulcer lesion, suggesting that CMV infection had caused the bowel hemorrhage. After treatment with ganciclovir, the bleeding was resolved and the CMV antigens became negative. We considered that this patient was further complicated by thrombotic thrombocytopenic purpura (TTP) because of thrombocytopenia, hemolytic anemia and neurologic symptoms. She was treated by plasma exchange. We report here a case of WG complicated by acute intestinal ulcer due to CMV infection and by TTP.

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