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

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

doi:10.2169/internalmedicine.46.0050

Cited by 16 publications

(7 citation statements)

References 15 publications

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“…However, hemoperitoneum for small-vessel vasculitis involving microscopic polyangiitis is rarely observed. There are only nine reports of hemoperitoneum with small vessel vasculitis (Table 1) [8][9][10][11][12][13][14][15][16]. Moreover, our case is the fourth case reporting hemoperitoneum with MPO-ANCA-associated microscopic polyangiitis.…”

Section: Discussionmentioning

confidence: 80%

“…Moreover, gastrointestinal ulcers and perforations due to cytomegalovirus infection occurs more frequently with corticosteroid therapy [27]. Cases of gastrointestinal cytomegalovirus infection have been reported in patients with Wegener's granulomatosis [16,28] and Henoch-Shonlein purpura [29]. It also must be considered in patients with gastrointestinal signs and symptoms.…”

Section: Discussionmentioning

confidence: 99%

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Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

et al. 2009

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Microscopic polyangiitis is a vasculitis which primarily affects capillaries, venules or arterioles. Involvement of small and medium-sized arteries may also occur. A 70-year-old Japanese female with a fever and cough was diagnosed with pneumonia and antibiotics were administered. Her symptoms initially improved, but her fever recurred and she experienced malaise and loss of appetite. Her renal function gradually worsened and she was positive for myeloperoxidase antineutrophil cytoplasmic antibodies (MPO-ANCA). She was referred to our hospital on the suspicion of ANCA-associated glomerulonephritis. However, her depressive mental symptoms did not allow her to undergo a renal biopsy. She was clinically diagnosed with ANCA-associated glomerulonephritis, and oral corticosteroids and intravenous methylprednisolone were administered. Her symptoms and renal function were improved, but she died suddenly 15 days after admission. An autopsy disclosed approximately 700 mL bloody ascites. Coagulation adhered to the lesser curvature of the stomach, but the source of hemorrhage could not be detected macroscopically because the gastric mucosa did not show abnormal findings. The histological findings revealed that the left gastric artery showed necrotizing angiitis and rupture. In the kidneys, cellular crescents were found in approximately 10%, fibrous crescents were found in approximately 10%, sclerosis and collapse were found approximately 30% of the glomeruli, and necrotizing angiitis was observed in interlobular arteries and arterioles. From these findings, she was finally diagnosed with microscopic polyangiitis. Microscopic polyangiitis is an extremely rare cause of spontaneous intraperitoneal bleeding, but it must be carefully considered in the differential diagnosis for the appropriate management of such patients.

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Section: Discussionmentioning

confidence: 80%

Section: Discussionmentioning

confidence: 99%

Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

et al. 2009

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“…CMV-related disease is a relatively frequent complication of immunosuppressive treatment for systemic vasculitis such as WG (16)(17)(18). In the present patient, gastrointestinal symptoms, positivity of CMV antigenemia assay on the 36th hospital day, detection of cells containing inclusion bodies in their nuclei in bronchial washings, and diffuse ground-glass opacities on chest CT were found, all of which suggest CMV infection.…”

Section: Discussionmentioning

confidence: 45%

Pulmonary Capillaritis in Wegener's Granulomatosis Detected Via Transbronchial Lung Biopsy

Ishiguro¹,

Takayanagi²,

Yamaguchi³

et al. 2012

Intern. Med.

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“…For some infections, the pathogenesis of microangiopathic hemolytic anemia has been described. Examples are bacterial endocarditis, [62] bacterial infections associated with microvascular injury such as brucellosis [32,33] streptococcal infections resulting in acute glomerulonephritis, [66] angioinvasive fungi such as aspergillosis, [83], and viruses and rickettsiae that cause endothelial injury such as CMV, [71][72][73] HIV, [16] erlichiosis, [44] and Rocky Mountain spotted fever [56]. However for other infections the mechanisms of microangiopathic hemolytic anemia are unclear.…”

Section: Discussionmentioning

confidence: 99%

Systemic infections mimicking thrombotic thrombocytopenic purpura

et al. 2011

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The absence of specific diagnostic criteria, the urgency to begin plasma exchange treatment, and the risk for complications from plasma exchange make the initial evaluation of patients with suspected thrombotic thrombocytopenic purpura (TTP) difficult. Systemic infections may mimic the presenting clinical features of TTP. In the Oklahoma TTP-HUS (hemolytic-uremic syndrome) Registry, 1989–2010, 415 consecutive patients have been clinically diagnosed with their first episode of TTP; in 31 (7%) the presenting clinical features were subsequently attributed to a systemic infection. All 31 patients had diagnostic criteria for TTP; 16 (52%) had the complete “pentad” of microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, renal failure and fever. Four (16%) of 25 patients who had ADAMTS13 measurements had <10% activity; three patients had a demonstrable ADAMTS13 inhibitor. Compared to 62 patients with severe ADAMTS13 deficiency (<10%) who had no recognized alternative disorders, patients with systemic infections had more frequent fever, coma, renal failure, and the complete “pentad” of clinical features. Seventeen different infectious etiologies were documented. A systematic literature review identified 67 additional patients with a diagnosis of TTP or HUS and also a systemic infection. Among all 98 patients, infections with 41 different bacteria, viruses, and fungi were documented, suggesting that many different systemic infections may mimic the presenting clinical features of TTP. Initial plasma exchange treatment is appropriate in critically ill patients with diagnostic features of TTP, even if a systemic infection is suspected. Continuing evaluation to document a systemic infection is essential to determine the appropriateness of continued plasma exchange.

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

Abstract: A 61-year-old woman was admitted to our hospital because of acute kidney injury. She

Cited by 16 publications

References 15 publications

Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

Fatal hemoperitoneum due to rupture of the left gastric artery in a patient with microscopic polyangiitis

Pulmonary Capillaritis in Wegener's Granulomatosis Detected Via Transbronchial Lung Biopsy

Systemic infections mimicking thrombotic thrombocytopenic purpura

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