Microscopic Polyangiitis Associated with Marked Systemic Bleeding Tendency Caused by Disseminated Intravascular Coagulation

Saito, Takatoshi; Tsuchiya, Masahito; Shikata, Chihiro; Yamaguchi, Hiroshi; Miyata, Shuichi; Matsuo, Seiichiro; Ishizawa, Shin; Yoshimura, Kazuya

doi:10.2169/internalmedicine.42.850

Cited by 9 publications

(7 citation statements)

References 20 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Cerebral infarction and hemorrhage are rare complications of MPA. However, they are often lethal – half of the Japanese patients with brain lesions died, according to one review of the literature . In the present study, vasculitis and thrombi were absent in the middle and anterior cerebral arteries and its branches.…”

Section: Discussionsupporting

confidence: 52%

Presence of Citrullinated Histone H3‐Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case

Matsuda

Hamayasu

Seki

et al. 2016

Pathology International

View full text Add to dashboard Cite

A 76-year-old man was admitted with general fatigue, weight loss, fever, headache, renal failure, and a high serum level of myeloperoxidase-antineutrophil cytoplasmic antibody. Biopsy revealed citrullinated histone H3 (citH3)-positive neutrophils adherent to the temporal artery endothelium. Three days after completing pulse steroid therapy, he suffered from a sudden disturbance of consciousness and died. On autopsy, the kidneys showed the most severe vasculitis with dense infiltration of citH3-positive neutrophils. The lungs showed intra-alveolar hemorrhage due to capillaritis. Severe brain hemorrhage was found in the left frontal lobe and putamen with uncal herniation. No vasculitis or thrombi was observed in the brain. The right dura mater was thickened due to fibrosis and inflammation. In conclusion, autopsy revealed systemic vasculitis with infiltration of abundant citH3-positive neutrophils, suggesting that the neutrophil extracellular trap formation and citH3 might play important roles in the early phases and development of microscopic polyangiitis.

show abstract

Section: Discussionsupporting

confidence: 52%

Presence of Citrullinated Histone H3‐Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case

Matsuda

Hamayasu

Seki

et al. 2016

Pathology International

View full text Add to dashboard Cite

show abstract

“…7,8 The literature reported two cases of ANCA-positive MPA associated with DIC and treated with gabexate with improvement of hematological disorder and renal function. 9,10 We assume that the improvement of our patient's condition was due not only to the beneficial effect on coagulation disorders but inhibition of inflammatory pathways too.…”

Section: Discussionmentioning

confidence: 95%

“…9 Therefore, it may not be easy to detect the presence of latent DIC. In our patient, however, the immunosuppressive therapy for the treatment of MPA could not be used and we could diagnose DIC without any pharmacological interference.…”

Section: Discussionmentioning

confidence: 99%

Gabexate Mesylate as Treatment in the Course of ANCA-Negative Microscopic Polyangiitis

et al. 2013

View full text Add to dashboard Cite

Patients with small vessel vasculitis present fluctuating antineutrophil cytoplasmic antibodies (ANCA) levels to the point that positive ANCA may be missed even if only up to 10% of patients with microscopic polyangiitis (MPA) are ANCA-negative. The first-line treatment of MPA is the association of steroids and cyclophosphamide, especially in the presence of a rapidly progressive glomerulonephritis. Plasmapheresis, intravenous immunoglobulins, and tumor necrosis factor inhibitors have been proposed as alternative to standard therapy. Disseminated intravascular coagulation (DIC) is a possible event in the course of small vessel vasculitis. Gabexate mesylate is a protease inhibitor able to suppress endothelial cell injury, and it may be administered to treat DIC related to different diseases. In ANCA-associated vasculitis, cytokines play a key role in promoting endothelial damage. DIC-related thrombocytopenia may be misinterpreted as drug-induced because of the immunosuppressive properties of cyclophosphamide. Two cases of ANCA-positive MPA associated with DIC and treated with gabexate are reported in the literature with improvement of both hematological disorder and renal function. Our patient presented a rapidly progressive glomerulonephritis, and the renal biopsy showed MPA, in the absence of ANCA. After two weeks of steroid treatment, our patient developed a DIC. This case represents the first report of ANCA-negative MPA managed with gabexate, which showed improvement of coagulation disorders and kidney function. In conclusion, the anti-inflammatory properties of gabexate could be helpful in MPA at increased bleeding risk when immunosuppressive treatment is contraindicated, even in ANCA-negative vasculitis.

show abstract

“…Although respiratory and renal involvements are common in MPO-ANCA-associated MPA, cerebrovascular involvement is rare, accounting for up to 4 % [1][2][3]. To our knowledge, 12 cases of brain involvement in patients with MPO-ANCA-associated vasculitis have been reported in the English literature or Japanese literature with English abstract (Table 1) [8][9][10][11][12][13][14][15][16][17][18][19]. Cases with pachymeningitis in this entity have been excluded.…”

Section: Discussionmentioning

confidence: 99%

AP-VAS 2012 case report: a case of systemic MPO-ANCA-associated vasculitis that demonstrated brain infarction and immunohistochemically MPO-positive capillaries

et al. 2013

View full text Add to dashboard Cite

We present a case of an aged male with myeloperoxidase anti-neutrophil cytoplasmic autoantibody (MPO-ANCA)-associated vasculitis with onset of brain infarction that demonstrated immunohistochemically MPO-positive capillaries at autopsy. The patient initially presented with gait difficulty and right-sided weakness. Since an imaging study revealed brain infarction, he was admitted to our hospital and medicated by antiplatelet agents. Continuous fever and elevated serum C-reactive protein (CRP), hematuria of glomerular origin, renal dysfunction, and high serum titer of MPO-ANCA were detected. Systemic toxicoderma appeared, and skin biopsy revealed small-vessel vasculitis; thus, he was diagnosed with MPO-ANCA-associated vasculitis. Steroid therapy (methylprednisolone 30 mg/day) was started, and general status improved. However, he died of shock 6 days after the start of the therapy. Autopsy revealed massive retroperitoneal hemorrhage with necrotizing small-vessel vasculitis in systemic organs including retroperitoneum, skin, brain, testes, and kidneys. Immunohistochemically, infiltration of MPO-positive white blood cells into the capillaries was occasionally observed, along with the features of MPO-positive capillaries. Cerebrovascular involvement of MPO-ANCA-associated vasculitis is rare compared with renal and pulmonary manifestations, having been reported to occur in up to 4 % of patients. Furthermore, as we have recently reported, MPO-immunopositive capillaries may appear only during the hyperacute stage of the disease. Therefore, the present case represents the unique combination of these two rare manifestations.

show abstract

Microscopic Polyangiitis Associated with Marked Systemic Bleeding Tendency Caused by Disseminated Intravascular Coagulation

Abstract: A 57-year-old womanwas admitted to our hospital be-

Cited by 9 publications

References 20 publications

Presence of Citrullinated Histone H3‐Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case

Presence of Citrullinated Histone H3‐Positive Neutrophils in Microscopic Polyangiitis from the Early Phase: An Autopsy Proven Case

Gabexate Mesylate as Treatment in the Course of ANCA-Negative Microscopic Polyangiitis

AP-VAS 2012 case report: a case of systemic MPO-ANCA-associated vasculitis that demonstrated brain infarction and immunohistochemically MPO-positive capillaries

Contact Info

Product

Resources

About