Microscopic polyangiitis complicated with cerebral infarction and hemorrhage : a case report and review of literature

Abstract

We report a case of MPA with cerebral infarction and hemorrhage. A 72-year-old man was admitted to our hospital because of high fever, speech failure, and weakness of the left limbs in April 2008. Magnetic resonance imaging of the head showed cerebral infarction at the right corona radiata. Mononeuropathy multiplex and renal dysfunction (Cr 1.46 mg/dl) were noted. Urinalysis revealed occult blood, proteinuria and granular casts. White blood cell count was 11960/ul, CRP concentration was 12.9 mg/dl, and the MPO-ANCA titer was 330 EU. Computed tomography of the abdomen revealed arterial aneurysms in the kidney. The patient was diagnosed with microscopic polyangiitis (MPA). On the 8th day after hospital admission, a new cerebral hemorrhage occurred at the right thalamus. Prednisolone (1 mg/kg/day) and intravenous pulse cyclophosphamide (200 mg) were initiated. Because the patient's MPA was refractory, methylprednisolone pulse therapy (1000 mg/dayx3 days) was added to his treatment regimen, and he received plasma exchange (20 U/day) and double filtration plasmapheresis twice each ; after this, his MPA recovered. Cerebral infarction and hemorrhage are rare complications in MPA and are associated with poor prognosis. Published literature on these complications is reviewed.

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

“…However, they are often lethal 17 -half of the Japanese patients with brain lesions died, according to one review of the literature. 18 In the present study, vasculitis and thrombi were absent in the middle and anterior cerebral arteries and its branches. Mild atherosclerosis was found in the brain vessels, and there were no aneurysms.…”

“…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.…”

Development of intracerebral hemorrhage in the short-term clinical course of a patient with microscopic polyangiitis without neurological symptoms at diagnosis: an autopsy case

“…We suspect that there is no relationship between the presence of cerebral vasculitis and the severity of other organ symptom. This is because in our case and in other reported cases, the severity of MPA before developing cerebral hemorrhage varied [3][4][5][6][7][8][9][10][11].…”

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

“…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.…”