• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1040-1043
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• 2003

Henoch-Shonlein purpura(HSP) is a systemic small-vessel vasculitis that primarily affects the skin, gastrointestinal tract, joints, and kidneys. The nervous system may be involved, less commonly than other organs. When the central nervous system(CNS) was involved, headache, changes in mental status, seizures, and focal neurologic deficits have been reported. Hypertension, uremic encephalopathy, metabolic abnomalities, electrolyte abnormalities, or cerebral vasculitis were suggested as possible causes of the neurologic manifestation. Diagnosis of vasculitic involvement of CNS is difficult. Magnetic resonance imaging of the brain is the modality of choice for the evaluation of the CNS disease. Steroid or plasmapheresis are used in treatment of cerebral vasculitis. We experienced a case of 9-year-old boy who had presented with Henoch-Schonlein purpura nephritis complicating encephalopathy accompanied by hypertension and cerebral vasculitis. Brain MRI showed multiple small nodular-linear pattern enhancing lesions in whole cerebral hemispheres and focal increased T2 signal in the right basal ganglia. We used intravenous immunoglobulin in treatment of cerebral vasculitis. We report this case with a brief review of related literature.

• Tuberculosis and Respiratory Diseases
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• v.39 no.1
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• pp.89-94
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• 1992

A 16 years old girl with systemic lupus erythematosus had a high fever for 20 days. Skin and renal biopsy showed diffuse granular deposits (IgG, IgM, $C_3$, $C_{1q}$ at dermo-epideral junction and IgG, IgA, IgM, $C_3$, $C_{1q}$, fibrinogen in the renal mesangium and segmentally along the capillary walls) which were compatable with systemic lupus erythematosus. The chest X-ray revealed patchy mottled densities in whole lung field when she complained more dyspnea at 9th hospital days. Even with the parenteral administration of broad-spectrum antibiotics, the symptoms of high fever, cough, tachydyspnea and hypoxia were continued. At 24th hospital day, the clinical course was rapidly deteriorated after sudden loss of consciousness with focal seizure which suggested CNS involvement during hydrocortisone administration for 10 days. She died of respiratory failure despite the mechanical ventilatory support with PEEP. The limited necropsy showed interstitial pneumonia, alveolar hemorrhage and occlusive necrotizing vasculitis of acute lupus pneumonitis.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.73-76
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• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Tuberculosis and Respiratory Diseases
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• v.55 no.6
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• pp.623-630
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• 2003

Microscopic polyangiitis is a systemic small-vessel vasculitis that is primarily associated with necrotizing glomerulonephritis and pulmonary capillaritis. Lung involvement is characterized by a diffuse alveolar hemorrhage. However, rarely central nervous system involvement has been reported to be occurred with the microscopic polyangiitis. Relapse of microscopic polyangiitis are reported to be more frequent than those of polyarteritis nodosa, often after a reduction or discontinuation of the therapy. We would like to report two patients with microscopic polyangiitis. One presented with clinical manifestations of both lung and central nervous system involvements and the other was a case of recurrence during steroid tapering following the steroid pulse therapy.