An unusual manifestation of Wegener’s granulomatosis in a 4-year-old girl

• Published in: Pediatric neurology Vol. 27; no. 1; pp. 71 - 74
• Main Authors: Haas, Johannes-Peter, Metzler, Markus, Ruder, Hans, Waldherr, R.üdiger, Böswald, Michael, Rupprecht, Thomas
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.07.2002; Elsevier
• Subjects: Biological and medical sciences; Child, Preschool; Female; Granulomatosis with Polyangiitis - complications; Granulomatosis with Polyangiitis - diagnostic imaging; Humans; Intracranial Hemorrhages - etiology; Intracranial Hemorrhages - pathology; Magnetic Resonance Imaging; Medical sciences; Radiography; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Human; Intracranial; Nervous system diseases; Prognosis; Cardiovascular disease; Wegener granulomatosis; Hemorrhage; Cerebral disorder; Vascular disease; Case study; Vasculitis; Systemic disease; Central nervous system disease; Female; Diagnosis; Child; Inaugural sign; Cerebrovascular disease
• ISSN: 0887-8994; 1873-5150
• DOI: 10.1016/S0887-8994(02)00392-2

We report a female who was diagnosed with Wegener’s granulomatosis at 4 years of age with life-threatening intracranial bleeding. The patient’s serum was positive for c-antineutrophilic cytoplasmic antibodies, and histologic analysis of the lung biopsy revealed evidence of granulomatous vasculitis. Initial treatment with steroids and cyclophosphamide was successfully converted to a long-term medication regimen consisting of azathioprine, trimethoprim, and sulfamethoxazole. Thereafter the patient showed no signs of disease activity for more than 3 years and manifested only a low-grade neurologic handicap. In February 2001, 5 years after the initial diagnosis, she presented with altered consciousness and myoclonic multifocal seizures. Subsequent diagnostic studies confirmed the diagnosis of disseminated cerebral vasculitis unresponsive to steroid treatment. Acute neurologic symptoms relented immediately after cyclophosphamide pulse therapy. Magnetic resonance imaging of the brain demonstrated complete remission within 8 weeks. Her current treatment includes steroids and monthly pulses of cyclophosphamide.