CASE HISTORY – January 2006

Primary systemic vasculitis – Wegener`s granulomatosis: a case report and short overview


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A thirty-one-year-old male patient had been diagnosed with chronic sinusitis in spring 2004. Antibiotic treatment had been ineffective and surgical treatment was suggested. Histological investigation revealed necrotizing sinusitis with fibrosis. Despite antibiotic treatment, the patient had headache and dizziness, and two weeks later periorbital oedema, nausea and vomiting appeared. Subdural empyema was suspected and the patient was referred to the clinic of neurology. Repeated MRI and CT investigations led to the diagnoses of meningitis and brain oedema. Antibacterial treatment was combined with glucocorticoids: the patient recovered and the treatment was stopped. In February 2005, a nephrologist consulted the patient for persistent periorbital oedema. Renal disease was not diagnosed. Due to chronic sinusitis, the patient  underwent secondary surgical treatment. On the basis of repeated histological investigations, Wegener`s granulomatosis was suspected. The diagnosis was confirmed at the department of rheumatology by using additional investigations (skin biopsy, CT of the lungs). With aggressive glucocorticoid and cyclophosphamid treatment the patient recovered. The treatment is continued.
Wegener`s granulomatosis is a vasculitis that usually begins as a localized granulomatous inflammation of the upper and/or lower respiratory tract mucosa.
The disease can progress to generalized vasculitis. The etiology of the disease is unknown. The brain is rarely affected. Without treatment mortality is high. This case illustrates difficulties with diagnosing of an uncommon systemic vasculitis.