Churg-Strauss-Syndrom mit zerebralen Krampfanfällen und terminaler Niereninsuffizienz
Autor: | Tilman Sauerbruch, M. Schepke, H. U. Klehr, W. D. Paar, B. Terjung, A. Hufnagel |
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Rok vydání: | 2008 |
Předmět: |
Creatinine
medicine.medical_specialty Kidney medicine.diagnostic_test medicine.drug_class business.industry General Medicine medicine.disease Gastroenterology chemistry.chemical_compound medicine.anatomical_structure chemistry Internal medicine Erythrocyte sedimentation rate medicine Prednisolone Corticosteroid Eosinophilia Renal biopsy medicine.symptom business Cerebral vasculitis medicine.drug |
Zdroj: | DMW - Deutsche Medizinische Wochenschrift. 122:853-858 |
ISSN: | 1439-4413 0012-0472 |
DOI: | 10.1055/s-2008-1047700 |
Popis: | HISTORY AND CLINICAL FINDINGS A 67-year-old man with known bronchial asthma was admitted to hospital because of deteriorating general state of health, fever, progressive renal failure and confusional states. INVESTIGATIONS Erythrocyte sedimentation rate was 70/95 mm and the concentration of C-reactive protein raised to 30 mg/dl. WBC count was 19,000/microliter with 39% eosinophilia. Anticytoplasmatic antibodies (cANCA) had a high titre (1:160). On admission the creatinine level was 5.6 mg/dl. Renal biopsy indicated marked glomerular and tubulo-interstitial scarring. Chest radiograms showed transient pulmonary infiltrates. Churg-Strauss syndrome (CSS) was diagnosed on the basis of the clinical and biochemical findings. TREATMENT AND COURSE Haemodialysis was instituted to counteract the renal failure with water retention. Inflammatory parameters and clinical symptoms rapidly responded to administration of corticosteroids (prednisolone, initially 250 mg/d for 3 days, then 150 mg/d for 5 days followed by slowly decreasing doses). Two weeks after starting prednisolone he had secondary generalised seizures. Magnetic resonance imaging (MRI) of the skull demonstrated marked hyperintense focal changes which in their pattern were characteristic of cerebral vasculitis. As a steroid-refractory condition had to be assumed, cyclophosphamide was also given (100 mg/d). Within 6 weeks the clinical symptoms gradually regressed and the MRI changes became practically normal. CONCLUSION Early combined immunotherapy should be given if CSS runs a complicated course, rather than the usually recommended corticosteroid monotherapy. |
Databáze: | OpenAIRE |
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