Long-term immunosuppressive treatment of a child with Takayasu's arteritis and high IgE immunoglobulins

• Published in: Pediatric nephrology (Berlin, West) West), 1996-02, Vol.10 (1), p.67-69
• Main Authors: BRUNETTE, M. G, BONNY, Y, SPIGELBLATT, L, BARRETTE, G
• Format: Article
• Language: English
• Subjects: Anticoagulants - therapeutic use; Biological and medical sciences; Child; Humans; Immunoglobulin E - metabolism; Immunosuppressive Agents - therapeutic use; Male; Medical sciences; Regional Blood Flow - physiology; Renal Artery - pathology; Renal Circulation - physiology; Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis; Takayasu Arteritis - drug therapy; Takayasu Arteritis - immunology; Takayasu Arteritis - pathology; Testis - blood supply
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/BF00863450

A 7-year-old child presented with a severe form of Takayasu's arteritis, with two consecutive episodes involving the right testis and then the left kidney 6 months later. The renal artery obstruction was accompanied by severe hypertension. An aortography showed a complete obstruction of the left renal artery and a narrowing of the right subclavian artery. Plasma renin activity was high. Serum immunoglobulins were within the normal range, except for an increase in IgE (880 mu/l). Despite 4 months', treatment with antihypertensive drugs, prednisone, cyclophosphamide, and anticoagulant, the blood pressure never returned to normal and the left renal function remained completely absent. A nephrectomy was performed which immediately normalized plasma renin activity and blood pressure. The child was subsequently treated with alternate-day prednisone for 3 months, alternating with 3 months of cyclophosphamide or, later, azathioprine. Persantine (dipyridamole) and acetylsalicylic acid were administered continuously. The right radial pulse returned to normal within 2 years. An 8-year follow-up failed to detect any new episode of arteritis. The right kidney showed signs of compensatory hypertrophy. Finally, a recent arteriography demonstrated not only a normal right renal artery blood flow but almost total disappearance of the right subclavian artery obstruction. However, the IgE remained abnormally high (2,023 micrograms/l).