Renal manifestations in toddlers with Takayasu’s arteritis and malignant hypertension

• Published in: Pediatric nephrology (Berlin, West) Vol. 24; no. 6; pp. 1227 - 1230
• Main Authors: Hijazi, Rana, Chandar, Jayanthi, Nwobi, Obioma, Muneeruddin, Samina, Zilleruelo, Gastón, Abitbol, Carolyn L.
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.06.2009; Springer; Springer Nature B.V
• Subjects: Angiotensin Receptor Antagonists; Angiotensin-Converting Enzyme Inhibitors - therapeutic use; Antihypertensive Agents - therapeutic use; Biopsy; Brief Report; Care and treatment; Child, Preschool; Diagnosis; Female; Follow-Up Studies; Glomerulosclerosis, Focal Segmental - diagnosis; Glomerulosclerosis, Focal Segmental - drug therapy; Humans; Hypertension, Malignant - drug therapy; Hypertension, Malignant - surgery; Immunosuppressive Agents - therapeutic use; Infant; Kidney - physiopathology; Kidney - surgery; Male; Medicine & Public Health; Mycophenolic Acid - analogs & derivatives; Mycophenolic Acid - therapeutic use; Nephrology; Patient outcomes; Pediatrics; Renal Artery Obstruction - drug therapy; Renal Artery Obstruction - surgery; Takayasu Arteritis - diagnosis; Takayasu Arteritis - drug therapy; Takayasu Arteritis - surgery; Takayasu's arteritis; Time Factors; Treatment Outcome; Urology; Vasculitis; Renovascular hypertension; Children
• ISSN: 0931-041X; 1432-198X
• DOI: 10.1007/s00467-008-1088-3

Three children under the age of 3 years presented with malignant hypertension, proteinuria, and acute kidney injury. Takayasu’s arteritis was diagnosed on the basis of clinical symptoms of weight loss and low grade fever in conjunction with elevated sedimentation rate and radiographic evidence of aortic and renal artery stenosis. One patient had a renal biopsy which showed arteriolar sclerosis and focal glomerulosclerosis. All three patients required multiple antihypertensive agents, ultimately including angiotensin receptor blockers and/or angiotensin converting enzyme inhibitors. The vasculitis was treated with pulse corticosteroids followed by cyclophosphamide in one patient and mycophenolate mofetil as maintenance therapy in all. Follow-up has ranged from 2 to 8 years. Although global renal function has normalized in each patient, two have unilateral non-function of one kidney. The last patient has persistent aortic and renal artery stenosis with complex collateralization requiring ongoing medical and anticipated surgical management.