Renal vein thrombosis as the major cause of renal failure in familial Mediterranean fever.
Eighteen out of 57 patients (31-6 per cent) suffering from Familial Mediterranean Fever (FMF) were found to have the nephrotic syndrome, histologically proven amyloidosis and progressive renal failure. In 14 cases renal function deteriorated rapidly after the first appearance of significant proteinuria, and 12 cases (66-7 per cent) required regular haemodialysis. Seven of these patients, seen in the early stages of renal impairment, were subsequently diagnosed clinically as probably having developed renal vein thrombosis. There was radiological proof of intrarenal or major renal vein occlusion in five which in one patient progressed to inferior vena cave obstruction. Treatment with heparin, plasminogen activators and fibrinogenolytic agents was disappointing although renal function has stabilized in one patient on long term oral anticoagulant therapy. It is suggested that renal vein thrombosis is common in FMF with renal amyloidosis and usually causes rapid deterioration of function and irreversible renal failure requiring dialysis. Renal phlebography may delineate clot in the main renal veins or indicate areas of reduced blood flow due to thromboses in intrarenal venules. Treatment is only partially satisfactory but there is some evidence to suggest that renal phlebography should be undertaken promptly when renal function begins to fall followed by anticoagulant therapy to prevent further thromboembolic complications.
D058186:Acute Kidney Injury / Q000209:etiology*; D000293:Adolescent; D000328:Adult; D000686:Amyloidosis / Q000150:complications; D002648:Child; D002675:Child, Preschool; D010505:Familial Mediterranean Fever / Q000150:complications* / Q000000981:diagnostic imaging; D005260:Female; D006801:Humans; D008297:Male; D010690:Phlebography; D012082:Renal Veins; D013927:Thrombosis / Q000150:complications*