ADULT HEMOLYTIC UREMIC SYNDROME WITH RENAL MICROANGIOPATHY - OUTCOME ACCORDING TO THERAPEUTIC PROTOCOL IN 53 CASES

We gathered data on adult patients suffering from hemolytic uremic syndrome with renal biopsy-proven thrombotic microangiopathy (n = 85) and excluded those associated with cancer, lymphoma, progressive systemic sclerosis and AIDS (n = 32). The 53 patients selected had a follow-up of more than 6 months (m = 38 months ; range : 6-180 months). The aim of the study was to analyze the therapy-dependent outcome in these 53 patients. In such a retrospective and multicentric survey, different therapeutic protocols were used, including plasma exchange (PE), infusions of fresh-frozen plasma, corticosteroids, IV immunoglobulins or supportive therapy alone ; all were used differently. The 5 year patient and kidney survival rates were 90 and 60 %, respectively, for the selected group. A better patient survival rate was observed in the PE-treated group, compared to all other patients without PE therapy (p < 0.03). In addition, for patients requiring dialysis at presentation, renal improvement was better in the PE-treated group compared to patients receiving only supportive therapy (p = 0.05). When looking at the therapy-dependent outcome of renal function according to the pathologic type (glomerular, arteriolar or mixed), we observed that all PE-treated patients with pure glomerular lesions recovered, unlike those with arteriolaror or mixed lesions (p = NS). On the other hand, no long-term beneficial effects of << active therapy >> (37 patients) compared to supportive therapy alone (16 patients) were observed on renal function, but the 2 groups were not completely comparable. These data seem sufficient to justify the establishment of a prospective trial comparing PE therapy with fresh-frozen plasma as the replacement fluid to supportive therapy alone 2nd with different subgroups defined by pathological findings.