Adult hemolytic uremic syndrome with renal microangiopathy. Outcome according to therapeutic protocol in 53 cases. French Cooperative Study Group for Adult HUS.
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 arteriolar 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.(ABSTRACT TRUNCATED AT 250 WORDS)
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D005500:Follow-Up Studies; D006463:Hemolytic-Uremic Syndrome / Q000150:complications* / Q000401:mortality / Q000628:therapy; D006801:Humans; D007668:Kidney / Q000098:blood supply*; D008297:Male; D008875:Middle Aged; D010949:Plasma; D010951:Plasma Exchange; D012189:Retrospective Studies; D013927:Thrombosis / Q000150:complications* / Q000401:mortality / Q000628:therapy; D013997:Time Factors