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Atypical haemolytic uremic syndrome complicated by microangiopathic antiphospholipid-associated syndrome

A Megli

Department of Nephrology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia

Grosek

Department of Pediatric Surgery and Intensive Therapy, University Medical Center Ljubljana, Ljubljana, Slovenia

M Benedik-Dolniar

Department of Hematology and Oncology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia

T Avin

Department of Allergology, Rheumatology and Clinical Immunology, University Children's Hospital, University Medical Center Ljubljana, Ljubljana, Slovenia

A 4-year-old boy with an atypical course of haemolytic uremic syndrome (HUS), who developed microangiopathic antiphospholipid-associated syndrome (MAPS) with signs of multiple organ failure during the course of his disease, is reported. Early and aggressive treatment with intravenous gammaglobulin, pulse methylprednisolone and plasmapheresis resulted in an excellent clinical recovery. Our patient showed a concomitant presence of multiple factors that could precipitate atypical HUS, including positive antiphospholipid antibodies, decreased level of factor H and positive anti-ADAMTS-13 antibodies. We suggest that, along with infections, autoimmune conditions or defined genetic abnormalities of complement regulatory genes, MAPS should be considered among the pathogenic mechanisms in patients with atypical HUS.

Key Words: ADAMTS-13 • factor H • haemolytic uremic syndrome • microangiopathic antiphospholipid-associated syndrome

Lupus, Vol. 17, No. 9, 842-845 (2008)
DOI: 10.1177/0961203308091634


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