IngentaConnect Thrombotic microangiopathy in adult Still's disease

Authors: Quéméneur, T.¹; Noel, L-H.²; Kyndt, X.¹; Droz, D.³; Fleury, D.¹; Binaut, R.¹; Lemaitre, V.¹; Gobert, P.¹; Vanhille, P.¹

Source: Scandinavian Journal of Rheumatology, Volume 34, Number 5, 01Oct2005 , pp. 399-403(5)

Publisher: Informa Healthcare

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Abstract:

Adult Still's disease (ASD) is a rare systemic disorder characterized by fever, arthralgia, cutaneous rash, and lymphadenopathy, with high polymorphonuclear leucocytosis and low glycosylated ferritinaemia. Kidney involvement has been reported rarely. We present a patient with ASD who developed haemolytic uraemic syndrome (HUS). The 42-year-old patient was admitted for unexplained fever related to ASD according to Yamaguchi's classification criteria. As Still's disease was resistant to prednisone, high-dose intravenous immunoglobulins (IV Ig) were administered. During the follow-up the patient developed acute renal failure and non-immune haemolytic anaemia with high levels of antiphospholipid antibodies (IgG anticardiolipin antibodies and anti- beta

2 glycoprotein 1 antibodies). Renal biopsy disclosed thrombotic microangiopathy (TMA) with arteriolar and glomerular involvement. Treatment with steroids and intravenous IV Ig was reinitiated but renal function worsened towards end-stage renal failure. In this case, we suggest that antiphospholipid antibodies could have promoted arteriolar and glomerular TMA. HUS may be the cause of acute renal failure in Still's disease.

Document Type: Research article

DOI: 10.1080/03009740510026689

Affiliations: 1: Department of Nephrology and Internal Medicine, Valenciennes Hospital, Valenciennes 2: INSERM U507 and Department of Pathology, Necker Hospital, Paris 3: Department of Pathology, St Louis Hospital, Paris, France