Thrombotic microangiopathy in adult Still's disease

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...

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Veröffentlicht in:Scandinavian journal of rheumatology 2005-10, Vol.34 (5), p.399-403
Hauptverfasser: Quéméneur, T., Noel, L-H., Kyndt, X., Droz, D., Fleury, D., Binaut, R., Lemaitre, V., Gobert, P., Vanhille, P.
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container_end_page 403
container_issue 5
container_start_page 399
container_title Scandinavian journal of rheumatology
container_volume 34
creator Quéméneur, T.
Noel, L-H.
Kyndt, X.
Droz, D.
Fleury, D.
Binaut, R.
Lemaitre, V.
Gobert, P.
Vanhille, P.
description 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- 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.
doi_str_mv 10.1080/03009740510026689
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subjects Acute Kidney Injury - etiology
Acute Kidney Injury - immunology
Adult
Antibodies, Antiphospholipid - immunology
Biological and medical sciences
Diseases of the osteoarticular system
Hemolytic-Uremic Syndrome - etiology
Hemolytic-Uremic Syndrome - immunology
Humans
Immunoglobulins, Intravenous
Inflammatory joint diseases
Male
Medical sciences
Microcirculation
Renal Circulation
Steroids - therapeutic use
Still's Disease, Adult-Onset - complications
Still's Disease, Adult-Onset - drug therapy
Still's Disease, Adult-Onset - immunology
Thrombosis - etiology
Thrombosis - immunology
title Thrombotic microangiopathy in adult Still's disease
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