Nephrology Dialysis Transplantation, Vol 13, Issue 2 296-302, Copyright © 1998 by Oxford University Press
F Bridoux, F Vrtovsnik, C Noel, P Saunier, B Mougenot, V Lemaitre, M Dracon, G Lelievre and P Vanhille
Background. Renal thrombotic microangiopathy (TMA) is
an uncommon vascular complication of systemic lupus erythematosus (SLE).
Its clinical symptoms and impact on renal survival remain unclear.
Methods. Eight patients aged 25±6 years
with biopsy-proven renal TMA and at least four ARA criteria for the
diagnosis of SLE were retrospectively studied over a 7-year period.
Results. All patients presented with renal failure
(creatinine 3.3±2.1 mg/dl), six had proteinuria
(2.5±1.3 g/day) with microscopic haematuria in four cases. Six
patients had hypertension, which was severe in five cases. Renal histology
disclosed arterial and/or arteriolar thrombosis with parietal thickening
without angeitis (8 patients), glomerular microthrombi (3 patients), and
vascular fibrin deposits (5/6 patients). In two cases, vascular lesions
were associated with a mesangial or a proliferative glomerulonephritis.
Thrombocytopenia was present in four patients with haemolytic
microangiopathic anaemia in one case. Lupus anticoagulant (LA) was detected
in five of eight patients, who also had anticardiolipin antibodies (3/7
patients) and/or were positive for VDRL (3/6 patients). Four patients with
LA experienced arterial thrombosis and/or repeated spontaneous abortions.
Treatment consisted of corticosteroids (8 patients), cytotoxic drugs (4
patients), plasma exchanges and/or intravenous immunoglobulins (4 patients)
and antiplatelet and/or anticoagulant therapy (3 patients). Two patients
recovered normal renal function and five had persistent renal
insufficiency. One patient started haemodialysis on admission and died of
sepsis 2 months later. Conclusions. TMA may also be
the sole renal complication in SLE and is not usually associated with
haemolytic microangiopathic anaemia. In our series renal survival was
influenced by the extent and severity of vascular lesions. Despite a
frequent association with antiphospholipid antibodies, pathophysiological
mechanisms of renal TMA in SLE remain unknown. Renal histology is mandatory
for the diagnosis and the prognostic evaluation of renal vasculopathy in
SLE. Keywords: systemic lupus erythematosus;
thrombotic microangiopathy; acute renal failure; antiphospholipid
antibodies; renal biopsy
ORIGINAL ARTICLES
Renal thrombotic microangiopathy in systemic lupus erythematosus: clinical correlations and long-term renal survival
Department of Nephrology, Valenciennes, France; Department of Nephrology, Lille, France; Department of Pathology, Hopital Tenon, Paris, France; Corresponding author address: Service de Nephrologie, Centre Hospitalier de Valenciennes, Avenue Desandrouins, BP 479, F-59322 Valenciennes, France
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