Angioplasty of the renal artery as a trigger for acute anti-phospholipid syndrome
Sir,Anti-phospholipid syndrome (APS, Hughes’ syndrome) is a multifaceted systemic autoimmune disease, whose spectrum ranges from tempestuous and deadly to silent and smouldering [1–3]. APS is characterized by recurrent deep venous thrombosis, although the involvement of arterial vessels has been increasingly described [1–3]. The contribution of APS to the development of ‘idiopathic’ renal artery stenosis remains unclear, also because the assessment of antibody status is not a part of the routine diagnostic work-up.
We report here a case in which angioplasty of renal arteries triggered acute APS, a life-threatening complication, never described thus far.
The patient was a 77-year-old woman, affected by type 2 diabetes, moderate hypertension, diffuse vascular disease and recurrent atrial fibrillation. Three years before this report, she was diagnosed with ‘moderate’ right renal artery stenosis. Renal function was normal. The patient was managed conservatively for 2 years, until the right renal artery stenosis progressed to >70%, and left renal artery stenosis <70% was detected. After a severe hypertensive crisis and a rise in serum creatinine, to 1.4 mg/dl, percutaneous transluminal angioplasty (PTA) with stenting of both renal arteries was successfully performed.
A sudden drop of thrombocytes was observed a few hours after the procedure (116 000 pre-angioplasty, 33 000 after 8 h); serum creatinine rose from 1.4 to 3.1 mg/dl. Echo-doppler confirmed the patency of renal arteries, with high intraparenchymal resistances. On the hypothesis of an autoimmune phenomenon, she was empirically started on low-dose corticosteroids and fresh plasma infusion. In the following days, serum creatinine reached 4.7 mg/dl, with nephrotic proteinuria (7 g/day). The immunological tests revealed positivity in the Lupus anticoagulant test, anti-cardiolipin and Anti-GPI: Anti Beta2 glycoprotein 1 antibodies. In keeping with a diagnosis of acute burst of APS, triggered by the procedure, on a pre-existent APS with a smouldering course, the patient was treated with three bolus doses of methylprednisolone 500 mg, and with six sessions of plasmapheresis, with a rapid rise of the platelet count and normalization of coagulation.
At hospital discharge, 25 days later, on chronic Warfarin and oral prednisone, serum creatinine was 2 mg/dl, proteinuria 2.7 g/day, creatinine clearance 35 ml/min, haemoglobin 11 g/dl and platelet count 90–110 000 platelets/mm3.
This case is characterized by the presence of a mechanical trigger, arteriography and renal stenting, which presumably released into the circulation factors that induced a prompt immunological response in the presence of pre-formed antibodies, thus precipitating an acute APS. According to a systematic search on Medline and Embase, this is the first report of a life-threatening acute APS triggered by renal artery stenting.
In the context of systemic vasculitis, the risk of accelerated atherosclerosis is recognized, and systematic echo-doppler surveillance is increasingly advised [4,5]. On the contrary, although an increased prevalence of autoantibodies in severe vascular disease has been reported, a systematic search for autoimmunity is not routinely performed [6,7].
The present case, describing a new trigger for acute severe autoimmune bursts, suggests that a minimal immunological diagnostic work-up should be considered, at least in high-risk patients in whom an immune-mediated reaction could have severe, even deadly, consequences.
1 Chair of Nephrology University of Torino 2 Nuclear Medicine Unit 3 Interventional Radiology ASO ‘S. Giovanni Battista’ Torino, Italy Email: gbpiccoli{at}yahoo.it; giorgina.piccoli{at}unito.it; gbpiccoli{at}hotmail.com
Acknowledgments
The authors would like to thank Dr P. Christie, for his careful language revision.
Conflict of interest statement. None declared.
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