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Vol. 28. Issue. 3.July 2008
Pages 241-359
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Beçet's disease in a patient on haemodialysis
Enfermedad de Bechet en un paciente en hemodiálisis
Ana María Suárez Lauresa, L.. Quiñonesa, A.. Pobesa, R.. Forascepia
a Sección de Nefrología, Hospital de Cabueñes, Gijón, Asturias, España,
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Behçet¿s disease is a rare  inflammatory  disorder  of  an unknown  origin  defined  by  the  presence  of  relapsing  oral  ulcers  and  at least two of the following: genital ulcers, eye or skin lesions, and a positive  pathergy  test.1 Vascular  involvement as venous or arterial thrombosis and aneurysms (vascular Behçet¿s disease) may be predominant and cause morbidity  and  mortality.2 Renal  involvement  has  been  reported  in  one third of  cases.  In  a  series of 159 patients,  it was secondary  to amyloidosis in 69, to glomerulonephritis in 51, from  a  vascular  cause  in  35,  and  to interstitial nephritis in 4 patients.3

Glucocorticoids  are  the drugs most commonly  used  in  patients with moderate  to  severe  involvement,4,5 but there  is  evidence  of  the  value  of  cyclosporin,6,7 azathioprine,8 cyclophosphamide5 and,  in  recent  years,  infliximab.9

We report the case of a 47-year old male  patient  who  first  attended  the nephrology  outpatient  clinic  in  2000. He had been diagnosed Behçet¿s disease based  on  clinical  criteria,  including  the  presence  of  oral  and  genital ulcers  and  recurrent  uveitis.  He  had experienced  an  episode  of  deep  vein thrombosis.  The  patient  was  a  HCV carrier  with  no  viral  replication  data and  normal  liver  function  tests.  He had chronic  renal  failure with plasma creatinine  levels  of  2.3 mg/dL and  a creatinine  clearance  of  37  mL/min, normal urinary sediment and negative proteinuria.  This  was  related  to  a nephritis-uveitis  syndrome  associated to Behçet¿s disease. Patient was  treated  with  steroids  first  and  then  with cyclosporin,  adjusting  dose  to  maintain trough  levels  of  50-100  ng/mL. Kidney  function  gradually  impaired, and in May 2007 haemodialysis had to be  started. Because of  the  absence of signs secondary to Behçet¿s disease in the last years, cyclosporin was discontinued.  In  the  following  months,  the patient  experienced  skin  lesions  consistent with seborrhoeic eczema in the scalp and face, and a decreased visual acuity  probably  related  to  a  new  episode  of  uveitis. An  angio-CT scan  of the  lower limbs  requested  before  inclusion  in  the waiting  list  for  kidney transplant showed in the distal part of the  superficial  femoral  artery  an aneurysmal dilation up to the popliteal artery (fig. 1).

After  haemodialysis  start  and  discontinuation  of  immunosuppressive therapy,  the  patient  developed  eye, skin, and vascular manifestations that required surgery and  led us  to  reconsider  resumption  of  immunosuppressive  treatment. Cyclosporin was used as  immunosuppressant because nephrotoxicity  was not  a  concern  at  this time  and  avoidance  of  steroids,  that could  promote HCV replication, was desirable. Behçet¿s disease is therefore  a  multisystemic condition  requiring  a  multidisciplinary  team  for  its management. Though treatment duration  is not clearly defined because of the  possibility  of  recurrence involving  a  high  morbidity,  an  indefinite therapy consisting of an induction period with more aggressive drugs and a maintenance  period  switching  to drugs with less side effects should be considered.

Pre-transplant  evaluation  is  another aspect  to  be  defined  because  vascular signs, as occurred in the case reported, may be a casual finding and affect virtually any vascular territory. A vascular systemic  evaluation  should  be  made even in asymptomatic patients.

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