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Posterior reversible encephalopathy syndrome and systemic vasculitis: report of six cases


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
  2. CHRU de Tours, Service de Médecine Interne, Tours, France.
  3. CHU de la Conception, Service de Médecine Interne, Assistance Publique, Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
  4. CHU de la Conception, Service de Médecine Interne, Assistance Publique, Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
  5. CH Jacques Cœur, Service de Médecine Interne, Bourges, France.
  6. CHU Kremlin Bicêtre, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
  7. CHU Kremlin Bicêtre, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
  8. CHU Avicenne, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Bobigny, France.
  9. CHU de Caen, Service de Médecine Interne, Caen, France.
  10. CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
  11. CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.

on behalf of the Société Nationale Française de Médecine Interne (SNFMI) and the CRI (Club Rhumatismes et Inflammation)

CER8392
2016 Vol.34, N°3 ,Suppl.97
PI 0007, PF 0011
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PMID: 26412031 [PubMed]

Received: 21/02/2015
Accepted : 28/05/2015
In Press: 28/09/2015
Published: 27/05/2016

Abstract

OBJECTIVES:
Our objective was to describe the characteristics of posterior reversible encephalopathy syndrome (PRES) associated with systemic vasculitis.
METHODS:
A standardised questionnaire was used for a nationwide retrospective multicentre study in 2013 to collect clinical, radiological and outcome data about PRES associated with systemic vasculitis.
RESULTS:
We included six patients (all women; mean age 22.6±19.8 years (20-62)): two with polyarteritis nodosa and one case of each granulomatosis with polyangiitis, cryoglobulinaemic vasculitis, hypocomplementemic urticarial vasculitis, and Takayasu arteritis. PRES was the first manifestation of systemic vasculitis in three patients. Arterial hypertension was suspected to be the cause of PRES in five patients. Several other plausible causes including drugs, renal failure, and pneumonia were found in three patients. Clinical findings included headache, seizure, blurred or loss of vision, confusion, and altered cognition. Radiological study showed oedema in the occipital region in all patients, with a reversible state in MRIs performed one week to one month after the onset of PRES. Therapies used included antihypertensive therapy (n=5), immunosuppressive therapy (corticosteroids (n=5), cyclophosphamide (n=4), azathioprine (n=1), methotrexate (n=1), plasma exchange (n=1)), antibiotics (n=1), anticonvulsant therapy (n=2)), and analgesics. No relapse of PRES was reported during the follow-up period (mean: 47.5 ±29.9 months, 13–98); one patient continued to complain of vision loss.
CONCLUSIONS:
Our study indicates that PRES is a rare condition associated with systemic vasculitis; which may be present at the onset vasculitis symptoms. Antihypertensive drugs should be prescribed if blood pressure is elevated. The impact of immunosuppressive therapy remains unclear.

Rheumatology Article