title: Posterior reversible encephalopathy syndrome and systemic vasculitis: report of six cases.
abstract: OBJECTIVE
  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.
authors.affiliation: CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Lioger
authors.affiliation: CHRU de Tours, Service de Médecine Interne, Tours, France.
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Diot
authors.affiliation: CHU de la Conception, Service de Médecine Interne, Assistance Publique, Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
authors.forename: Michael
authors.initials: M
authors.lastname: Ebbo
authors.affiliation: CHU de la Conception, Service de Médecine Interne, Assistance Publique, Hôpitaux de Marseille, Aix-Marseille Université, Marseille, France.
authors.forename: Nicolas
authors.initials: N
authors.lastname: Schleinitz
authors.affiliation: CH Jacques Cœur, Service de Médecine Interne, Bourges, France.
authors.forename: Laurent
authors.initials: L
authors.lastname: Aaron
authors.affiliation: CHU Kremlin Bicêtre, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
authors.forename: Jean-Marie
authors.initials: JM
authors.lastname: Michot
authors.affiliation: CHU Kremlin Bicêtre, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Le Kremlin-Bicêtre, France.
authors.forename: Olivier
authors.initials: O
authors.lastname: Lambotte
authors.affiliation: CHU Avicenne, Service de Médecine Interne, Assistance Publique-Hôpitaux de Paris, Bobigny, France.
authors.forename: Robin
authors.initials: R
authors.lastname: Dhote
authors.affiliation: CHU de Caen, Service de Médecine Interne, Caen, France.
authors.forename: Hubert
authors.initials: H
authors.lastname: De Boysson
authors.affiliation: CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
authors.forename: Elodie
authors.initials: E
authors.lastname: Ponce
authors.affiliation: CHRU de Tours, Service de Médecine Interne; and Université François Rabelais, Tours, France.
authors.forename: François
authors.initials: F
authors.lastname: Maillot
mesh_terms: D000328:Adult; D000959:Antihypertensive Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008875:Middle Aged; D054038:Posterior Leukoencephalopathy Syndrome / Q000188:drug therapy / Q000209:etiology*; D056647:Systemic Vasculitis / Q000150:complications*
chemical_list: D000959:Antihypertensive Agents; D007166:Immunosuppressive Agents