Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study

Delphine Larivière1, Karim Sacre, Isabelle Klein

  • 1From the Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Hôpital Bichat; Département de Médecine Interne, Paris, France (DL, KS, MPC, TP); Département Hospitalo-Universitaire (DHU) Fire, Paris, France (KS, TP); INSERM U1146, Paris, France (KS, TP); Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Hôpital Bichat; Département de Radiologie, Paris, France (IK); Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Hôpital Bichat; Département de Médecine Nucléaire, Paris, France (FH); and Université Paris Diderot, PRES Sorbonne Paris Cité, Paris, France; Assistance Publique Hôpitaux de Paris, Hôpital Bichat; Département de Pathologie, Paris, France (LC).

Medicine
|December 20, 2014
PubMed

Insights

Giant cell arteritis (GCA) stroke in the elderly often presents with headache and vertebral/basilar artery involvement. Early immunosuppressive therapy alongside steroids may improve outcomes and reduce relapses in GCA patients.

Area of Science:

  • Rheumatology
  • Neurology
  • Vascular Medicine

Background:

  • Giant cell arteritis (GCA) diagnosis can be challenging in patients presenting with stroke.
  • Cerebrovascular accidents (CVAs) are a potential manifestation of GCA, necessitating specific diagnostic considerations.

Purpose of the Study:

  • To elucidate the clinical spectrum of GCA-specific CVAs.
  • To report on the long-term follow-up of patients experiencing stroke at the time of GCA diagnosis.

Main Methods:

  • Retrospective review of medical charts of GCA patients diagnosed between January 2008 and January 2014.
  • Inclusion criteria: patients with CVAs at GCA diagnosis, excluding atherosclerotic or cardioembolic causes.
  • Analysis of clinical features, diagnostic workup, neuroimaging, CSF studies, treatment, and follow-up data.

Main Results:

  • Eight biopsy-proven GCA patients (mean age 70) experienced stroke at diagnosis.
  • Headache and visual impairment were reported in six patients.
  • Vertebral/basilar artery involvement and infratentorial lesions were common; intracranial involvement occurred in 4 cases.
  • Long-term steroid therapy (mean 28.1 months) led to side effects in 6 patients; 4 relapsed, requiring immunosuppressants.
  • Complete remission was achieved in all but one patient after a mean follow-up of 36.4 months.

Conclusions:

  • Headache combined with vertebral/basilar artery involvement strongly suggests stroke associated with GCA in the elderly.
  • Cerebral angiitis and long-lasting MRI lesions can occur in GCA.
  • The combination of frequent relapses and steroid side effects supports using immunosuppressive agents with steroids as first-line therapy for GCA-related stroke.

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