title: Extra- and intracranial cerebral vasculitis in giant cell arteritis: an observational study.
abstract: Recognizing giant cell arteritis (GCA) in patients with stroke may be challenging. We aimed to highlight the clinical spectrum and long-term follow-up of GCA-specific cerebrovascular accidents. Medical charts of all patients followed in a French Department of Internal Medicine for GCA between January 2008 and January 2014 were retrospectively reviewed. Patients with cerebrovascular accidents at GCA diagnosis were included. Diagnosis of GCA was based on American College of Rheumatology criteria. Transient ischemic attacks and stroke resulting from an atherosclerotic or cardioembolic mechanism were excluded. Clinical features, GCA-diagnosis workup, brain imaging, cerebrospinal fluid (CSF) study, treatment, and follow-up data were analyzed. From January 2008 to January 2014, 97 patients have been followed for GCA. Among them, 8 biopsy-proven GCA patients (mean age 70±7.8 years, M/F sex ratio 3/1) had stroke at GCA diagnosis. Six patients reported headache and visual impairment. Brain MR angiography showed involvement of vertebral and/or basilar arteries in all cases with multiple or unique ischemic lesions in the infratentorial region of the brain in all but one case. Intracranial cerebral arteries involvement was observed in 4 cases including 2 cases with cerebral angiitis. Long lasting lesions on diffusion-weight brain MRI sequences were observed in 1 case. All patients received steroids for a mean of 28.1±12.8 months. Side effects associated with long-term steroid therapy occurred in 6 patients. Relapses occurred in 4 patients and required immunosuppressive drugs in 3 cases. After a mean follow-up duration of 36.4±16.4 months, all but 1 patient achieved complete remission without major sequelae. The conjunction of headache with vertebral and basilar arteries involvement in elderly is highly suggestive of stroke associated with GCA. Intracranial cerebral arteries involvement with cerebral angiitis associated with long lasting brain lesions on diffusion-weight brain MRI sequences may occur in GCA. Both frequent relapses and steroid-induced side effects argue for the use of immunosuppressive agents combined with steroids as first-line therapy.
authors.affiliation: From 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).
authors.forename: Delphine
authors.initials: D
authors.lastname: Larivière
authors.forename: Karim
authors.initials: K
authors.lastname: Sacre
authors.forename: Isabelle
authors.initials: I
authors.lastname: Klein
authors.forename: Fabien
authors.initials: F
authors.lastname: Hyafil
authors.forename: Laurence
authors.initials: L
authors.lastname: Choudat
authors.forename: Marie-Paule
authors.initials: MP
authors.lastname: Chauveheid
authors.forename: Thomas
authors.initials: T
authors.lastname: Papo
mesh_terms: D000368:Aged; D000369:Aged, 80 and over; D000792:Angiography; D001488:Basilar Artery / Q000000981:diagnostic imaging / Q000473:pathology; D001706:Biopsy; D003937:Diagnosis, Differential; D005260:Female; D013700:Giant Cell Arteritis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D020521:Stroke / Q000175:diagnosis / Q000209:etiology*; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000175:diagnosis; D014711:Vertebral Artery / Q000000981:diagnostic imaging / Q000473:pathology