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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
Published on: February 8, 2019
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).
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.
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.
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