Accidents ischémiques cérébraux et atteinte cardiaque associés à un syndrome de Churg et Strauss

Cerebral vasculitis is a very rare extra-articular complication of rheumatoid arthritis (RA) that is often challenging to diagnose. Elevated titers of rheumatoid factor (RF), anti-cyclic citrullinated peptide antibodies (anti-CCP), and antinuclear antibodies (ANA) have been linked with severe complications. The absence of highly elevated titers of RF, anti-CCP, and ANA can complicate the diagnosis of RA-associated cerebral vasculitis. We report the case of a 59-year-old woman with long-standing arthritis maintained on rituximab and leflunomide who developed sudden headaches and altered level of consciousness. Laboratory work-up revealed normal lymphocyte count and mildly elevated total serum protein and anti-CCP with negative RF and ANA and no evidence for viral or bacterial infections. Cerebrospinal fluid analysis (CSF) showed slightly elevated anti-CCP with normal levels of CXCL-13 and interleukin 6 (IL-6). Brain magnetic resonance imaging (MRI) showed ill-defined lesion of high T2 signal. Using MR angiogram, MR perfusion, and MR spectroscopy, the diagnosis of rheumatoid cerebral vasculitis was confirmed. The patient was treated with intravenous methyl-prednisolone with fast complete improvement. We conclude that adequate immunosuppression in RA might not be able to prevent rare extra-articular manifestations such as rheumatoid cerebral vasculitis.

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“…35,36 However, CSF may also be entirely unremarkable, with absence of inflammatory markers. 26,37,38…”

Section: Discussionmentioning

confidence: 99%

Rheumatoid Cerebral Vasculitis in a Patient in Remission

Hasbani

Ouweini

Dabdoub

et al. 2022

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Stroke due to Systemic Diseases

Morales-Vidal

Biller

2013

Neurological Disorders Due to Systemic Disease

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Multiple cerebral infarctions in a patient with Churg-Strauss syndrome

et al. 2010

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Neurological manifestation of Churg-Strauss syndrome (CSS) is common and usually consists of peripheral neuropathy due to small-vessel vasculitis, while cerebral manifestation is less frequent. We report the case of a 77-year-old woman with multiple cerebral infarctions and hypereosinophilia. The presence of hypereosinophilia, asthma, sinusitis and vasculitis led to the diagnosis of CSS. As cerebral infarctions occurred monophasically and an elevation of heart enzymes was present, we assumed cardiac involvement and multiple cerebral infarctions due to cardiac embolism. Treatment with high-dose IV methylprednisone and cyclophosphamide pulses led to significant improvement. This case illustrates multiple cerebral infarctions in CSS. CSS should always be considered in patients with hypereosinophilia and stroke.

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Accidents ischémiques cérébraux et atteinte cardiaque associés à un syndrome de Churg et Strauss

Cited by 9 publications

References 5 publications

Rheumatoid Cerebral Vasculitis in a Patient in Remission

Rheumatoid Cerebral Vasculitis in a Patient in Remission

Stroke due to Systemic Diseases

Multiple cerebral infarctions in a patient with Churg-Strauss syndrome

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