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Carotid dissection in IgG4-related disease: an uncommon cause of amaurosis fugax
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  1. Sandro Marini1,2 and
  2. Steven K Feske3,4
  1. 1Program in Medical and Population Genetics, Broad Institute of Harvard and the Massachusetts Institute of Technology, Harvard University, Cambridge, Massachusetts, USA
  2. 2Department of Neurology, Massachusetts General Hospital, Boston, Massachusetts, USA
  3. 3Department of Neurology, Boston Medical Center, Boston, Massachusetts, USA
  4. 4Department of Neurology, Boston University, Chobanian & Avedisian School of Medicine, Boston, Massachusetts, USA
  1. Correspondence to Dr Sandro Marini; smarini1{at}mgh.harvard.edu

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Description

A man in his 50s presented with 3 months of recurrent painless transient monocular right eye vision loss. The patient denied any recent trauma or neck manipulation. Neurological and ophthalmological examinations were unremarkable. Notably, the patient had no family history, medical history or clinical signs of connective tissue disease, such as joint hypermobility, easy bruising or poor wound healing that might suggest underlying vulnerability to vascular dissection. Erythrocyte sedimentation rate (104 mm/hour) and C reactive protein (19.6 mg/L) were markedly elevated. CT angiogram showed tortuosity and ectasia of the cervical internal carotid arteries with septa suggestive of past dissections (figure 1). Brain MRI was unremarkable. A few months earlier the patient had suffered acute renal failure and was found to have obstructive uropathy due to retroperitoneal fibrosis. Fluorodeoxyglucose positron emission tomography showed hypermetabolic soft tissue thickening along the aorta and iliac arteries (figure 2). MRI showed slight bilateral lacrimal gland enlargement (figure 3). Serum IgG4 concentration was twice the upper limit of normal. There was no cryoglobulinemia, leucopenia or eosinophilia, and tests for autoantibodies were negative. Biopsy of the retroperitoneal tissue was not pursued as it was considered unsafe. Even in the absence of histopathology, the patient was diagnosed with IgG4-related disease (IgG4-RD) based on the recently revised criteria.1 The patient was treated with prednisone for IgG4-RD and aspirin for the carotid dissection with normalisation of the IgG4 levels and no further episodes of vision loss. IgG4-RD is a systemic inflammatory disorder characterised by matted infiltration of IgG4-positive plasma cells and fibrosis in multiple tissues.2 The most frequent neurological manifestation is hypertrophic pachymeningitis, and the most common carotid-associated pathology is atherosclerosis, but arteritis is described in up to 30% of patients. We cannot be certain that there had not been prior dissections, but in the absence of a history of trauma, clinical signs of connective tissue disease, evidence of fibromuscular dysplasia in other vessels or other identifiable risk factors for carotid dissection, we suggest that the patient’s arterial dissections are likely a manifestation of IgG4-RD with vasculitis presenting as amaurosis fugax.3 4 IgG4-RD is an increasingly recognised disorder with cerebrovascular consequences and thus should be considered among the causes of vasculitis.

Figure 1

Multiplanar reconstruction image of the CT angiogram with coronal (A, B) and axial views at the corresponding dashed lines (C, D, respectively). Images show tortuosity and ectasia of both internal carotid arteries with septa of the right (C) and left (D) internal carotid arteries suggesting past arterial dissections (white arrows).

Figure 2

Fluorodeoxyglucose positron emission tomography (18F-FDG PET) showing patchy increased radiopharmaceutical uptake along the infrarenal aorta involving the proximal common iliac arteries (white arrowheads).

Figure 3

MRI showing engorgement of the retrobulbar vessels of the left eye. Contrast-enhanced T1-weighted coronal image showing well-enhanced and enlarged lacrimal glands (white arrowheads).

Learning points

  • Elevated serum IgG4 and retroperitoneal fibrosis are key features of IgG4-related disease (IgG4-RD).

  • IgG4-RD should be considered among unusual causes of vasculitis.

  • Arteritis due to IgG4-RD should be considered in cases of stroke without vascular risk factors.

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References

Footnotes

  • Contributors SM and SKF were responsible for drafting of the text, sourcing and editing of clinical images, investigation results, drawing original diagrams and algorithms, and critical revision for important intellectual content. SM and SKF gave final approval of the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.