Refractory Giant Cell Arteritis Complicated by Vision Loss From Optic Atrophy and Maculopathy Associated With Pachymeningitis

Abstract

Background: We describe a 75-year-old woman who experienced vision loss in her left eye due to biopsy-proven giant cell arteritis (GCA). She subsequently developed pachymeningitis causing refractory headaches and bilateral optic neuropathy and maculopathy.

Methods: Case report with literature review.

Results: Eighteen months after the initial diagnosis of GCA, imaging studies in our patient demonstrated pachymeningeal enhancement, and meningeal biopsy confirmed lymphoplasmacytic tissue infiltrates with low frequencies of IgG4+ plasma cells. Laboratory investigation revealed the presence of 3 antiretinal antibodies and antimyeloperoxidase antibodies, consistent with autoimmune retinopathy. Treatment with B-cell-depleting anti-CD20 antibodies suppressed meningeal inflammation and prevented further vision loss.

Conclusions: This case illustrates that bilateral vision loss and chronic headaches in patients with GCA may result from retina-directed autoimmunity and pachymeningitis.

Figure 1

Temporal artery biopsy. A. There is transmural fibroinflammatory features of giant cell arteritis. Marked intimal proliferation has produced pinpoint luminal narrowing (hematoxylin & eosin, × 50). B. CD 138 immunohistochemical staining shows plasma cells within the intimal and medial infiltrates (× 60). C, F. Rare IgG4+ plasma cells are observed at low and high power magnification (C. ×60; F. ×200). D, E. CD3 and CD4 immunostaining, respectively, reveal dense CD4+ T-cell infiltrates within all layers of the vessel wall (D. × 100; E × 60).

Figure 2

A. Automated perimetry performed at the time of diagnosis of giant cell arteritis. B. Less field loss is detected three and one-half years later.

Figure 3

One and one-half years after the diagnosis of giant cell arteritis, optical coherence tomography shows normal retinal nerve fiber layer thickness (A) and total retinal thickness (B). Two years later there is diminished retinal nerve fiber layer (C) and total retinal (D) thickness.

Figure 4

Brain MRI performed one and one-half years after initial presentation shows pachymeningeal enhancement (arrows) on postcontrast axial (A) and coronal (B) T1 scans. Three and one-half years after initial presentation, there is increased pachymeningeal enhancement on postcontrast axial (C) and coronal (D) T1 images.

Figure 5

Meningeal biopsy. A. Thickened dural tissue demonstrates dense collagenous fibrosis and clusters of chronic inflammatory cells (hematoxylin & eosin, × 100). B. Higher magnification shows perivenular infiltrates composed predominantly of lymphocytes and occasional plasma cells without vascular mural injury (hematoxylin & eosin, × 400). C. IgG4 immunohistochemical staining reveals only rare IgG4-positive cells (× 200).