A 51-Year-Old Woman With Subarachnoid Hemorrhage and Secondary Central Nervous System Vasculitis With Progression to Diffuse, Serpiginous Dolichoectasia

Abstract

A 51-year-old woman presented with acute onset of a severe headache, and was found to have diffuse subarachnoid hemorrhage with prominent cisternal and left cortical convexity blood on head computed tomography. The first 2 conventional angiograms were negative for aneurysm, but a third angiogram revealed a mycotic aneurysm of a distal left middle cerebral artery branch. Brain biopsy, associated with clipping of the aneurysm, demonstrated pathology consistent with vasculitis. Over the course of a month, she developed diffuse, serpiginous dolichoectasia of the cerebral arteries. Further investigation into the cause of vasculitis supported a diagnosis of either eosinophilic granulomatosis with polyangiitis (EGPA) or IgG4-Related Disease (IgG4-RD). The following clinical pathologic conference discusses the diagnostic challenges in discriminating between these 2 diseases, particularly in the setting of secondary angiitis of the central nervous system.

Keywords: CNS vasculitis; IgG4-related; dilating arteriopathy; disease; dolichoectasia; eosinophilic granulomatosis with polyangiitis; mycotic aneurysm.

Figure 1.

Head CT on arrival (A,B). (A) Thick subarachnoid hemorrhage is noted at the basal cisterns without intraventricular involvement. (B) Subarachnoid hemorrhage is also noted at the cortical convexity of the left parietal lobe. Brain MRI on hospital day 7 (C-E). (C) About 5 small, scattered ischemic strokes were noted bilaterally on DWI (and ADC). (D) The 2 larger infarcts had surrounding vasogenic edema on FLAIR (the second 1 located at the left parietal lobe). DSA on hospital day 14. (E) Ring enhancement was seen co-localized to these 2 lesions on gadolinium contrast. Minimal leptomeningeal and pachymeningeal enhancement was also observed at the left hemisphere. (F) A left M4 cortical branch aneurysm (arrow) measuring 3 × 2 × 3 mm was found on the third DSA.

Figure 2.

Cerebral aneurysm specimen revealed necrotizing vasculitis. (A) Transmural inflammatory infiltrate of the vessel wall (Hematoxylin and eosin stain, 100x). (B) Destruction of the internal elastic lamina by infiltrate (Verhoeff-Van Gieson, 100x). (C) Numerous CD138 staining plasma cells (Immunohistochemical stain, 200x). (D) Nearly 100% of the plasma cells (>150/hpf) stained positive for IgG4 (Immunohistochemical stain, 200x).

Figure 3.

Lung core needle specimen revealed necrotizing granulomatous inflammation and eosinophilic vasculitis. (A) Eosinophilic pneumonia characterized by a fibrinous airspace exudate with prominent eosinophils (H&E stain, 400x). (B) Vasculitis involving a small artery with disruption of the elastic lamina and infiltration by inflammatory cells. (200x) (C) Necrotizing granulomatous inflammation with eosinophils surrounded by palisaded histiocytes (H&E stain, 100x).

Figure 4.

Brain MRI on the second admission, 7 weeks after the first MRI. (A), (B) Prominent vasogenic edema on FLAIR was appreciated at the right prefrontal gyrus, splenium of the corpus callosum, left parietal cortex, and the left medial temporal lobe. (C), (D) Susceptibility-weighted imaging showed serpiginous, dilated arteriopathy underlying these brain regions with vasogenic edema (E), (F) Vessel-wall imaging using T1-SPACE post-contrast demonstrated concentric enhancement of left M2/3 branches (E) and bilateral M1/2 branches as well as distal M4 branches on the left (F). Minimal pachymeningeal enhancement was seen on at the left hemisphere.