Case Report Shows for First Time That AAV May Also Affect Brain, Spinal Cord

Researchers reported for the first time the case of a patient with ANCA-associated vasculitis whose condition caused bleeding in the brain and spinal cord, most likely because of a ruptured aneurysm.

The study, “Anti-neutrophil cytoplasmic antibody associated central nervous system vasculitis with brain and spinal cord subarachnoid hemorrhage: A rare case report and review of the literature,” was published in the Journal of Clinical Neuroscience.

ANCA-associated vasculitis affects small blood vessels, leading to their inflammation and destruction. The condition affects multiple organs, including the lungs, kidney, stomach, and intestine. In rare cases, the central nervous system may be affected.

Researchers at the University of Colorado School of Medicine presented a rare case of ANCA-associated central nervous system vasculitis that affected the brain, spinal cord, and external carotid artery.

The 48 year-old woman had been diagnosed with rheumatoid arthritis and Hashimoto’s thyroiditis (both autoimmune disorders), and presented with altered mental status, slurred speech, weakness in the lower extremities, abnormal skin sensation, and low back pain.

She also reported fever and night sweats over the past month, and had lost 40 pounds (approximately 18 kilograms) over the past four months.

Imaging scans of the brain revealed changes consistent with bleeding into the subarachnoid space (the area between the protective membranes and the central nervous system structures) or meningitis (inflammation of the protective membranes that cover the brain and spinal cord).

Analysis of the spinal cord through a lumbar puncture also revealed signs of inflammation, and the patient was started on treatment for meningitis.

However, additional imaging of the spinal cord showed some alterations within the spinal canal consistent with subarachnoid hematoma (accumulation of clotted blood). Also, a detailed evaluation of the blood vessels in the neck, brain, and spine showed segmental narrowing of several main arteries.

Blood analysis showed high levels of ANCA antibodies, without signs of kidney inflammation, leading to a diagnosis of ANCA-associated central nervous system vasculitis that caused both  cerebral and spinal subarachnoid bleeding.

“The spinal cord is rarely involved in primary or secondary central nervous system vasculitis,” researchers said. “In fact, review of case reports of 89 patients with ANCA central nervous system vasculitis showed only cerebral manifestations.”

The woman was then treated with Rituxan (rituximab) and high-dose steroids, which led to mild improvements in lower extremity weakness and normalization of inflammatory markers.

She was discharged from the hospital and admitted into a skilled nursing facility for rehab. One week after treatment initiation, she continued to have lower extremity weakness with abnormal skin sensation and difficulty swallowing.

Although spinal symptoms previously have been reported in rare cases of ANCA-associated disorders, this is the first report to describe subarachnoid bleeding in AAV, researchers said.

“This case demonstrates the important finding that AAV can have spinal cord manifestations and cervical vasculature involvement along with the more classic intra-cranial vasculitis findings,” they said.