Neurological Symptoms in AAV Patients Do Not Impact Likely Long-term Survival, Study Reports

Almost half of patients diagnosed with ANCA-associated vasculitis (AAV) showed neurological symptoms in a single-site, retrospective study from India. But while nervous system involvement appears to be more common than thought, it does not affect long-term survival, its researchers said.

The study, “Neurological Manifestations Do not Affect Cumulative Survival in Indian Patients with Antineutrophil Cytoplasmic Antibody Associated Vasculitis,” was published in the journal Neurology India.

Most of the damage seen in AAV occurs in the lungs and kidneys, but patients can experience a wide range of neurological symptoms affecting both the central and peripheral nervous system.

“Traditionally thought to occur during the course of a well-established vasculitic illness,” the researchers wrote, “our data shows that a significant proportion of patients may present with either central or peripheral nervous system involvement early in the course of their disease.”

Peripheral neuropathy is the most common neurological symptom, marked by damage to the nerves carrying messages from the brain to the rest of the body, and causing pain, numbness in the arms and legs, and impaired muscle movement. Seizures, cerebrovascular events, or meningitis may also occur in people with this neuropathy.

The clinical manifestations of AAV can vary according to geographical location, which led investigators with the Post Graduate Institute of Medical Education and Research in North India to assess the prevalence of neurological symptoms in AAV patients being followed at their institute’s rheumatology division.

They also looked at whether these symptoms might lead to more severe disease and earlier death.

Their study included 92 AAV patients: 67 with granulomatosis with polyangiitis (GPA), 14 with microscopic polyangiitis, eight with eosinophilic granulomatosis with polyangiitis (EGPA), and three with undifferentiated AAV. The majority (63%) were women, the mean age was 42, and all were followed for a median of 31.1 months (about 2.6 years).

Neurological symptoms were seen in 45.7% of these people, including 10.9% for whom they were the first clinical evidence of this disease.

Similar to prior reports, peripheral neuropathy was the most common neurological symptom, seen in 21.7% of these people. This symptom was particularly more frequent among those diagnosed with EGPA (87.5%) and undifferentiated AAV (100%).

Hearing loss (17.9%) and facial paralysis (10.5%) were detected only in GPA patients.

People with neurological symptoms were more likely to have musculoskeletal symptoms but less likely to have kidney problems, the researchers found. No association was seen with disease evidence in other organs, namely the cardiac, gastrointestinal, and upper respiratory systems.

Almost all patients (97.6%) with nervous system involvement were being treated with oral or intravenous steroids, including everyone with peripheral neuropathy.

Survival rates were not seen to be significantly affected. People with nervous system involvement were estimated to live a median of 95 months after diagnosis, compared to 114 months for those without it. “Nervous system involvement, central or peripheral, does not contribute to mortality in these patients,” the researchers concluded.

“Our study confirms that neurological manifestations occur in a significant number of Indian patients suffering from systemic ANCA-associated vasculitis” they added, but “survival is unaffected by nervous system involvement.”