Greater baseline motor impairment and shorter diagnostic delay were associated with neurological improvement in vasculitic neuropathy patients, with 59.1% improving by at least 2 NIS points over a median follow-up of 13.5 months.
Key Findings
Results
The majority of vasculitic neuropathy patients (59.1%) experienced neurological improvement over the follow-up period, while 33.3% worsened and 7.6% remained stable.
Of 66 patients meeting inclusion criteria, 39 (59.1%) improved by ≥2 NIS points, 5 (7.6%) were stable, and 22 (33.3%) worsened.
Improvement was defined a priori as a decrease in Neuropathy Impairment Score (NIS) of at least 2 points (ΔNIS ≤ −2).
Median follow-up was 13.5 months (IQR 6.2–31.2 months).
The cohort was retrospectively reviewed from a single tertiary-care center (Hospital of the University of Pennsylvania) between October 2020 and December 2025.
Results
Patients who improved had significantly higher baseline NIS-motor subscores compared to those who did not improve.
Baseline NIS-motor subscores were 17.3 ± 14.6 in the improvement group versus 6.7 ± 7.9 in the non-improvement group (p < 0.001).
This suggests that patients with greater initial motor impairment had more room for measurable recovery.
Neurological impairment was quantified using the Neuropathy Impairment Score (NIS).
Continuous variables were compared using Welch two-sample t-tests.
Results
Patients who improved had significantly shorter diagnostic delays compared to those who did not improve.
Diagnostic delay was 12.3 ± 16.4 months in the improvement group versus 30.3 ± 41.7 months in the non-improvement group (p = 0.042).
This association suggests that earlier diagnosis may be associated with better neurological outcomes.
Categorical variables were compared using Fisher exact tests.
Results
The most common vasculitic neuropathy subtypes in this cohort were non-systemic vasculitic neuropathy (NSVN), ANCA-associated vasculitis, and diabetic lumbosacral radiculoplexus neuropathy (LRPN).
NSVN was the most common subtype at 27.3% (n = 18).
ANCA-associated vasculitis accounted for 22.7% of cases.
Diabetic LRPN accounted for 19.7% (n = 13 of 15 total LRPN patients).
Non-systemic disease was analyzed as two distinct entities: the NSVN subtype and lumbosacral radiculoplexus neuropathy (LRPN).
Systemic vasculitic neuropathy comprised n = 33 patients.
Results
Biopsy confirmed vasculitis in the majority of patients who underwent the procedure, with the remainder classified based on clinical, electrodiagnostic, and serologic criteria.
Biopsy was performed in 48 of 66 patients and demonstrated vasculitis in 41 of those biopsied.
Overall, 59 patients were classified as definite vasculitic neuropathy and 7 as possible vasculitic neuropathy.
Diagnostic classification followed the Peripheral Nerve Society 2010 criteria and the 2022 ACR/EULAR classification criteria for systemic vasculitides.
Definite vasculitis was defined by biopsy confirmation or fulfillment of formal classification criteria; probable vasculitis was supported by clinical, electrodiagnostic, and serologic features.
Methods
The study cohort of 66 patients was derived from a retrospective single-center review of 85 initially identified adult patients with vasculitic neuropathy.
85 patients were initially reviewed; 66 met inclusion criteria.
The study was conducted at the Hospital of the University of Pennsylvania.
The review period spanned October 2020 to December 2025.
The study aimed to describe clinical features, diagnostic workup, and neurological outcomes of VN patients.
What This Means
This research studied 66 patients with vasculitic neuropathy — a condition where inflammation of blood vessels damages peripheral nerves, causing weakness and sensory problems — treated at a major academic medical center over approximately five years. Researchers tracked how patients' nerve function changed over time using a standardized scoring system called the Neuropathy Impairment Score (NIS), which measures things like muscle strength and reflexes. The most common forms of the disease in this group were non-systemic vasculitic neuropathy (affecting only peripheral nerves without widespread organ involvement), ANCA-associated vasculitis (a systemic immune condition), and a diabetes-related nerve condition affecting the lower back and legs.
Over a median follow-up of about 13.5 months, nearly 60% of patients showed meaningful neurological improvement, while about one-third worsened. Two factors stood out as being linked to better outcomes: patients who had more severe motor (muscle) weakness at the start of treatment were more likely to improve — possibly because they had more room to recover — and patients who received their diagnosis sooner after symptoms began also tended to do better. The difference in diagnostic delay between those who improved and those who did not was substantial (about 12 months versus about 30 months on average).
This research suggests that timely diagnosis may play an important role in neurological recovery for people with vasculitic neuropathy. Because this is a relatively rare and heterogeneous condition, studies like this one — which carefully characterize patient populations and track outcomes using objective measures — help establish baseline data that future, larger multicenter studies can build upon. The findings highlight vasculitic neuropathy as a condition where prompt recognition and treatment evaluation appear clinically meaningful.
Rakhmonova S, Karam C. (2026). Vasculitic neuropathy: clinical features, diagnostic approach, and neurological outcomes in a single-center cohort.. Frontiers in neurology. https://doi.org/10.3389/fneur.2026.1886100