Vasculitic Peripheral Neuropathy, differences between Systemic and Non-Systemic etiologies. (P2.4-008)

Abstract

Objective: The objective of this study is to differentiate the clinical presentation, complementary studies and evolution between the Systemic Vasculitis (SV) vs Non-Systemic Vasculitic Neuropathy (NSVN) attended in our center.

Background: The inflammation of the vessels that causes the injury of the peripheral nerves is known as Vasculitic Peripheral Neuropathy (VPN). It can occur in the context of SV or NSVN.

Design/Methods: In this retrospective analysis, we assessed medical records of patients diagnosed with VPN between 2002 and 2018. All the patients were evaluated clinically and diagnosed from the laboratory, neurophysiological study and peripheral nerve biopsy. The comparison between SV vs. NSVN was performed by parametric and non-parametric methods.

Results: We included 23 patients with VPN confirmed by biopsy: n = 11 VS (eosinophilic granulomatosis with polyangiitis = 4, rheumatoid arthritis = 4, granulomatous with polyangiitis = 2, Sjögren syndrome = 1) and 12 NSVN. The average age of presentation was 67 and 71 years respectively. The M:F ratio was 1: 1. The diagnostic delay was higher in NSVN (11.5 months vs. 5 months, p = 0.0037). High values of erythrocyte sedimentation rate (ESR) were more frequent in patients with SV (100% vs. 58%, p = 0.03). The most frequent form of presentation was mononeuritis multiplex (86.9%), followed by asymmetric polyneuropathy (8.7%) and symmetric (4.4%). All patients received initial treatment with corticosteroids, while 16 required other immunosuppressive drugs: cyclophosphamide (7), intravenous Immunoglobulin (6), methotrexate (5), azathioprine (3) or rituximab (2). The majority evolved with relapses that led to therapeutic scaling (8).

Conclusions: Neuropathies iniciated as mononeuritis multiplex should make us suspect VPN, although this is not the only form of presentation. Systemic compromise, elevated ESR and specific antibodies point to SV. Nerve biopsy should be performed early, considering that this is the gold standard for diagnosis and it can change the prognosis of these diseases.

Disclosure: Dr. Castiglione has nothing to disclose. Dr. Marrodan has nothing to disclose. Dr. Taratuto has nothing to disclose. Dr. Alessandro has nothing to disclose. Dr. Brand has nothing to disclose. Dr. Barroso has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Pfizer. Dr. Barroso has received research support from Alnylam.