P-ANCA vasculitic neuropathy with 12-year latency between onset of neuropathy and systemic symptoms

Abstract

Background: The differential diagnosis of chronic progressive multifocal asymmetric neuropathies is challenging. Vasculitic neuropathies, multifocal forms of chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathies, and asymmetric lower motor neuron disorders are important considerations.

Clinicopathologic features of nonsystemic vasculitic neuropathy and microscopic polyangiitis-associated neuropathy: A comparative study

“…Considering reports of vasculitis confined to a single organ such as the skin [20], gastrointestinal viscera [21], uterine cervix [22], urinary bladder [23], lungs [24], kidneys [25], or central nervous system [26], features of MPA might differ artifactually between series of patients collected by different medical specialists [5]. As for NSVN, a reported patient initially given this diagnosis showed systemic manifestations 12 years later [27]. Patients with pathologically proven vasculitis in both skin and peripheral nerve but manifesting no other systemic symptoms have also been reported [28,29].…”

Approach to Vasculitic Neuropathies

“…23 Systemic symptoms may rarely lag behind the onset of vasculitic neuropathy by 12 years or longer. 29 Immunopathogenesis Vasculitic neuropathy is caused by an autoimmune attack on blood vessels of the vasa nervorum, resulting in ischemic, primarily axonal, injury to 1 or more peripheral nerves. 24,30 These blood vessels are relatively small and range in diameter from about 50 to 250 microns.…”