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The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood–nerve barrier. We describe its normal anatomy and function. “Perineuritis” refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined perineuritis. We searched PubMed, Embase, Scopus, and Web of Science for “perineuritis.” We identified 20 cases (11 M/9F) of PP: progressive, unexplained neuropathy with biopsy showing perineuritis without vasculitis or other known predisposing condition. Patients ranged in age from 18 to 75 (mean 53.7) y and had symptoms 2–24 (median 4.5) mo before diagnosis. Neuropathy was usually sensory‐motor (15/20), painful (18/19), multifocal (16/20), and distal‐predominant (16/17) with legs more affected than arms. Truncal numbness occurred in 6/17; 10/18 had elevated cerebrospinal fluid (CSF) protein. Electromyography (EMG) and nerve conduction studies (NCS) demonstrated primarily axonal changes. Nerve biopsies showed T‐cell‐predominant inflammation, widening, and fibrosis of perineurium; infiltrates in epineurium in 10/20 and endoneurium in 7/20; and non‐uniform axonal degeneration. Six had epithelioid cells. 19/20 received corticosteroids, 8 with additional immunomodulators; 18/19 improved. Two patients did not respond to intravenous immunoglobulin (IVIg). At final follow‐up, 13/16 patients had mild and 2/16 moderate disability; 1/16 died. Secondary causes of perineuritis include leprosy, vasculitis, neurosarcoidosis, neuroborreliosis, neurolymphomatosis, toxic oil syndrome, eosinophilia‐myalgia syndrome, and rarer conditions. PP appears to be an immune‐mediated, corticosteroid‐responsive disorder. It mimics nonsystemic vasculitic neuropathy. Cases with epithelioid cells might represent peripheral nervous system (PNS)‐restricted forms of sarcoidosis.
The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood–nerve barrier. We describe its normal anatomy and function. “Perineuritis” refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined perineuritis. We searched PubMed, Embase, Scopus, and Web of Science for “perineuritis.” We identified 20 cases (11 M/9F) of PP: progressive, unexplained neuropathy with biopsy showing perineuritis without vasculitis or other known predisposing condition. Patients ranged in age from 18 to 75 (mean 53.7) y and had symptoms 2–24 (median 4.5) mo before diagnosis. Neuropathy was usually sensory‐motor (15/20), painful (18/19), multifocal (16/20), and distal‐predominant (16/17) with legs more affected than arms. Truncal numbness occurred in 6/17; 10/18 had elevated cerebrospinal fluid (CSF) protein. Electromyography (EMG) and nerve conduction studies (NCS) demonstrated primarily axonal changes. Nerve biopsies showed T‐cell‐predominant inflammation, widening, and fibrosis of perineurium; infiltrates in epineurium in 10/20 and endoneurium in 7/20; and non‐uniform axonal degeneration. Six had epithelioid cells. 19/20 received corticosteroids, 8 with additional immunomodulators; 18/19 improved. Two patients did not respond to intravenous immunoglobulin (IVIg). At final follow‐up, 13/16 patients had mild and 2/16 moderate disability; 1/16 died. Secondary causes of perineuritis include leprosy, vasculitis, neurosarcoidosis, neuroborreliosis, neurolymphomatosis, toxic oil syndrome, eosinophilia‐myalgia syndrome, and rarer conditions. PP appears to be an immune‐mediated, corticosteroid‐responsive disorder. It mimics nonsystemic vasculitic neuropathy. Cases with epithelioid cells might represent peripheral nervous system (PNS)‐restricted forms of sarcoidosis.
Objectives To assess self-reported symptoms of neuropathy, disability, pain, health-related quality of life (HR-QOL), and autonomic dysfunction in patients with vasculitis. Methods Patients with vasculitis (with or without neuropathy) were invited by Vasculitis UK to complete an anonymous online survey. Results 312 patients (71% female) responded. Median age was 61–70 years. Median duration of vasculitis was 4 years (<2 months to > 15 years). Vasculitic types included granulomatosis with polyangiitis (34%), unspecified ANCA-associated vasculitis (13%), microscopic polyangiitis (11%), eosinophilic granulomatosis with polyangiitis (11%), giant cell arteritis (10%), non-systemic vasculitic neuropathy (2%), and other (19%). Many patients reported foot/hand symptoms suggestive of neuropathy, including numbness (64%), pain (54%), or weakness (40%). 242 patients (78%) met our definition of probable vasculitic neuropathy: diagnosis of neuropathy by vasculitis team OR numbness OR weakness in feet/hands. Only 52% had been formally diagnosed with neuropathy. Compared with 70 patients without neuropathy, neuropathy patients had greater disability measured by the inflammatory Rasch-built Overall Disability Scale (centile mean 63.1 (SD 17.3) vs 75.2 (16.7); p< 0.0001), Inflammatory Neuropathy Cause and Treatment scale (median 2 (IQR 1–4) vs 0.5 (0–2); p< 0.0001), and modified Rankin scale (median 2 (IQR 1–3) vs 2 (1–2); p= 0.0002); greater pain on an 11-point rating scale (mean 4.6 (SD 2.6) vs 3.5 (2.8); p= 0.0009); and poorer HR-QOL on the EQ5D-3L (summary index mean 0.58 (SD 0.29) vs 0.69 (0.28); p<0.0001). Two-thirds reported autonomic symptoms (not associated with neuropathy). Conclusion Neuropathy is common and associated with significant disability, pain, and impaired HR-QOL in patients with systemic vasculitis.
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