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Vasculitic neuropathies
How are the vasculitic neuropathies classified?
Vasculitic neuropathies can be classified into either systemic vasculitic neuropathies (SVN) or nonsystemic vasculitic neuropathies (NSVN), where the vasculitis is largely restricted to the peripheral nervous system.
Vasculitis itself can be primary (e.g., Churg–Strauss syndrome, microscopic polyangitis, polyarteritis nodosa) or secondary (e.g., associated with connective tissue disorders such as rheumatoid arthritis; Sjögren’s syndrome; infections such as hepatitis B, CMV, or HIV; or other causes such as medications).
What are the symptoms of Vasculitic neuropathies
The classic presentation from a neuropathy point of view involves acute or subacute painful sensory loss and weakness in the distribution of multiple peripheral nerves (a mononeuritis multiplex pattern).
The stepwise progression and asymmetric multifocal involvement may become less evident as affected nerve territories become more confluent.
Rare patients do have a distal symmetric sensory or sensorimotor neuropathy, and a plexopathy or polyradiculoneuropathy pattern may also occur.
Pain and sensory symptoms are almost always present. In order of frequency, the most common nerves affected are the peroneal, sural, tibial, ulnar, median, radial, femoral, and sciatic.
In SVN, there may be constitutional symptoms (fever, weight loss, malaise) that generally are absent in NSVN.
How are Vasculitic neuropathies treated?
Treatment for SVN usually involves induction therapy with steroid and cyclophosphamide (intravenous pulsed or oral daily doses) transitioning after several months to methotrexate or azathioprine to maintain remission.
NSVN in contrast to SVN usually runs a more indolent course and may remit without treatment.
For vasculitides associated with viral infections, treatment is aimed at the underlying infection (e.g., hepatitis C).
Sources
- Muppidi S, Vernino S: Paraneoplastic neuropathies. Continuum (Minneap Minn) 20(5):1359–1372, 2014.
- Latronico N, Bolton C: Critical illness polyneuropathy and myopathy: A major cause of muscle weakness and paralysis. Lancet Neurol 10: 931–941, 2011.
- Guarneri B, Bertolini G, Latronico N: Long-term outcome in patients with critical illness myopathy of neuropathy: The Italian multicentre CRIMYNE study. J Neurol Neurosurg Psychiatry 79:838-841, 2008.
- Gwathmey KG, Burns TM, Collins MP, et al: Vasculitic neuropathies. Lancet Neurol 13:67–82, 2014.
