Abstract
Peripheral nervous system disease is a common and often debilitating feature of many
systemic rheumatologic disorders. Such involvement takes many forms, reflecting the
variety of underlying pathophysiology, though most patients present with painful multifocal
neuropathy (usually vasculitic) or a distal sensory more than motor peripheral neuropathy
(sometimes vasculitic and nearly always axonal). The presence of peripheral nervous
system involvement is often an early signal of the generalization of inflammatory
disease in blood vessels or extravascular tissues, though peripheral neuropathy is
not itself an independent predictor of mortality. Nonetheless, progressive multifocal
neuropathy, motor neuropathy, small fiber neuropathy, and sensory neuronopathy should
be treated early and aggressively with immunosuppression (or the gluten-free diet
in appropriate situations) to limit morbidity. Given the rapidly evolving therapeutic
landscape, partnership with a rheumatologist is essential. Treatment is usually sustained
for 1 to 2 years, and remission is possible in many cases within 6 to 12 months, with
variable rates of relapse and treatment resistance. Patients should be meticulously
monitored for relapse with serial laboratory testing, electrodiagnostic studies, and
clinical examination. Functional rating scores, such as the neuropathy impairment
scale and the total neuropathy score are useful for longitudinal assessment.
Keywords
vasculitis - rheumatologic - neuropathy - celiac - fibromyalgia
