Vasculitic neuropathy in rheumatoid disease and Sjögren syndrome

Abstract

Two patients with rheumatoid arthritis and one with Sjögren syndrome had a severe sensorimotor neuropathy preceding or up to 5 years after the onset of the disease. Electrophysiologic and sural nerve biopsy studies revealed an axonal neuropathy. Myelinated fibers were affected to a greater extent than unmyelinated axons. Peripheral nerve damage was related to occlusion of the vasa nervorum, since vasculitic involvement of epineurial vessels was observed in all patients. Despite the severity of the neuropathy, it may recover, because, compared with axons, Schwann cells are perhaps less vulnerable to ischemia.

Central and peripheral neurological complications of primary Sjögren's syndrome

“…A vasculitis pathogenesis was advanced for both pSS mononeuritis multiplex and axonal sensory-motor pSS-related neuropathies [1,13,18]. By contrast, the pathogenesis of sensory neuronopathies is related to lymphocyte infiltration into the dorsal root ganglia without any vasculitic process [1,13].…”

Intravenous immunoglobulin treatment for painful sensory neuropathy associated with Sjögren's syndrome

“…Various forms of peripheral neuropathy have been reported to be associated with Sjögren's syndrome, including sensory ataxic neuropathy, painful sensory neuropathy without sensory ataxia, trigeminal neuropathy, multiple mononeuropathy, multiple cranial neuropathy, radiculoneuropathy, and autonomic neuropathy with anhidrosis [1][2][3][4][5][6][7]. The presence of such a diverse array of neuropathic states suggests that multiple mechanisms are involved in the pathogenesis of neuropathy associated with Sjögren's syndrome.…”

Is there specific neurological disorders of primary Sjögren's syndrome?

“…The mechanisms of these disorders are not yet clearly defined. A vasculitis could cause mononeuritis multiplex and sensory-motor polyneuropahties [8,9]. Regarding mononeuropahty, as we have seen, she's secondary to infiltration of dorsal root ganglia and sensory neurons apoptosis [10].…”

“…Diagnosis is based on histological analysis of dorsal root ganglia, although, because of its invasive nature, this examination is not performed in daily routine. Histological analysis shows apoptosis of sensory neurons, mononuclear cell infiltrate (mostly T cells) surrounding nerve fibers and blood vessels, and diffuse inflammation of the lymph node with nodules of Nageotte [8,9]. Sensory neuronopathy can also be seen in paraneoplastic syndrome and sometimes in HIV infection [10].…”