Immunopathogenesis of Guillain-Barré Syndrome

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) shares many similarities with GBS but also important differences. For example, the autoimmune attack of CIDP is chronic, not self-limiting as with GBS. Furthermore, CIDP does not seem to follow an otherwise trivial infection. In CIDP, similar to GBS, T cells, antibodies, macrophages, and complement work together to induce an immune attack on peripheral nerve elements. On nerve biopsy, macrophages are found around endoneurial vessels.

The pathophysiology of vasculitis, on the other hand, is much different than of GBS and CIDP, in large part because what are being attacked in vasculitis are not peripheral nerve epitopes but rather blood vessels, causing secondary ischemic injury to nerve in cases involving blood vessels of nerve. The inflammation of vasculitis may affect blood vessels of any size anywhere in the body, and many forms of vasculitis affect the peripheral nervous system. When peripheral nerve vasculitis occurs, nerve axons are principally damaged, resulting in an “axonal” neuropathy, in contrast to the demyelinating neuropathies of AIDP and CIDP. Vasculitis may occur as a primary process or as a secondary phenomenon that is related to a variety of disorders, ranging from rheumatologic disorders to viral infections. The pathologic changes of nerve large arteriole vasculitides are seen in epineurial and perineurial vessels measuring 75 to 200 microns in diameter. Examples of nerve large arteriole vasculitis include polyarteritis nodosa and Wegener granulomatosis. Nerve “microvasculitis” principally involves the smallest arterioles (<40 µm), microvessels, and venules of the epineurium, although there is some overlap in vessel size with the large nerve arteriole vasculitides. Nerve microvasculitis occurs in classic nonsystemic vasculitic neuropathy (classic NSVN), classic Sjögren syndrome, many virus-associated vasculitic neuropathies, and diabetic lumbosacral radiculoplexus neuropathy (DLRPN).

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