A neuropathy is a derangement in the function and structure of peripheral motor, sensory, or autonomic neurons. Autoimmune disorders are one of the disease categories causing neuropathy. In many cases, evidence supports autoimmune pathogenesis. Demonstration of the relationships between specific neuro pathic syndromes and antibodies directed against glycolipid and neural antigens are important scientific advances.

In the autoimmune neuropathies, antibodies directed against peripheral nerve components are associated with specific clinical syndromes (Table 1). Knowledge of these syndromes and antibody tests can be used to identify a treat able neuropathy. In addition, many autoimmune neuropathic syndromes are associated with malignancies, which they often precede. Recognition of these syndromes can lead to early identification and treatment.

Table1. Neuropathy Syndromes Associated With Antibodies Directed Against Peripheral Nerve Components

Most antibodies implicated in the development of autoimmune-mediated neuropathies are directed against carbo hydrate epitopes of glycoproteins or glycolipids. Glycolipids are concentrated in neural membranes, in which the lipid portion is immersed in the membrane bilayer and the carbohydrate portion is exposed extracellularly. The extracellular domain of the carbo hydrate epitopes makes them vulnerable to antibody binding.

Systemic sclerosis (scleroderma) is an autoimmune disease characterized by a wide spectrum of clinical, pathologic, and serologic abnormalities. More than 90% of patients with systemic sclerosis spontaneously produce ANA. The structure and function of the intracellular antigens to which these ANAs are directed have been characterized. These serum autoantibodies are helpful markers because they correlate with certain clinical features of systemic sclerosis (Table 2). A more recently developed marker autoantibody, anti–RNA polymerase III anti body, has been identified in many patients who have systemic sclerosis with diffuse or extensive cutaneous involvement.

Table2. Clinical Types of Systemic Sclerosis (SSc) and Associated Antibody Markers

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