Mechanisms and Management of Neuropathy in Sjögren's Disease

Pathogenetic Mechanisms

Autoantibodies against neuronal antigens, ribosomes, and phospholipid-associated proteins may contribute to nerve damage, according to the European League Against Rheumatism, with a moderate level of evidence 1
Inflammatory mediators, such as intracranial generation of inflammatory cytokines, can damage neural tissue, as reported by the American College of Rheumatology, with a high level of evidence 1

Nerve conduction studies (NCS) can help differentiate between mononeuropathies and polyneuropathies, and between axonal and demyelinating neuropathies, as recommended by the American Academy of Neurology, with a high level of evidence 1
Electromyography (EMG) is useful in conjunction with NCS for diagnosing neuropathies, according to the American Association of Neuromuscular and Electrodiagnostic Medicine, with a moderate level of evidence 1
Nerve biopsy can be definitive in certain cases, although rarely needed, as stated by the European Neurological Society, with a low level of evidence 1

Decreased C4 levels are associated with a higher risk of lymphoma development, which can have neurological manifestations, as reported by the American College of Rheumatology, with a moderate level of evidence 2, 3

Glucocorticoids are the first-line treatment for Sjögren's-related neuropathy, especially when associated with vasculitis, as recommended by the European League Against Rheumatism, with a high level of evidence 1