Peripheral nervous system manifestations of rheumatological diseases.

Rheumatological diseases result in immune-mediated injury to not only connective tissue, but often components of the peripheral nervous system. These overlap conditions can be broadly categorized as peripheral neuropathies and overlap myositis. The peripheral neuropathies are distinctive as many have unusual presentations such as non-length-dependent, small fiber neuropathies and sensory neuronopathies (both due to dorsal root ganglia dysfunction), multiple mononeuropathies (e.g. vasculitic neuropathies), and even cranial neuropathies. Overlap myositis is increasingly recognized and is often associated with specific autoantibodies. Sarcoidosis also has widespread neurological manifestations and impacts both the peripheral nerves and muscle. Much work is needed to fully characterize the vast presentations of these overlap diseases. Given the rarity of these disorders, they are understudied, resulting in significant knowledge gaps with regards to their underlying pathophysiology and the best treatment approach. A basic knowledge of these disorders is mandatory for both practicing rheumatologists and neurologists as prompt recognition and early initiation of immunotherapy may prevent significant morbidity and permanent disability.

Vasculitic Neuropathies.

Vasculitic neuropathies are disorders that result from inflammation in the peripheral nerves' vascular supply, resulting in ischemic injury. These disorders may be a result of systemic inflammation or may be confined to the peripheral nervous system. Causative etiologies include primary systemic vasculitis, vasculitis secondary to other conditions such as primary connective tissue disorders, infectious, paraneoplastic, and drug-induced conditions, and nonsystemic vasculitic neuropathy. Early recognition and treatment of these conditions is imperative to prevent substantial morbidity and mortality. The goal of this review is to provide an organization of the vasculitic neuropathies and an overview of principles of diagnosis and treatment for the clinical neurologist.