Peripheral nervous system lesion syndromes and the mechanisms of their formation in connective tissue diseases

Abstract

Systemic rheumatological diseases are often accompanied by the development of central and peripheral nervous system pathology. Data providing evidence of the high incidence of peripheral nervous system lesions in systemic lupus erythematosus and systemic scleroderma are presented. These diseases in particular are characterized by polyneuropathies and tunnel syndromes. Our own observations, along with published data, revealed the following major pathogenetic mechanisms of peripheral nervous system lesions in diffuse connective tissue diseases - ischemic, immunological, and metabolic. Consideration of these mechanisms will lead to pathogenetically based treatment and improved therapeutic outcomes.

Prevalence and Characteristics of Immunomediated Neuropathies in a Group of Patients With Autoimmune Diseases

“…2,3 They may be the form of presentation of connective tissue disorders and vasculitis, whereby their quick recognition alerts physicians to the potential compromise of vital organs. Their early diagnosis and treatment with immunosuppressors prevent severe and disabling sequelae.…”

Limited Scleroderma with Pauci-Immune Glomerulonephritis in the Presence of Renal Cell Carcinoma

“…Damage to the peripheral nerves typically involves a combination of axonal demyelination and lesions affecting the nerve fibers, causing a mixed neuropathy. 11 Damage occurs as a result of ischemia secondary to vasculitis of the vasa nervorum, and also from humoral and cell-mediated autoimmune destruction. 11 Renal cell carcinomas can also produce peripheral neuropathies through a paraneoplastic mechanism.…”

“…11 Damage occurs as a result of ischemia secondary to vasculitis of the vasa nervorum, and also from humoral and cell-mediated autoimmune destruction. 11 Renal cell carcinomas can also produce peripheral neuropathies through a paraneoplastic mechanism. However, this is unlikely in our patient since peripheral neuropathies associated with renal cell carcinoma tend to impact either the sensory or motor nervous system.…”

Unusual Peripheral Neuropathies. Part II: Intrinsic Reactive Causes

“…5 Curiously, the incidence drops to only 5% based on clinical criteria. 5,6 Peripheral neuropathy does not commonly accompany the initial manifestations of SLE. Peripheral neuropathy manifestations are variable, but commonly include a lateonset, symmetric distal axonal sensorimotor or sensory neuropathy.…”