[Involvement of the peripheral nervous system in systemic connective tissue diseases: report on clinical cases].

BACKGROUND

The peripheral nervous system is usually involved in the majority of systemic connective tissue diseases, particularly in systemic lupus erythematosus, Sjögren's syndrome, vasculitis and systemic sclerosis. The pathogenesis of lesions in the peripheral nervous system associated with the autoimmune process is complex and it appears that two mechanisms, immunological and ischemic, are of greatest importance. Structures of the nervous system may be damaged by several autoantibodies (e.g. antineuronal, anti-nerve growth factor, anti-neurotrophins), by cytotoxic effects ofproinflammatory cytokines and by activated cells of the immune system. Local ischemia and hypoxia of neurons caused by inflammation of vasa nervosum represents the second significant mechanism leading to damage of nerve fibres in the peripheral nervous system.

METHODS

We present 3 cases with involvement of the peripheral nervous system as a dominant feature in the clinical picture of systemic connective tissue diseases. Clinical conditions in which the peripheral nervous system is involved include peripheral sensory and sensorimotor polyneuropathy, mononeuropathies, cranial neuropathies, acute inflammatory demyelinating polyneuropathy (Guillian-Barré syndrome), chronic inflammatory demyelinating polyneuropathy, plexopathy, myasthenia gravis, and dysfunctions of the autonomic nervous system. The diagnosis is based on clinical symptoms reported by the patient and disclosed during neurologic examination. The importance of electrophysiologic tests is advocated. Selection of treatment depends on the patient's clinical condition, as well as on the clinical form and type of disease. Treatment relies principally on glucocorticosteroids, intravenous immunoglobulins, cyclophosphamide, and other immunosuppressive drugs. Plasmapheresis and rituximab are administered in severe cases. Rehabilitation of the patient appears to be an important element of therapy.

CONCLUSIONS

Cases with neurologic symptoms as the first and often the sole manifestation of systemic connective tissue disease are particularly problematic requiring a multidimensional approach; their process of diagnosis and treatment is usually long.