Nervous system involvement of connective tissue disease: mechanisms and diagnostic approach

Connective tissue diseases can present with a wide spectrum of neurological symptoms. The present review summarizes the neurological involvement in connective tissue diseases and highlights recent findings on the diagnosis of neurological manifestations and potential underlying mechanisms.

The wide range in the prevalence of neurological manifestations in systemic lupus erythematosus (SLE) and Sjögren's syndrome reveals the necessity of clear decision algorithms for the association of neurological symptoms with a systemic autoimmune disease. For this purpose new recommendations for the diagnosis and treatment of neuropsychiatric SLE have been published. Recent studies identified potential paraclinical markers for the diagnosis of nervous system involvement in connective tissue diseases. Antiaquaporin-4 antibodies occur highly specifically in those patients with connective tissue diseases that present with symptoms typical for neuromyelitis optica.

To date there is no specific marker available to prove neurological manifestation of connective tissue diseases. However, some progress has been achieved in characterizing typical clinical features and potential disease associated autoantibodies, which may lead to a better management of these patients.