Increased intrathecal immunoglobulin synthesis of both kappa and lambda types in patients with systemic lupus erythematosus and central nervous system involvement.
The concentration of immunoglobulins in paired serum and cerebrospinal fluid (CSF) specimens from 15 patients with systemic lupus erythematosus (SLE) and central nervous system (CNS) involvement (CNS-SLE) were determined based on their light chain properties. Eight patients with SLE without CNS involvement and 20 patients with noninflammatory neurological diseases were also studied for comparison. We found that both of the CSF Ig-k (immunoglobulin kappa) and Ig-1 (immunoglobulin lambda) indices were significantly elevated, indicating the increased intrathecal immunoglobulin synthesis of both kappa and lambda types in patients with CNS-SLE. Moreover, the CSF Ig-k and Ig-1 indices correlated well with each other, and both indices were found to decrease remarkably when the CNS manifestations subsided after successful treatment. In 4 of 15 patients, intrathecal synthesis of free light chains in addition to Ig bound light chains seemed to be increased. These observations provide substantial evidence for polyclonal B lymphocyte activation within the CNS.
D000293:Adolescent; D000328:Adult; D001402:B-Lymphocytes / Q000276:immunology; D002493:Central Nervous System Diseases / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology*; D005260:Female; D006801:Humans; D007145:Immunoglobulin kappa-Chains / Q000134:cerebrospinal fluid*; D007146:Immunoglobulin lambda-Chains / Q000134:cerebrospinal fluid*; D008180:Lupus Erythematosus, Systemic / Q000134:cerebrospinal fluid / Q000150:complications / Q000276:immunology*; D008213:Lymphocyte Activation; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use
D007145:Immunoglobulin kappa-Chains; D007146:Immunoglobulin lambda-Chains; D011239:Prednisolone