Background Neuropsychiatric systemic lupus erythematosus (NPSLE) comprises of various neurological and psychiatric conditions in patients with SLE. The frequencies, clinical manifestations and prognosis may vary significantly among different disease entities.
Methods Clinical records of 431 Chinese SLE patients were reviewed. Manifestations of NPSLE were defined based on the 1999 American College of Rheumatology (ACR) nomenclature.1 ‘Common’ neuropsychiatric events (anxiety, headache, mild depression, mild cognitive dysfunction, and polyneuropathy without electrophysiological confirmation) described by the Ainiala’s criteria were excluded.2 Other neuropsychiatric evens in the absence of alternative contributing factors were attributed to SLE. The frequencies of different NPSLE manifestations were reported. Clinical features of patients with central nervous system (CNS) diseases and peripheral nervous system (PNS) diseases were described and compared.
Results Among 431 Chinese SLE patients, 396 (91.9%) were female and the median age of SLE onset was 26 (IQR 15) years. NPSLE occurred in 88 (20.4%) patients, including 77 and 11 patients with CNS and PNS diseases, respectively. The most frequent manifestations were mood disorder (26/431; 6.0%), seizure disorder (15/431; 3.5%), acute confusion (11/431; 2.6%), mononeuritis (7/431; 1.6%), and myelopathy (6/431; 1.4%). The median time from SLE diagnosis to onset of NPSLE was 6 (IQR 16) years and 10 (IQR 7.5) years for CNS and PNS diseases, respectively. Compared to CNS diseases, patients with PNS diseases had older age at SLE diagnosis (PNS 39 years vs CNS 25 years, p=0.04) and older age at NPSLE onset (PNS 47 years vs CNS 34 years, p=0.02). The 10-year survival rates were 96.1% and 100% for CNS and PNS diseases, respectively.
Conclusions PNS diseases are important manifestations in patients with long-standing SLE, especially in patients with older age at SLE diagnosis. The outcome is favorable compared with patients with CNS diseases. Prospective studies are needed to confirm these findings.
The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum 1999;42(4):599–608.
Ainiala H, Hietaharju A, Loukkola J, Peltola J, Korpela M, Metsanoja R, Auvinen A, Validity of the new American College of Rheumatology criteria for neuropsychiatric lupus syndromes: a population-based evaluation. Arthritis Rheum 2001;45(5):419–23.
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