Background SLE is a chronic autoimmune disease. Although NP events are well recognized in SLE, there is uncertainty regarding true prevalence of events, their attribution to SLE, their etiology and clinical significance. There are several NP manifestations of SLE other than seizures and psychosis. NP involvement is the indicator of disease severity and important determinant of quality of life in these patients.
Objectives (1) To study pattern of NP involvement in patients of SLE and its relation with disease activity; (2) To study the cognitive dysfunction in SLE and its relation to disease activity.
Methods This is an observational single center prospective study carried out at a tertiary care hospital from September 2010 to August 2013 in Mumbai.
Inclusion criteria – 60 Patients satisfying 4 or more ACR classification criteria for SLE were included. Patients involved in the study were subjected to detailed clinical history and drug history. All patients of SLE who had clinical neurological or psychiatric features were included in this study. The patients' demographic data, findings on general examination, neuro-psychiatric manifestations, results of laboratory investigations and imaging study were recorded. Blood, CSF and urine culture were done in relevant clinical set up. MRI and CT were used for brain imaging for NP event. ANA, dsDNA and anti Cardiolipin antibody were obtained for each patient. Cognitive assessment was done using Addenbroke's Cognitive Examination Scale 2005 (ACER). Patients were assessed under 6 cognitive domains i.e. attention, orientation, memory, verbal fluency, language and visuo-spatial processing. Screening was done for psychiatric symptoms using Brief Psychiatric Rating Scale. Symptom severity score for each symptom was done ranging from 1 (absence) to 7 (extremely severe). The mean Systemic Lupus Activity Measure (SLAM) score and mean SLICC damage index were determined in these patients.
Exclusion criteria – Patients with SLE who had NP manifestations secondary to infections, electrolyte imbalance, metabolic abnormalities or drugs were excluded from study.
Results Mean duration of disease in these patients was 20±(6-72) months and that of neurological illness was 15.86 months. Mean SLAM score and SLICC damage index revealed mild disease activity (SLAM 8.33) and minimal cumulative organ damage (SLICC DI 1.83), respectively. Out of 60 patients 30 patients (50%) had at least one NPS and 10 patients (16.67%) had more than one NP event. NP manifestations noted were anxiety, headache, seizures, depression, major psychosis, CVA, demyelination and neuropathy. In patients with Cognitive dysfunction (56.6%), cognitive domains affected were memory (100%), verbal fluency (88%), visuospatial functions (86%), attention and orientation (82%).
Conclusions NP syndromes in SLE have varied manifestations ranging from mild headache and anxiety to life threatening cerebrovascular accidents. Cognitive dysfunction is the most common neurological manifestation in patients with SLE, and is also recognized in patients without overt NP syndrome.
ACR Ad Hoc Committee on Neuropsychiatric Lupus Nomenclature. The American College of Rheumatology nomenclature and case definitions for neuropsychiatric lupus syndromes. Arthritis Rheum. 1999;42:599-608.
Hay EM, Black D, Huddy A et.al. Psychiatric disorder and cognitive impairment in systemic lupus erythematosus. Arthritis Rheum 1992;35:411-6.
Disclosure of Interest None declared