Dong Y

Clinical features and outcome of neuropsychiatric lupus in Chinese: analysis of 240 hospitalized patients

Neuropsychiatric (NP) events are severe manifestations of systemic lupus erythematosus (SLE) and relate to poor outcome. The aims of this study are to investigate the NP manifestations of SLE and to identify the predictive factors for clinical outcome. There was a retrospective review of 240 hospital patients with primary NP events of SLE (NPSLE) from 1990 to 2004. Neuropsychiatric manifestations, SLE disease activity index (SLEDAI) score, System lupus International Collaborating Clinic/American College of Rheumatology Damage Index (SLICC/ACR-DI) score, magnetic resonance imaging (MRI) findings, treatment and mortality rate were included for analysis. From this group of patients, 15 NP syndromes were identified. The most frequent manifestation was headache, followed by seizure. The mean SLEDAI and SLICC/ACR-DI scores were 19.9 ± 6.9 and 3.5 ± 1.6, respectively. Abnormal MRI features were found in 67% (61/91) patients. At least one intrathecal (IT) injection of methotrexate (MTX) plus dexamethasone (DXM) was administered to 109 (45.4%) patients. High dose (1 g) intravenous methylprednisolone pulse therapy (IVMP) was administered to 167 (69.5%) patients. Multifactor analysis revealed that high SLICC/ACR-DI scores and sets of concurrent NP symptoms were independently associated with poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM were protective factors against poor outcome. From our data, NPSLE is heterogeneous and is usually associated with high disease activity and organ damage scores. High SLICC/ACR-DI score and having more than two sets of NP symptoms are the predictors for poor outcome, whereas pulse IVMP and IT injection of MTX plus DXM can improve the prognosis. Lupus (2008) 17, 93—99.

Antineutrophil cytoplasmic antibody-associated vasculitis complicated with diffuse alveolar hemorrhage: a study of 12 cases.

Objective:To summarize the clinical features and therapeutic response of patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis complicated with diffuse alveolar hemorrhage (DAH). Method:A retrospective chart review of the patients having ANCA-associated vasculitis with DAH was made with regard to their clinical symptoms, laboratory test results, responses to therapy and outcomes. Results:During January 1994 to June 2007, 131 ANCA-associated vasculitis patients were admitted to Peking Union Medical College Hospital. During this period, 12 of these cases consisting of 9 males and 3 females with a mean age of 59.9 ± 16.7 years developed DAH. The mean duration of vasculitis before the onset of DAH was 9.0 ± 14.3 months (range: 0–48 months). Dyspnea was the most consistent presenting symptom, while frank hemoptysis occurred in only 5 cases at the onset of DAH. The most common extrapulmonary findings associated with DAH were glomerulonephritis (100%). Symptoms including fever and arthralgia-myalgia (91.7%) as well as complications involving gastrointestinal (41.7%), mucocutaneous (25%), ear-nose-throat (25%), cardiovascular (16.7%), and nervous system (16.7%) were also frequently seen. The Birmingham Vasculitis Activity Score at the onset of DAH was 21.8 ± 4.9. All patients were treated with systemic corticosteroids combined with cyclophosphamide including methylprednisolone pulse therapy in 7 patients. In addition, plasmapheresis (41.7%), dialysis (25%), and mechanical ventilation (41.7%) were applied. The overall mortality rate was 58.3% (7 patients). Three patients died of fulminant DAH and respiratory failure during the first week of treatment. The other 4 patients died of septic shock (2 patients), severe heart failure (1 patient), and systemic fungus infection with septic shock (1 patient) 10 to 32 days after the onset of DAH. Conclusion:DAH is the most serious complication of ANCA-associated vasculitis. The episode of DAH always occurs simultaneously with multiple system involvement. The most constant signs are newly developed dyspnea and new infiltration of bilateral lungs. Prompt bronchoalveolar lavage can be helpful for timely diagnosis of the patients without overt hemoptysis and a useful tool to exclude pulmonary infection.

Cell-ELISA detection of antineuronal antibodies in central nervous system involvement in systemic lupus erythematosus

Objective: To develop a cell-ELISA method to detect antineuronal antibodies (anti-Ns) and evaluate the diagnostic value of anti-Ns in central nervous system involvement in systemic lupus erythematosus (CNS-SLE). Method: Anti-N was assessed in both serum and cerebrospinal fluid (CSF) samples from 38 patients with CNS-SLE, 29 with SLE without CNS involvement (non-CNS-SLE), 36 with other rheumatic diseases and 59 with non-rheumatic diseases with the CNS manifestations using a cell-ELISA method with 1% paraformaldehyde-fixed SK-N-MC neuroblastoma cells as substrate. Serum samples from 37 healthy donors were also included in this study. Patients with CNS-SLE who were anti-N positive in CSF were studied serially for CSF anti-N levels at times of treatment-associated improvement in CNS symptoms. Results: Serum anti-N levels were significantly increased in patients with SLE compared with other groups, with a sensitivity of 61.2% (41/67) and a specificity of 91.8% (p<0.001). CSF anti-N levels were significantly increased in patients with CNS-SLE, with a sensitivity of 47.4% (18/38) and a specificity of 89.7%, whereas only 10.3% (3/29) of patients with non-CNS-SLE had increased anti-N in CSF (p<0.001). CSF anti-N levels decreased significantly after effective treatment of CNS-SLE (p<0.05). Conclusion: Serum anti-N is relatively specific to SLE. CSF anti-N is a sensitive and relatively specific antibody in diagnosing CNS-SLE and correlates with CNS-SLE activity.