Table 1

 Characteristics of 10 female patients with neuropsychiatric systemic lupus erythaematosus at study entry

PatientAge(years)Duration of diseasePrevious treatmentNP classificationMRI/SPECTIgG index /IL6 (pg/ml)Clinical manifestationsSLEDAI
The disease activity was high in all patients and none had responded to conventional immunosuppressants.
AIHA, autoimmune haemolytic anaemia; AZ, azathioprine; CS, corticosteroid; CsA, cyclosporine; CY, cyclophosphamide; DFPP, double filtration plasmapheresis; ESR, erythrocyte sedimentation rate; Hb, haemoglobin; IA, immunoadsorption; MTX, methotrexate; MZ, mizoribine; PE, plasma exchange; SLE-DAI, Systemic Lupus Erythaematosus Disease Activity Index; VCR, vincristine.
For IV-CY, PE and IA, numbers in parentheses represent the number of treatments. For CS, CsA, AZ and MZ, the doses in parentheses represent maximum dosage. For VCR in patient 1 and MTX in patient 7, the dose in parentheses expresses total dosage. MRI finding: II, small areas of increased signal intensity secondary to microinfarctions; III, focal areas of signal intensity in grey matter (Am J Roentgenol 1985;144:1027–31).
13519 yearsCS (40 mg, pulse 14), IV-CY (22), VCR (10 mg), CsA (300 mg, 3 years), AZ (100 mg, 2 months), MTX (8 mg/w, 4 months), PE (11), IA (15)Acute confusional state, seizure, psychosisNormal/abnormalNot done/not doneFever, fatigue, nephritic syndrome, leukopenia, low Hb, high ESR, CH50, anti-ds DNA↑49
25525 yearsCS (40 mg, pulse 3), IV-CY (7), PE (2)Acute confusional stateII, III/abnormal0.73↑/ 1.8Paresthesia of fingers, severe AIHA, anti-ds DNA↑2
3463 monthsCS (50 mg), IV-CY (1), PE (2), IA (3)Acute confusional state, seizureII, III/abnormal0.46/33.8↑Leukopenia, low Hb, thrombocytopenia, proteinuria, AIH, anti-ds DNA↑37
4201 yearCS (50 mg), CsA (175 mg, 1 m)HeadacheNormal/not done1.05↑/3.1Fever, fatigue, skin rash, alopecia, cardiomyopathy, polyneuropathy, leukopenia, C4↓, anti-ds DNA↑16
5343 yearsCS (60 mg), IV-CY (8), MZ (150 mg, 25 months)Demyelinating syndromeII, III/normal0.85↑/0.9Sensory deficit, photosensitivity, mouth ulcer, lymphocytopenia, C4↓16
63022 yearsCS (40 mg), MZ (150 mg, 22 years)Mood disorderNormal/abnormal0.54/1.5Polyneuropathy, muscular pain, skin rash, leukopenia, anti-ds DNA↑17
7217 yearsCS (60 mg, pulse 3), IV-CY (14), MTX (intrathecal 30 mg), MZ (300 mg, 2 years)Myelopathy, mood disorder, anxiety disorderII, III/abnormal0.80↑/4.7Periungual erythaema, leukopenia3
8209 monthsCS (45 mg), IV-CY (6), AZ (50 mg, 1 month)Psychosis, cognitive dysfunctionIII/abnormal0.56/1.0Lymphadenopathy, alopecia, malar rash, lymphocytopenia18
9208 monthsCS (60 mg, pulse 3), IV-CY, DFPP (4)Acute confusional state, psychosisIII/abnormal0.98↑/4.2Fever, lymphadenopathy, low Hb, lymphocytopenia, high ESR, anti-Sm↑28
102917 yearsCS (40 mg, pulse 2), AZ (100 mg, ly), CsA (300 mg, 1 month), IV-CY (2), PE (4)Acute confusional state, psychosisNormal/abnormal0.60/2.4Severe AIHA, CH50↓18