Characteristics of 10 female patients with neuropsychiatric systemic lupus erythaematosus at study entry
| Patient | Age(years) | Duration of disease | Previous treatment | NP classification | MRI/SPECT | IgG index /IL6 (pg/ml) | Clinical manifestations | SLEDAI | |||
|---|---|---|---|---|---|---|---|---|---|---|---|
| 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). | |||||||||||
| 1 | 35 | 19 years | CS (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, psychosis | Normal/abnormal | Not done/not done | Fever, fatigue, nephritic syndrome, leukopenia, low Hb, high ESR, CH50, anti-ds DNA↑ | 49 | |||
| 2 | 55 | 25 years | CS (40 mg, pulse 3), IV-CY (7), PE (2) | Acute confusional state | II, III/abnormal | 0.73↑/ 1.8 | Paresthesia of fingers, severe AIHA, anti-ds DNA↑ | 2 | |||
| 3 | 46 | 3 months | CS (50 mg), IV-CY (1), PE (2), IA (3) | Acute confusional state, seizure | II, III/abnormal | 0.46/33.8↑ | Leukopenia, low Hb, thrombocytopenia, proteinuria, AIH, anti-ds DNA↑ | 37 | |||
| 4 | 20 | 1 year | CS (50 mg), CsA (175 mg, 1 m) | Headache | Normal/not done | 1.05↑/3.1 | Fever, fatigue, skin rash, alopecia, cardiomyopathy, polyneuropathy, leukopenia, C4↓, anti-ds DNA↑ | 16 | |||
| 5 | 34 | 3 years | CS (60 mg), IV-CY (8), MZ (150 mg, 25 months) | Demyelinating syndrome | II, III/normal | 0.85↑/0.9 | Sensory deficit, photosensitivity, mouth ulcer, lymphocytopenia, C4↓ | 16 | |||
| 6 | 30 | 22 years | CS (40 mg), MZ (150 mg, 22 years) | Mood disorder | Normal/abnormal | 0.54/1.5 | Polyneuropathy, muscular pain, skin rash, leukopenia, anti-ds DNA↑ | 17 | |||
| 7 | 21 | 7 years | CS (60 mg, pulse 3), IV-CY (14), MTX (intrathecal 30 mg), MZ (300 mg, 2 years) | Myelopathy, mood disorder, anxiety disorder | II, III/abnormal | 0.80↑/4.7 | Periungual erythaema, leukopenia | 3 | |||
| 8 | 20 | 9 months | CS (45 mg), IV-CY (6), AZ (50 mg, 1 month) | Psychosis, cognitive dysfunction | III/abnormal | 0.56/1.0 | Lymphadenopathy, alopecia, malar rash, lymphocytopenia | 18 | |||
| 9 | 20 | 8 months | CS (60 mg, pulse 3), IV-CY, DFPP (4) | Acute confusional state, psychosis | III/abnormal | 0.98↑/4.2 | Fever, lymphadenopathy, low Hb, lymphocytopenia, high ESR, anti-Sm↑ | 28 | |||
| 10 | 29 | 17 years | CS (40 mg, pulse 2), AZ (100 mg, ly), CsA (300 mg, 1 month), IV-CY (2), PE (4) | Acute confusional state, psychosis | Normal/abnormal | 0.60/2.4 | Severe AIHA, CH50↓ | 18 | |||