2 CASE HISTORY
A 20-year-old woman, who had fever and red rash on her face, and was admitted firstly on April 2016. Plasma antinuclear antibody (ANA), anti-dsDNA and proteinuria were increased. She was diagnosed as SLE and lupus nephropathy (type Ⅱ) by kidney biopsy. Oral methylprednisolone (MP, 40mg daily) and hydroxychloroquine (HCQ, 100mg, twice daily) were prescribed. The rash fade and the proteinuria completely relieved after 2 months treatments. She regularly decreased the dosage of MP. On August 2018, when the MP was reduced to 4mg daily, the rash on face reappeared. Two months later, her right eye presented with blurred vision. The blood tests indicated an elevated of ANA titer to 1:1280, anti-dsDNA concentration to 74.5 IU/ml. The blood IgG concentration was 18.1 g/L. The plasma C3 level slightly declined and proteinuria stayed normal. HCQ was stopped immediately and MP of 12mg daily was given. Although the rash soon improved, the patient had fever, and the visual impairment developed to both sides. The first cerebrospinal fluid (CSF) examination showed a normal tension of 170 mmH2O, with an increased protein concentration of 922 mg/L, a slightly elevation of IgG level and leukocyte count. Pathogen examinations including bacterial, fungal cultures and variety of viral antibodies were all negative in the CSF and blood tests. In brain MRI scan, the axial fluid attenuated inversion recovery (FLAIR) sequence showed hyper-intense in the right basal ganglia and bilateral peri-ventricular white matter, without abnormal enhancement (Figure 1 A-C). Ophthalmic examinations suggested retinal vasculitis in both eyes. The patient was identified as NPSLE with a disease activity score of 19 (brain damage, visual impairment, rash, and fever). Intravenous MP (500mg daily for 3 days) and cyclophosphamide (200mg every other day) were prescribed. Prednisolone of 45 mg daily was given subsequently.
However, the vision did not improve after intravenous MP combined with cyclophosphamide treatment. The second brain MRI scan showed that lesions of abnormal hyper-intense were increased in the right lateral temporal cortex, right thalamus bilateral basal ganglia, insular lobe external capsule, and peri-ventricular white matter on FLAIR images. Intravenous MP (500 mg daily for 3 days) were used again, and rituximab (500mg) was prescribed twice. Intrathecal injections of methotrexate and dexamethasone were performed weekly for a total of six times. Although the patient was treated with intensive therapy, the CSF examinations turned out to be a significantly increase of tension (218 mmH2O) and protein concentration (2187 mg/L), and area of brain lesions still expanded by MRI scan (Figure 1 D-F). The patient’s consciousness become worse accompanied with dysphagia.
Because prednisolone and immunosuppressive agents had no effects on retinal vasculitis and consciousness, protein A IAS (KONCHEN Biotech. Co., Ltd., Guangzhou, China) was performed. Three protein A IAS sessions were executed. Each session ran 6 circles and total volume of plasma adsorption was 3600 ml. Her consciousness got better soon after the first session. After the second session, examination of CSF showed a normal tension of 165mmH2O and a decrease of protein concentration to 547 mg/L. The brain lesions on MRI were found to be significantly reduced (Figure 1 G-I). Plasma IgG decreased significantly after each IAS sessions (Table 1).The protein A IAS was interrupted because of catheter related deep vein thrombosis after the third section. Two weeks later, the patient discharged with oral MP (40mg daily). Her consciousness and strength completely recovered after one month, but the vision has still not restored.