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.