An 11-year-old girl was admitted to our hospital because of recurrent rashes for half a year and high fever for 2 weeks. The patient often complained of knee and instep pain in the past few years, and she had acute pancreatitis twice in the past year. She had no history or family history of autoimmune disease, cancer, or tuberculosis.
The patient presented with fever, an ulcer on the lip, alopecia, malar rash, and rashes on the limbs. The initial laboratory results revealed leukopenia (white blood cell count 2.02 x 109/L, lymphocyte count 0.70 x 109/L), autoimmune hemolysis (hemoglobin 79 g/L), low C3 [0.26 g/L (normal range 0.9-1.8 g/L)], low C4 [0.04 g/L (normal range 0.1-0.4 g/L)], and antibody abnormalities. The antinuclear antibody was positive with a titer of 1:160; the anti-double-stranded DNA and anti-Smith antibodies were positive. Besides, laboratory data on admission also showed hyperferritinemia (ferritin > 1,500 mug/L), hypertriglyceridemia (4.87 mmol/L), and elevated levels of aspartate aminotransferase (123 U/L) and lactate dehydrogenase (621 U/L). The fibrinogen level (2.75 g/L) and urinalysis were normal. Cerebrospinal fluid and bone marrow examination on day 2 showed no infection or macrophages phagocytosing hematopoietic cells. Cerebral magnetic resonance imaging (MRI) was taken on day 4, revealing T2-white matte hyperintensities in her left frontal lobe and left lentiform nucleus (Figure 1). The presence of clinical and laboratory criteria led to the diagnosis of SLE and SLE-associated MAS.
The patient suffered from high fever twice a day after admission. Glucocorticoid therapy was started on day 5, with intravenous methylprednisolone 2 mg/kg/day. The next day her temperature returned to normal.
On day 7 after admission the patient appeared weak and ill, then the next day she exhibited dizziness, drowsiness, apathia, and dysarthria, and her temperature reached 37.9 C. The patient was given high-dose methylprednisolone pulse therapy (30 mg/kg/day) for 3 days. On day 9 the second cerebral MRI showed rapid progress (Figure 2), with multi-focal abnormal signals mainly involving white matter (bilateral temporal, cerebellum, insular lobes, and basal ganglias; left frontal and parietal lobes; right corpus callosum). The signals were hyperintense on fluid-attenuated inversion recovery (FLAIR). Cyclophosphamide (8.5 mg/kg/day for 2 days) and intravenous immunoglobulin (2 g/kg) were initiated in the therapy. On day 10 the patient showed significant improvements in all clinical conditions, including the rashes. On day 13 the third MRI demonstrated small infarcts in the left lentiform nucleus, left frontal lobe, temporal lobe, insular lobe, parietal lobe, and right corpus callosum, revealed by decrease in apparent diffusion coefficient in the gray matter, and an increased swelling in the surrounding white matter (Figure 3). She was given intrathecal dexamethasone once a week for three times. The second pulse of high-dose methylprednisolone was administered 1 week after the first pulse. Cyclophosphamide and immunoglobulin were administered with an interval of 2 weeks. On day 25 she had a fourth MRI scan and achieved neuroimaging improvements (Figure 4). The patient was discharged on day 30, with prednisone and addition of hydroxychloroquine.