Case Review: Lupus Patient with Acute Disseminating Encephalomyelitis

Autoimmune workup revealed positive antinuclear antibody (ANA) (titer 1:640, speckled pattern). ANA subset serology identified positive SS-A, ribonucleoprotein and anti-Smith antibodies. Interestingly, double-stranded DNA (ds-DNA) and SS-B were negative (see Table 2, opposite). An antiphospholipid antibody panel was positive for elevated lupus anticoagulant, but it might have been falsely positive because the patient was already on anticoagulation with a direct thrombin inhibitor started a few weeks before this admission for DVT.

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Table 2: The Patient’s Autoimmune Evaluation

Urinalysis was positive for nephritic range proteinuria (protein in urine was 2.17 g/24 hr); hypoalbuminemia was noted (2.0 g/L) as well. Urine and serum electro­phoresis were also completed, and were negative for monoclonal protein. MRI of the cervical spine and a CT angiogram of the patient’s head and neck were also completed, and showed no signs of vasculitis.

The patient’s clinical situation remained unchanged: He was nonresponsive and had severe paresis on the left side of his body. Because the infectious workup was negative, the team discontinued antibiotics.

Based on these findings, the patient was diagnosed with new-onset SLE with CNS involvement. Both the ACR and the SLICC Criteria for the SLE classification were met, including six ACR criteria and eight SLICC Criteria: serositis, renal disorder, neurologic disorder, hematologic disorder, ANA and immunologic disorder (i.e., ANA, anti-Smith, antiphospholipid and low complement levels).

The patient was started on IV methylprednisolone 1 g daily for five days, followed by IV cyclophosphamide at 0.75 g/m2 body surface. Following IV methyl­prednisolone, oral prednisone 1 mg/kg was continued. The team added hydroxy­chloroquine 400 mg daily shortly after.

Soon after he received this regimen, the patient’s mental status improved dramatically. After one month of physical therapy, he was able to ambulate by himself, and he featured minimal paresis on his left lower extremity. Markers of inflammation decreased to normal, and proteinuria resolved.

After six monthly IV cyclophosphamide infusions, the patient is back to normal, with no residual weakness, and he is ready to resume his job. The oral prednisone will be slowly tapered and discontinued soon. The plan: Continue the monthly therapy with cyclophosphamide up to one year, and then decrease the infusion frequency to every three months.

SLE Discussion

SLE is a severe rheumatic disease. Neuropsychiatric symptoms occur in up to two-thirds of patients and include a wide range of manifestations, including headache, cerebrovascular events, cognitive dysfunction and psychosis, as well as even more serious presentations, such as neuropathies, myelitis and encephalomyelitis.^2,3 These symptoms can present alone or concomitantly with other system involvement.³