Case 1: A 43-year-old woman with focal cerebral involvement in SLE
A 43-year-old woman, with a 1-year history of systemic lupus erythematosus (SLE) was admitted to the Hospital because of paresthesia in left arm and speech difficulties. Diagnosis of SLE had been made at the age of 42 years based on discoid lupus lesions, photosensitivity, arthritis, oral ulcers and detection of antinuclear, anti-dsDNA and anti-Ro/SS-A antibodies. Other anti-ENA and antiphospholipid antibodies (lupus anticoagulant and anticardiolipin antibodies) were negative at diagnosis. Relevant past history included: (i) Diagnosis of hypothyroidism due to autoimmune thyroiditis at the age of 22, treated with levothyroxine; (ii) two pregnancies at the ages of 29 (spontaneous abortion at Week 8) and 31 years (normal pregnancy). Lupus nephritis was diagnosed nine months later, and kidney biopsy disclosed a Class IV-S-A/C (Activity Index: 6/24; Chronicity Index:1/12). Methotrexate was discontinued and lupus nephritis induction of response therapy was started at Day Hospital with three daily pulses of methylprednisolone (1 g each) and low-dose pulse cyclophosphamide (‘Euro-lupus’ regimen). She was advised to continue on hydroxychloroquine (200 mg/day) and 30 mg/day of prednisone.
One week after the administration of the first pulse of cyclophosphamide (500 mg), the patient presented with numbness and paresthesia in left arm and hand as well as speech difficulties and was admitted at the Emergency Room. At physical examination, dysarthria and paresis of left upper limb was confirmed. Blood pressure was 160/95 mm Hg.
Recognising and treating focal cerebral involvement in SLE
Recognise and describe clinical, laboratory and imaging features, which help assess focal cerebral involvement in patients with SLE.
Explain the principles and strategies for the management of cerebral manifestations in SLE.
Demonstrate clinical awareness of potential severe cerebral complications in SLE
Learning Objectives Case 2: A woman with recurrent myelitis and SLE
A woman was diagnosed with systemic lupus erythematosus (SLE) at the age of 28 years old based on arthritis, skin erythema, mouth ulcers, sunlight hypersensitivity, ANF +, anti dsDNA + and anticardiolipin antibodies. At the age of 34 she developed papillitis of both eyes with vision loss. Her MRI revealed no abnormalities and she was treated with methylprednisone 500 mg for 5 days. At the age of 35 she developed a recurrence, no neuritis bulbaris was observed and SLE was thought to be the most likely cause so she was treated with cyclophosphamide 750 mg/kg for 6 months and prednisone 60 mg for 4 weeks and then 10 mg/kg lowering every 4 weeks. After 6 months she had completely improved. At the age of 37 she gradually developed, over a number of weeks, problems urinating and sensory disturbances left thigh and right leg, physical exam revealed hypesthesia in her left leg, and feelings of a different temperature in legs compared to arms.
MRI revealed transverse myelitis at C6-Th2, compatible with SLE and she was retreated with cyclophosphamide and prednisone (figure 1). Subsequently, she did quite well for a number of years under subsequent treatment with azathioprine and cyclosporin for joint and skin involvement. At the age of 52 she developed hypesthesia of the thorax and MRI revealed a myelitis over a long span Th2-Th10 while receiving cyclosporin. A laboratory test for aquaporin 4 antibodies was negative. Combination treatment with prednisone and rituximab followed by belimumab was given, to which she responded quite well. After 2 years she developed less strength in both legs, sensibility disturbance in both legs and specifically C9 and Th7 dermatome, new miction problems and inability to defecate. MRI revealed new lesions at TH4-TH8 while a sharp reduction of previous lesions was observed; aquaporin antibodies tested positive. Based on the recurrence, rituximab maintenance therapy was given every half year. In 2020 she started to suffer from recurrent infections and her IgG levels were 5.6 mg/ml after which intravenous IgG suppletion was started and the infectious problems disappeared.
Diagnosis, management and recurrence of myelitis in SLE
Describe the clinical presentation of myelitis and its recurrence
Explain treatment options of myelitis
Discuss the role of aquasporin-4 antibodies in myelitis
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