Stroke in Young Female as a Presenting Feature of Systemic Lupus Erythematosus with Central Nervous System Vasculitis and Its Management

Abstract

Systemic lupus erythematosus (SLE) is a chronic, inflammatory, autoimmune disorder involving multiple systems. A 18-year-old female patient presented with complaints of headache, weakness, and tingling sensation in the left upper limb and lower limb. On magnetic resonance imaging (MRI), acute nonhemorrhagic infarct in right thalamo-capsular region was seen. The patient gave a history of malar rash and intermittent joint pains for which she had taken nonspecific treatment. Antineutrophilic antibody (ANA) by innumoflourescence and ANA blot was suggestive of SLE. Hence, the patient had central nervous system vasculitis and stroke due to SLE. The patient was started on antiplatelet therapy, tablet prednisolone 40 mg once daily, and tablet warfarin 5 mg once daily. The patient was readmitted after 2 weeks with menorrhagia and deranged INR. Tablet Warfarin was with-held, fresh frozen plasma transfusions were given. Repeat MRI did not show any new changes. Patient was given tablet nicoumalone and three doses of injection cyclophosphamide 800 mg. Hemiplegia has improved by 90%, her menstrual cycles are now regular.

INTRODUCTION

Systemic lupus erythematosus (SLE) is a chronic multisystemic autoimmune disorder with musculoskeletal and mucocutaneous systems commonly affected parts while also affecting cardiovascular, nervous, renal, and respiratory systems.

CASE REPORT

A 18-year-old female, presented to emergency with severe left temporal headache, tingling in left upper limb and lower limb. Five hours later, she developed left sided hemiparesis. The patient gave a history of rash (malar rash) on cheeks which was more prominent during menstrual phase and intermittent joint pains in small joints of fingers and wrist for the past 1 year for which the patient has taken symptomatic treatment. On examination, the patient had the pulse rate of 90 beats/min, blood pressure of 110 by 60 mm of mercury, and saturation of 98%. General examination: Pallor was present, no icterus, cyanosis, clubbing, edema, and lymphadenopathy. Central nervous system (CNS): Patient had normal tone, power of 4/5, exaggerated reflexes, and extensor plantar reflex on left side. Hence, a left hemiparesis was noted. There was no evidence of papilledema. Other systemic examination was normal.

Investigations

Hemoglobin – 9 g/dL, total leukocyte count – 13,500/µL (neutrophils – 80%, lymphocytes – 10%, and monocytes – 10%), platelet count – 278,000/µL, X-ray chest: no abnormality detected, electrocardiogram: normal sinus rhythm, Urea – 54 mg/dL, Creatinine – 0.9 mg/dL, Bilirubin total – 0.3 mg/dL, conjugated bilirubin – 0.1 mg/dL, unconjugated bilirubin – 0.2 mg/dL, alanine transaminase – 11 units/L, aspartate transaminase – 18 units/L, alkaline phosphatase – 61 units/L, and thyroid function test – normal.

Magnetic resonance imaging (MRI) of the brain shows hyperacute to acute infarct in the right thalamo-capsular region and acute lacunar infarct in the left parietal region [Figure 1].

Figure 1.

Magnetic resonance imaging of the brain with yellow arrow showing hyperacute to acute infarct in right thalamo-capsular region and acute lacunar infarct in the left parietal region

Antineutrophilic antibody (ANA) by immunefluorescence: 1:320 (nucleoplasm speckled pattern), ANA blot: positive for DS-DNA, nucleosome and KU factor; Lupus anticoagulant: weak positive; cardiolipin antibody and beta 2-glycoprotein were negative. The patient was started on injection enoxaparin 0.4 mL once daily, tablet warfarin 5 mg once daily and tablet aspirin 75 mg, tablet clopidogrel 75 mg once daily, tablet atorvastatin 40 mg once daily, tablet prednisolone 40 mg once daily. Neurological deficit improved significantly. The patient was discharged on tablet warfarin 5 mg once daily and prednisolone 40 mg and aspirin 75 mg once daily. The patient was readmitted 2 weeks later for menorrhagia. The patient was given fresh-frozen plasma transfusion. Warfarin was discontinued. On clinical examination, power was normal in all four limbs. Repeat MRI did not show any new findings. On advice from rheumatologist, three doses of injection cyclophosphamide dose 800 mg were administered. The patient was discharged on tablet aspirin 75 mg once daily, tablet nicoumalone 2 mg once daily, and tablet prednisolone 40 mg once daily. There have been no more episodes of menorrhagia or neurological symptoms.

DISCUSSION

Cerebral lupus is diagnosed by clinical condition, serological autoimmune assay, and neuroimaging which may show thickening of vessel wall, stenosis, ischemic, or hemorrhagic lesion.[1] Inflammation promotes thrombosis and hence increased risk of stroke, especially in patients with generalized severity of disease.[2] There is the high risk of small vessel cerebral vascular disease, mechanism being endothelial injury, increased neutrophil activation, trap formation, and oxidative stress.[3] As a result, there is increased risk of ishemic stroke due to reduced cerebral perfusion and increased risk of hemorrhagic stroke due to weakening of the vessel walls.[4] Interferon-1 causes endothelial damage and leads to vasculitis.[5] Systemic inflammation causes deranged lipid profile, thereby explaining the progression of atherosclerotic cardiovascular disease. Early diagnosis by using European Alliance of Association for Rheumatology or American College of Rheumatology and management is necessary to reduce the mortality of disease.[6] Cerebral lupus mainstay of treatment includes long-term anticoagulation, high-dose steroid therapy, and immunoregulation by intravenous cyclophosphamide which reduces circulating auto-antibodies and prevents organ damage.[7]

CONCLUSION

Our patient had the symptoms of SLE, which were not diagnosed till a catastrophic presentation of stroke occurred. Anticoagulation in young girls always poses a risk of menorrhagia. Hence, close monitoring and follow-up are essential in the management of young females of SLE with CNS vasculitis.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed.

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.