INTRODUCTION age of onset for SLE is between 20

INTRODUCTION

Systemic lupus erythematosus (SLE)
is a heterogenous autoimmune disease that involves multi organ.¹ It can affect
skin, joint, lung, kidneys, nervous system and other organ of the body. The name
itself describes the disease in which systemic means the disease can affect any
organ and tissue in the body. Lupus came from a Latin word which refers to the
rash that extend across nose bridge and cheek bones, meanwhile erythematous
came from a Greek word for red which was the colour of the rash.² It is much more
common in women compared to men. The peak age of onset for SLE is between 20
and 40 years old.³  The flares of SLE are
episodic and varied in severity as well as manifestation.?

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CASE REPORT

A 16 year old Malay girl with newly
diagnosed Systemic Lupus Erythematous (SLE) presented with worsening headache
for 3 days and multiple fitting episodes on the day of admission. The headache
localized at the centre of the head and it was throbbing in nature. It was
associated with blurring of vision, aggravated by movement and relieved by pain
killer. She scored the pain as 9/10, and reduced to 5/10 after taking oral pain
killer. She also complained of painful swelling of left side of face for 3
days. She also had multiple episodes of seizure which lasted than one minute
each. It was associated with stiffness of all four limbs with no jerky
movements. There was no drooling of saliva, tongue biting or uprolling eye ball.
During the seizure, she was unresponsive to call and touch. Post ictally, she
remained unresponsive for about 5 minutes before started to answer questions.
There was no muscle weakness of upper or lower limb. She could not recall what
happened pre-ictally but claimed she had her regular meal around 30 minutes
before the first episode. Otherwise, there was no joint stiffness, morning
stiffness, cough, fever, frothy urine, blood in urine, slurred speech, loss of consciousness,
body weakness or numbness.

On further questioning, she actually
had been having severe persistent headache and intermittent fever since 1 year
ago. The nature of the headache was the same as the current presentation.
However, the pain scores for all that time was 7/10. The fever was intermittent
and temporarily relived by paracetamol. She had been having fever at least once
a week for the past 1 year. Her study was very much affected by this as she had
to take lots of medical leave. Besides, she also had significant hair loss,
rashes over the cheeks which worsens upon exposure to the sunlight, oral ulcer
and easily got tired. She seek medical attention 10 months ago after getting an
episode of sudden weakness of all four limbs while having a 50 meters run. She
felt forward on her face. She complained of knee pain and backache but remained
conscious. She was brought to Hospital Serdang and given some pain killer and
intravenous fluid. She was then referred for physiotherapy to improve her
conditions, however it only getting worse. She was then referred to
Rheumatology Department for further management. Blood test was done which
suggestive of SLE. She was then started on tablet Prednisolone 30 mg once
daily. Brain CT scan was done and revealed some brain involvement. Currently,
she was on tablet Prednisolone 40 mg once daily (OD), tablet Hydroxychloroquine
200 mg OD, tablet Rocaltriol 0.25 mg BD and tablet Calcium carbonate 500 mg OD.
She is actually having an identical twin that had almost the same presentation
as her which includes redness over the cheeks area upon sunlight exposure,
joint swelling and joint pain.

During this admission, she was
afebrile, not tachypnoeic, and not tachycardic. However, she was hypotensive with
blood pressure of 118/58 mmHg. There was no episode of seizure during the
admission. Generally, she was pale and her hydration status seemed adequate. Neurological
examination of upper limb revealed normal tone and good power bilaterally. However,
there were presences of brisk reflexes of bilateral upper limbs. Sensation,
proprioception and coordination remained intact. The examination of lower limb
and cranial nerves showed normal finding. Other systems were unremarkable.

Blood investigation revealed normocytic
normochromic anaemia with haemoglobin level of 9.7 g/dL. There was no sign of
infection. Her urea and creatinine level were low which were 1.7 mmol/L and 50
umol/L respectively. All electrolytes were within the normal range. Her fasting
blood sugar was 4.7 mmol/L and electrocardiogram (ECG) showed sinus rhythm. Brain
CT scan showed right middle cerebral artery beaded appearance which is non-specific
but may suggest of some vasculitis changes.

Thus, this patient was treated with
3 pints of intravenous Normal Saline due to her hypotensive state. She was also
given intravenous Hydrocortisone 100 mg TDS. Apart from those medications, she
was asked to continue all of her current medication.

DISCUSSION

The exact aetiology of SLE is still
unknown. However, there may be some involvement between various genetic and environmental
factor.? The concordance of the disease in monozygotic twins is around 25-50%
and around 5% in dizygotic twins. Thus, for this patient, her twins should be
investigated too as they are identical twins and the risk of manifesting the
disease is high.

Patient with SLE may present with
various systemic manifestations. The general symptom they may be presented with
includes fever, malaise, headache, weight loss and loss of appetite.? One of
the commonest system involvements is musculoskeletal system.? Joint pain is one
of the most common reasons for the patients to come and seek treatment.? Dermatological
manifestation of SLE comprised four diagnostic criteria of lupus which are
malar rash, photosensitivity, discoid rash and alopecia.¹? Almost 20% of
patient have skin manifestation during the first presentation. Lupus nephritis
is a common manifestation but serious problem of SLE.¹¹ Approximately 25-75%
patient with SLE manifested the neurological symptoms which include headache,
vasculitis, meningitis, transient ischemic attack and mood disorders.¹? For this
patient, her brain was affected as CT scan showed some vasculitis changes. Vasculitis
is inflammation of the blood vessel wall.? She also presented with headache and
seizure. Other systems involvements in SLE are pulmonary, gastrointestinal, obstetric,
endocrine, hematological, cardiac, vascular and ocular system.

 

Classifications of SLE: the Systemic Lupus International Collaborating
Clinics (SLICC) criteria

Clinical criteria
–         
Acute cutaneous lupus erythematosus
–         
Chronic cutaneous lupus erythematosus
–         
Oral ulcer
–         
Non scarring alopecia
–         
Synovitis (?2 joints) or tenderness on palpation (? 2 joints) and
morning stiffness (?30 minutes)
–         
Serositis for more than 1 day
–         
Renal involvement
–         
Neurological involvement
–         
Hemolytic anaemia
–         
Leukopenia
–         
Thrombocytopenia

Immunological
criteria
–         
ANA level above laboratory reference range
–         
Anti-dsDNA antibodies
–         
Anti-Sm antibodies
–         
Antiphospholipid antibodies
–         
Low complement
–         
Direct Coombs test

For classification
as SLE, 4 criteria must be fulfilled (at least one clinical and at least one
immunological) or lupus nephritis has been diagnosed histologically in the
presence of anti-dsDNA antibodies or ANA.¹

 

Other investigations that can be
done apart from the autoantibodies and serum complement levels are full blood
count and urea and creatinine. Full blood count may show leucopenia,
thrombocytopenia or anaemia.?

The treatment for SLE depends on
the type and severity of the disease.¹² Generally patients are advised to use
sun protection, have proper diet, exercise, stop smoking and manage the
comorbid condition. Medications that are used to manage SLE are NSAIDs,
anti-malarial medication, corticosteroid, immunosuppressive agent and
monoclonal antibodies.¹² The long term prognosis for SLE has improved greatly
due to earlier diagnosis and advance in treatment.¹

 

CONCLUSION

This case report describes about a
patient who had been diagnosed with Systemic Lupus Erythematosus (SLE) with
cerebral vasculitis. She is currently on tablet prednisolone and tablet
hydroxychloroquine. Knowledge regarding SLE is important to reduce the
mortality and morbidity rate of the patient.